The following list of references is by no means exhaustive. If you know of other articles on HH that can be included, please contact us at craigndeb@eisa.net.au

This section is divided into the following areas. Abstracts are included where available.

HH & Gelastic Epilepsy

Alvarez G (1983) Neurology of pathological laughter, apropos of a case of gelastic epilepsy. Revista Medica de Chile, 111(12): 1259-62 (in Spanish).
No abstract available.

Ames FR, Enderstein O (1980) Gelastic epilepsy and hypothalamic hamartoma. South African Medical Journal, 58(4): 163-65.
The clinical, electro-encephalographic and neuroradiological findings in 2 boys with gelastic epilepsy are described. Both patients had hypothalamic masses thought to be hamartomas, and 1 had precocious puberty. These 2 cases are compared with a previously published case of gelastic epilepsy with a left temporal focus. It was not possible to differentiate the hypothalamic lesions from the left temporal lesion on clinical grounds. The laughter in the hypothalamic group did not lack affect, as described by some other authors. Interictal EEGs in the patients with hypothalamic lesions showed generalized wave-spike activity, whereas localized abnormality was present in the patient with a left temporal EEG focus. Ictal recordings were the same in both groups. The combination of gelastic epilepsy and precocious puberty is rare. Only 10 cases have been reported in the literature, our patient being the 11th.

Arita K, Ikawa F, Kurisu K, Sumida M, Harada K, Uozumi T, Monden S, Yoshida J, Nishi Y (1999) The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. Journal of Neurosurgery, 91 (2): 212-20.
Hypothalamic hamartoma is generally diagnosed based on its magnetic resonance (MR) imaging characteristics and the patient's clinical symptoms, but the relationship between the neuroradiological findings and clinical presentation has never been fully investigated. In this retrospective study the authors sought to determine this relationship. The authors classified 11 cases of hypothalamic hamartoma into two categories based on the MR findings. Seven cases were the "parahypothalamic type," in which the hamartoma is only attached to the floor of the third ventricle or suspended from the floor by a peduncle. Four cases were the "intrahypothalamic type," in which the hamartoma involved or was enveloped by the hypothalamus and the tumor distorted the third ventricle. Six patients with the parahypothalamic type exhibited precocious puberty, which was controlled by a luteinizing hormone-releasing hormone analog, and one patient was asymptomatic. No seizures or mental retardation were observed in this group. All patients with the intrahypothalamic type had medically intractable seizures, and precocious puberty was seen in one. Severe mental retardation and behavioral disorders including aggressiveness were seen in two patients. The seizures were controlled in only one patient, in whom stereotactically targeted irradiation of the lesion was performed. This topology/symptom relationship was reconfirmed in a review of 61 reported cases of hamartoma, in which the MR findings were clearly described. The parahypothalamic type is generally associated with precocious puberty but is unaccompanied by seizures or developmental delay, whereas the intrahypothalamic type is generally associated with seizures. Two thirds of patients with the latter experience developmental delays, and half also exhibit precocious puberty. Classification of hypothalamic hamartomas into these two categories based on MR findings resulted in a clear correlation between symptoms and the subsequent clinical course.

Armstrong SC, Watters MR, Pearce JW (1990) A case of nocturnal gelastic epilepsy. Neuropsychiatry, Neuropsychology and Behavioural Neurology, 3: 213-6.
No abstract available.

Arroyo S, Santamaria J, Sanmarti F, Lomena F, Catafau A, Casamitjana R, Setoain J, Tolosa E (1997) Ictal laughter associated with paroxysmal hypothalamopituitary dysfunction. Epilepsia, 38 (1): 114-7.
Seizures with ictal laughter (also termed gelastic seizures) have been associated with hypothalamic hamartomas and precocious puberty. It is not known, however, where in the brain such seizures originate. We describe a child with gelastic seizures and a hypothalamic lesion (probably a hamartoma) in whom two dysfunctional phenomena were observed. Our findings suggest that gelastic seizures associated with hypothalamic hamartomas are generated in the hypothalamus or in its neighboring regions and that these seizures may cause paroxysmal dysfunction of the hypothalamopituitary axis.
(Servicio de Neurologia, Hospital Clinic i Provincial de Barcelona, Spain)

Arroyo S, Lesser R, Gordon B, Uematsu S, Hart J, Schwerdt P, Andreassdon K, Fisher R (1993) Mirth, laughter and gelastic seizures. Brain, 116(Pt 4): 757-80.
Little is known about what pathways subserve mirth and its expression laughter. We present three patients with gelastic seizures and laughter elicited by electrical stimulation of the cortex who provide some insight into the mechanisms of laughter and its emotional concomitants. The first patient had seizures manifested by laughter without a subjective feeling of mirth. Magnetic resonance imaging showed a cavernous haemangioma in the left superior mesial frontal region. Ictal subdural electrode recording showed the seizure onset to be in the left anterior cingulate gyrus. Removal of the lesion and of the seizure focus rendered the patient virtually seizure free over 16 months of follow-up. The other two patients had complex partial seizures of temporal lobe origin. Electrical stimulation of the fusiform gyrus and parahippocampal gyrus produced bursts of laughter accompanied by a feeling of mirth. These cases reveal a high likelihood of cingulate and basal temporal cortex contribution to laughter and mirth in humans, and suggest the possibility that the anterior cingulate region is involved in the motor act of laughter, while the basal temporal cortex is involved in processing of laughter's emotional content in man.

Asanuma H, Wakai S, Tanaka T, Chiba S (1995) Brain tumors associated with infantile spasms. Pediatric Neurology, 12 (4): 361-4.
Two patients with brain tumors associated with infantile spasms are reported. Both infants displayed typical clinical features of infantile spasms, comprising tonic spasms manifesting in series and hypsarrythmia. In Patient 1, magnetic resonance imaging revealed a tumor in the hypothalamic region, suggestive of hypothalamic hamartoma. In Patient 2, cranial computed tomography and magnetic resonance imaging indicated the existence of a primary brain tumor with calcification in the right temporal lobe. Adrenocorticotropic hormone therapy combined with clonazepam relieved seizures in both infants. In Patient 1, resection of the hypothalamic tumor is impossible because the tumor lacks a stalk. In Patient 2, pathologic investigation of removed tumor tissue demonstrated mixed-oligoastrocytoma. It is suggested that focal lesions, like those in our patients, are involved in the development of infantile spasms.
(Department of Pediatrics, Sapporo Medical University, School of Medicine, Japan)

Bachman DS, Shultz J, Cooper R (1981) Cursive and gelastic epilepsy: case report. Clinical Electroencephalography, 12(1): 32-4.
A child with cursive and gelastic epilepsy is reported. This particular case in unique in that the patient had no underlying neurological disease, his running and laughing seizures represented his only seizure type; and recorded ictal episodes originated bilaterally and anteriorly.

Beningfield SJ, Bonnici F, Cremin BJ (1988) Magnetic resonance imaging of hypothalamic hamartomas. Case reports. British Journal of Radiology, 61: 1177-80.
No abstract available.

Berkovic SF, Andermann F, Melanson D, Ethier RE, Feindel W, Gloor P (1988) Hypothalamic hamartomas and ictal laughter: Evolution of a characteristic epileptic syndrome and diagnostic value of magnetic resource imaging. Annals of Neurology, 23 (5): 429-39.
Detailed study of 4 patients and review of the literature allowed us to delineate further the epileptic syndrome associated with hypothalamic hamartomas, which characteristically begins in infancy with laughing seizures. Because early childhood psychomotor development is usually normal, the condition appears benign and may not even be recognized. The episodes of laughter are brief, frequent, and mechanical in nature. These features distinguish it from other forms of epileptic laughter, particularly that which occurs in temporal lobe epilepsy. Subsequently, the seizures become longer, other seizure types appear, and between the ages of 4 and 10 years, the clinical and electroencephalographic features of secondary generalized epilepsy develop. Cognitive deterioration occurs and severe behavior problems are frequent. Prognosis for seizure control and social adjustment is poor. Cortical abnormality occurs in association with the hypothalamic hamartoma. The lesions are best detected by magnetic resonance imaging but may be difficult to identify by computed tomographic scanning.
(Montreal Neurological Institute and Hospital, Quebec, Canada)

Berkovic SF, Kuzniecky RI, Andermann F (1997) Human epileptogenesis and hypothalamic hamartomas: new lessons from an experiment of nature. Epilepsia, 38 (1): 1-3.
No abstract available.

Black D (1982) Pathological laughter: a review of the literature. Journal of Nervous Mental Diseases, 170(2): 67-71.
Normal laughter is a unique human behavior with characteristic facial and respiratory patterns elicited by a variety of stimulus conditions. The neuroanatomy remains poorly defined but three levels seem likely: a) a cortical level; b) a bulbar, or effector, level; and 3) a synkinetic, or integrative, level probably at or near the hypothalamus. Pathological laughter occurs when laughter is inappropriate, unrestrained (forced), uncontrollable, or dissociated from any stimulus. Pathological laughter is found in three main conditions: a) pseudobulbar palsy; b) gelastic epilepsy; and c) psychiatric illnesses. It is also found in other pathological conditions. What brings these together is their clinical similarity and probable disinhibition at higher brainstem levels.

Cerullo A, Tinuper P, Provini F, Contin M, Rosati A, Marini C, Cortelli P (1998) Autonomic and hormonal ictal changes in gelastic seizures from hypothalamic hamartomas. Electroencephalography and Clinical Neurophysiology, 107 (5): 317-22.
We describe two patients with hypothalamic hamartoma and gelastic seizures. We performed ictal neurophysiological studies with polygraphic recordings of autonomic parameters and hormonal ictal plasma concentration measurements. Ictal recordings showed a stereotyped modification of autonomic parameters: increase in blood pressure and heart rate, peripheral vasoconstriction and modification of respiratory activity. At seizure onset, the norepinephrine plasma level was high and epinephrine unchanged, whereas prolactin and adrenocorticotropic hormone were increased in both cases. Growth hormone and cortisol plasma concentrations in each patient showed a different response to seizures. These data provide evidence that gelastic seizures are accompanied by an abrupt sympathetic system activation, probably due to the direct paroxysmal activation of limbic and paralimbic structures or other autonomic centres of the hypothalamus and medulla.
(Neurological Institute, University of Bologna, Italy)

Chen RC, Forster FM (1973) Cursive epilepsy and gelastic epilepsy. Neurology, 25: 1019-29.
No abstract available.

Cheng K, Sawamura Y, Yamauchi T, et al (1993) Asymptomatic large hypothalamic hamartoma associated with polydactyly in an adult. Neurosurgery, 32: 458-60.
No abstract available.

Cook RW (1977) Hypothalamic hamartoma in a dog. Veterinary Pathology, 14 (2): 138-45.
A 10-month-old female, Wire-haired Pointing Griffon dog had a hamartoma of the hypothalamus. Episodes of sudden flaccid collapse had increased in frequency and duration for 7 months. Cerebrospinal fluid pressure was normal. A flat, pedunculated mass, 2.5 X 3.0 X 0.9 cm, covered the brain stem between the pituitary gland and pons. Its 1.2-cm-diameter connection to the hypothalamus obliterated the mammillary bodies and extended to the tuber cinereum, distorting the hypothalamus and displacing the third ventricle which also divided the rostral part of the mass. The tissue of the hamartoma resembled gray matter with bullous cytoplasmic vacuolation of many neurons, spongiform change, gemistocytosis and microscopic foci of calcification.

Daigtneault S, Braun CM, Montes JL (1999) Hypothalamic hamartoma: detailed presentation of a case. Encephale, 25 (4): 338-44. (In French)
We describe a seven year old child with a hypothalamic hamartoma. Classical symptoms of hypothalamic hamartoma include gelastic epileptic laughter, precocious puberty, aggressiveness, and progressively worsening epilepsy. After a normal first few years of life, this case presents all these symptoms except the precocious puberty. He has a markedly morbid personality disorder: he assaults strangers and relatives, bites people, spits in their faces unpredictably, is coprolalic and coprophagic, has gelastic laughter, puts pencils, erasers, and other non-comestible objects in his mouth, chews and ingests them, has tics (plays noisily with his saliva, empty chewing, compulsive spitting) and is self-injurious. None of the medications attempted to date have been of any help. Medical prognosis is somber, and this case is difficult to institutionalize, the more "congenial" institutions being insufficiently equipped to protect him and the beneficiaries and staff from his aggressive behavior. MRI showed the typical profile of hypothalamic hamartoma, and the diagnosis was confirmed with partial resection. This case illustrates that a tiny lesion, the size of a small cherry, can have extremely morbid psychological consequences. Detailed neuropsychological evaluation, certain unusual electroencephalographic traits and neurosurgical issues are discussed.
(Departement de Psychologie, Hopital de Montreal pour Enfants, France)

Daly DO, Mulder DV (1957) Gelastic epilepsy. Journal of Neurology, 7: 189-92.
No abstract available.

DiFazio MP, Davis RG (2000) Utility of early single proton emission computed tomography (SPECT) in neonatal gelastic epilepsy associated with hypothalamic hamartoma. Journal of Child Neurology, 15 (6): 414-7.
Gelastic epilepsy, or laughing seizures, is a rare seizure manifestation often associated with hypothalamic hamartoma. This seizure type is well described in older children and adults, but has only rarely been reported in neonates, oftentimes recognized in retrospect when the children are older. We report a child diagnosed at 3 months of age with a large hypothalamic mass after evaluation for spells occurring since birth. The spells were characterized by bursts of hyperpnea, followed by repeated "cooing" respirations, giggling, and smiling. These spells were recognized soon after birth in the delivery room, and occurred at 15-20 minute intervals. They did not interrupt feeding and occurred during sleep. On referral to our center, the patient was noted to be thriving, with normal medical and neurologic examinations except for his spells. The laboratory evaluation was normal, as were endocrine and ophthalmologic evaluations. Neuroimaging was performed, with magnetic resonance imaging demonstrating a large 2.8-cm isodense, nonenhancing hypothalamic mass. Electroencephalogram was abnormal, demonstrating bi-frontal sharp and spike-wave discharges. Video-EEG did not demonstrate ictal discharges associated with the patient's spells. Single photon emission computed tomography (SPECT) demonstrated dramatic ictal uptake in the area of the tumor, with normalization during the interictal phase. Partial excision of hamartomatous tissue has minimally improved the spells. In conclusion, this patient manifested an unusual, early presentation of a rare seizure type. SPECT scanning confirmed the intrinsic epileptogenesis of the hamartoma, further justifying a surgical approach to such patients. Early surgical intervention is probably indicated in an attempt to minimize or prevent the cognitive and behavioral sequelae commonly seen with this seizure type.
(Department of Child and Adolescent Neurology, Walter Reed Army Medical Center, Washington, DC, USA)

Dreyer R, Wehmeyer W (1978) Laughing in complex partial seizure epilepsy. A video tape analysis of 32 patients with laughing as symptom of an attack. Fortschritte der Neurologie, Psychiatrie und Ihrer Grenzgebiete, 46(2): 61-75.
According to videotape analysis, laughter is a frequent (42.7%) symptom during psychomotor attacks. The results of our investigations show that it is no longer possible to regard it as a "curiosity", as did Janz (1969). It is an epileptic phenomenon like others and a symptom of automatism. It can occur in all phases of an attack. It is not remembered by the patient. We have been unable to establish any connection with age or sex. The form of expression is usually natural but inadequate and no affective motivation has been established. Laughter during an epileptic attack is an inborn emotional expression, structurally triggered by the involvement of the area around the hypothalamus-thalamic nucleus with the process causing the epilepsy. It is not actively experienced and is therefore not conscious and not an expression of the pleasant side of the affective complex moderated by the limbic system. The EEG's showed the usual variations occurring in psychomotor epilepsy. The temporal lobes are particularly involved. There is no "EEG Laughter Pattern". The group of patients considered here consist of severe, therapy-resistent cases of partial seizure epilepsy with pronounced cerebral lesions. In order to determine whether laughter is so common in less severe cases, a comparison group must be investigated. Laughter as a symptom of an epileptic attack is unknown to doctors and nursing staff and thus is either not recorded at all or, only very seldom. "Gelastic epilepsy" so-called does not exist as a nosology entity. This term should thus only be used--if at all--in cases where the laughter, together with a change in the level of consciousness, has over a period of years constantly been the only symptom of an attack, expecially when these attacks first became manifest in earliest childhood and are due to connatal changes in the hypothalamus-thalamic region.

Dreyer R, Wehmeyer W (1977) Fits of laughter (gelastic epilepsy) with a tumour of the floor of the third ventricle. A video tape analysis. Journal of Neurology, 214(3): 163-71.
A patient with fits of laughter due to a tumorous alteration (hyperplasia) of the floor of the third ventricle is described with electroencephalographic findings indicative of focal epilepsy (complex partial seizures = psychomotor fits). The laughter is interpreted as an inborn emotional expression with structural substrate in the hypothalamus and neighboring brain. With structures remaining intact functional disorders in this area can cause epileptic phenomena with participation of the limbic system.

Druckman R, Chao D (1957) Laughter in epilepsy. Neurology, 7: 26-36.
No abstract available.

Encha-Razavi F, Larroche JC, Rourne J, Migne G, Delezoide AL, Gonzales M, Mulliez N (1992) Congenital hypothalamic hamartoma syndrome: nosological discussion and minimum diagnostic criteria of a possibly familial form. American Journal of Medical Genetics, 42 (1): 44-50.
We report on congenital hypothalamic hamartomas, discovered at autopsy in 3 unrelated fetuses. In the first 2 patients, the tumor was associated with skeletal dysplasia only. In the third patient, it was part of a non-random congenital malformation association, suggestive of Meckel syndrome. In one family, a previous boy died soon after birth with similar craniofacial and skeletal abnormalities. As far as we know, the association between isolated skeletal dysplasia and congenital hypothalamic hamartomas has not yet been documented in the literature. Nevertheless, a spectrum of skeletal abnormalities has been described in association with congenital hypothalamic "hamartoblastoma" and a constellation of variable visceral malformations under the eponym of "Pallister-Hall syndrome" (PHS). A detailed analysis of the PHS reported cases shows that only skeletal dysplasia and oro-facial abnormalities are present constantly. They show similarities with those found in our first 2 cases. These findings prompt us to consider skeletal dysplasia and oro-facial abnormalities as common denominator and minimum criteria required to define a nosologically distinct, possibly familial entity, which we suggest calling "congenital hypothalamic hamartoma syndrome" (CHHS).
(Departement d'Histologie-Embryologie, CHU Henri Mondor, Creteil, France)

Feeks EF, Murphy GL, Porter HO (1997) Laughter in the cockpit: gelastic seizures – a case report. Aviation Space & Environmental Medicine, 68 (1): 66-8.
We present a case of gelastic seizures in a student naval aviator. He was noted to have uncontrollable fits of laughter on several occasions, but was not referred to his flight surgeon until he had a gelastic seizure while flying in formation, which jeopardized the safety of the flight. He had an aura consisting of lack of concentration, which was then followed by 10 s or less of hysterical laughter. For the previous year and a half, he had had frequent episodes of nocturnal laughter so loud that he woke members of his household and occasionally himself. His neurological evaluation was normal, except for an electroencephalogram (EEG) and a separate video recording, which documented the ictal nature of his events. Gelastic seizures have not previously been discussed in the literature of aerospace medicine. This case illustrates a rare condition that should be considered in patients presenting with inappropriate laughter, and serves as a reminder of the need for continuous, ongoing evaluation of all aircrew by the cognizant flight surgeon.
(Training Air Wing Five, Naval Air Station, Whiting Field, Florida, USA)

Frattali CM, Liow K, Craig GH, Korenman LM, Makhlouf F, Sato S, Biesecker LG, Theodore WH (2001) Cognitive deficits in children with gelastic seizures and hypothalamic hamartoma. Neurology, 57 (1):43-6.
Our objective was to characterize the cognitive deficits in children with gelastic seizures and hypothalamic hamartoma and investigate the relationship of seizure severity to cognitive abilities. Eight children with gelastic seizures and hypothalamic hamartoma completed a neuropsychological battery of standardized and age-normed tests, including the Woodcock-Johnson Psycho-Educational Battery-Revised: Tests of Cognitive Ability, Peabody Picture Vocabulary Test-III, and initial-letter word fluency measure. All children displayed cognitive deficits, ranging from mild to severe. Gelastic/complex partial seizure severity was correlated with broad cognitive ability standard scores (r = -0.79; r2 = 0.63; (F[1,6] = 10.28; p = 0.018]. Frequency of gelastic/complex partial seizures was also correlated with broad cognitive ability standard scores (r = -0.72; r2 = 0.52; F[1,6] = 6.44; p = 0.044). Significant intracognitive standard score differences were found, with relative weaknesses in long-term retrieval (mean = 64.1; SD = 13.3) and processing speed (mean = 67.7; SD = 21.6) and a relative strength in visual processing (mean = 97.6; SD = 12.8). Performance in visual processing differed from performance in long-term retrieval (p = 0.009) and processing speed (p = 0.029). These findings are consistent with cognitive functions and affective/emotional states associated with conduction pathways of the hypothalamus involving cortical association areas and amygdala and hippocampal formation. These abnormalities can account for the prominent deficit found in integrating information in the processing of memories.

Garcia A, Gutierrez MA, Barrasa J, Herranz JL (2000) Cryptogenic gelastic epilepsy of frontal lobe origin: a paediatric case report. Seizure, 9 (4): 297-300.
Gelastic (laughing) seizures are an uncommon seizure type which in most cases has an organic cerebral pathology and specifically a hypothalamic hamartoma. The interictal EEG frequently shows focal activity. This report describes a 3 1/2-year-old boy who presented with episodes of unmotivated laughter associated with other epileptic symptomatology before the age of 3 years. Prolonged ambulatory EEG monitoring recorded electroclinical seizures starting in the right frontal area and spreading to the adjacent frontotemporal region. Neurological examination and brain magnetic resonance imaging were normal. Vigabatrin resulted in immediate remission of the seizures and normalization of the EEG.
(Services of Clinical Neurophysiology, University Hospital Marques de Valdecila, Santander, Spain)

Gascon GG, Lombroso CT (1971) Epileptic (gelastic) laughter. Epilepsia, 12: 63-76.
No abstract available.

Georgakoulias N, Vize C, Jenkins A, Singounas E (1998) Hypothalamic hamartomas causing gelastic epilepsy: two cases and a review of the literature. Seizure, 7(2): 167-71.
Two cases of hypothalamic hamartomas causing gelastic epilepsy are described. The clinical presentations and the radiological features are presented, and the mechanisms involved in laughing attacks are discussed. The literature is reviewed and it is suggested the complete extirpation of the hamartomas is the treatment of choice in gelastic epilepsy.

Glassman JN, Dryer D, McCartney JR (1986) Complex partial status epilepticus presenting as gelastic seizures: a case report. General Hospital Psychiatry, 8(1): 61-4.
A middle-aged man, who presented to the emergency room because of bizarre outbursts of laughter, was found to be in partial complex status epilepticus. His seizure disorder had been misdiagnosed, at various times, as a variety of "functional" psychiatric disorders. Despite proper diagnosis and aggressive treatment, management was difficult, being complicated by postictal agitation and confusion, postictal psychosis, and interictal compulsive and paranoid personality features. This case is described, and issues of diagnosis and management in partial complex epilepsy are briefly discussed. The importance of not overlooking organic and especially epileptic factors, despite the presence of prior psychiatric illness, psychologic contributors, and environmental stressors, is emphasized.

Gomibuchi K, Ochiai Y, Kanraku S, Maekawa K (1990) Infantile spasms and gelastic seizure due to hypothalamic hamartoma. No to Hattatsu (Brain & Development), 22 (4): 392-4 (in Japanese).
No abstract available.

Guibaud L, Rode V, Saint-Pierre G, Pracros JP, Foray P, Tran-Minh VA (1995) Giant hypothalamic hamartoma: an unusual neonatal tumor. Pediatric Radiology, 25 (1): 17-8.
A case of neonatal manifestation of giant hypothalamic hamartoma is reported. It is suggested that hypothalamic hamartoma should be included in the list of neonatal intracerebral tumors. Magnetic resonance imaging appearance similar to that of normal gray matter on T1-weighted images and slightly hyperintense on T2-weighted images, without enhancement after gadolinium injection, is suggestive of the diagnosis. Hypothalamic hamartomas are congenital malformations, consisting of disorganized mature neuronal elements in proportions similar to that of normal tissue [1]. They are clinically evidenced in infants ranging from 1 to 7 years of age [1-5]. This report describes a histologically proved giant hypothalamic hamartoma diagnosed in the neonatal period. Magnetic resonance imaging (MRI) is helpful to distinguish this congenital non-evolutive malformation from more aggressive neonatal tumors.
(Department of Radiology, Montreal General Hospital, McGill University, PQ, Canada)

Gumpert J, Hansotia P, Upton P (1970) Gelastic epilepsy. Journal of Neurology, Neurosurgery and Psychiatry, 33: 479-83.
No abstract available.

Hahn FJ, Leibrock LG, Huseman CA, Makos MM (1988) The MR appearance of hypothalamic hamartoma. Neuroradiology, 30 (1): 65-8.
Hypothalamic hamartoma is the most common detectable cerebral lesion causing precocious puberty. Two histologically confirmed cases were studied by computerized tomography (CT) and magnetic resonance (MR) imaging. T2 weighted, sagittal MR images were superior to CT in delineating the tumor from surrounding grey matter. The lesion was isointense to grey matter on T1 weighted images allowing exclusion of other hypothalamic tumors. MR will undoubtedly become the imaging modality of choice in the detection of hypothalamic hamartoma.
(Department of Radiology, University of Nebraska Medical Center, Omaha, USA)

Holmes GL, Dardick KR, Russman BS (1980) Laughing seizures (gelastic seizures) in childhood. Clinical Pediatrics, 19(4): 295-6.
No abstract available.

Iannetti P, Spalice A, Raucci U, Atzei G, Cipriani C (1997) Gelastic epilepsy: video-EEG, MRI and SPECT characteristics. Brain & Development, 19 (6): 418-21.
Gelastic epilepsy, or ictal laughter, is a relatively uncommon type of seizure which may occur singly or, more frequently, with other types of convulsions. Gelastic seizures have been observed to be associated with many different conditions, mainly hypothalamic hamartomas. We report on a patient whose ictal laughter was the only neurologic disturbance. Ictal video-EEG demonstrated seizure arising from the left frontal region with subsequent involvement of the contralateral homologous area and secondary generalization. MRI showed an enlarged left frontal horn of the lateral ventricle. Postictal SPECT, performed 6 min after the seizure had ended, showed hypoperfusion in the bilateral frontoparietal region and in both cerebellar hemispheres; the presence of this abnormality may be due to the spreading of the cortical epileptogenic focus and to the complex intercommunication between the frontal cortex and the cerebellar hemispheres. Interictal SPECT, in accordance with MRI features, demonstrated a left frontoparietal hypoperfusion. The neurofunctional features observed in the reported child could suggest that gelastic epilepsy originates in the frontal cortex. However, further studies are undoubtedly needed to define the pathogenetic mechanisms of ictal laughter.
(Department of Pediatrics (VII), University La Sapienza, Roma, Italy)

Inoue HK, Kanazawa H, Kohga H, Zama A, Ono N, Nakamura M, Ohye C (1995) Hypothalamic Harmartoma: Anatomic, Immunohistochemical and Ultrastructural Features. Brain Tumor Pathol, 12 (1): 45-51.
Four patients with hypothalamic hamartoma were examined by CT and/or MR imaging, immunohistochemistry and electron microscopy. The hamartomas arose from the hypothalamus and extended inferiorly. LH-RH neurons were detected in three cases by immunohistochemistry. Electron microscopy revealed large myelinated axons, axon terminals containing dense-core vesicles and axon terminals with clear vesicles forming asymmetrical synapses. The development of hypothalamic hamartoma and its functional manifestations (precocious puberty and laugh attacks) are discussed in reference to the migration of LH-RH neurons from the olfactory placode.
(Department of Neurosurgery, Gunma University School of Medicine)

Ironside R (1956) Disorders of laughter due to brain lesions. Brain, 79: 589-609.
No abstract available.

Khadilkar S, Menezes K, Lele V, Katrak S (2001) Gelastic epilepsy – a case report with SPECT studies. Journal of the Association of Physicians of India, 49: 581-3.
A 24 years male presented with daily episodes of uncontrollable laughter followed by urinary incontinence since the age of nine years. Some of these attacks progressed to generalized tonic-clonic seizures. General and neurological examination did not reveal any abnormality. Ictal and interictal video EEGs were normal. MRI showed a hypothalamic hamartoma. Interictal SPECT scan showed normal perfusion in the hamartoma. SPECT scan obtained four minutes after beginning of seizure showed that the perfusion increased in right cingulate gyrus but not in the hamartoma, suggesting the involvement of the cingulate gyrus in the seizure origin or pathway.
(Department of Neurology, Grant Medical College and Sir JJ Group of Hospitals, Mumbai)

Kuzniecky R, Guthrie B, Mountz J, Bebin M, Faught E, Gilliam F, Lu H-G (1997) Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy. Annuals of Neurology, 42: 60-7.
Hypothalamic hamartomas and gelastic seizures are often associated with cognitive deterioration, behavioral problems, and poor response to anticonvulsant treatment or cortical resections. The origin and pathophysiology of the epileptic attacks are obscure. We investigated 3 patients with this syndrome and frequent gelastic seizures. Ictal single-photon emission computed tomography performed during typical gelastic seizures demonstrated hyperperfusion in the hamartomas, hypothalamic region, and thalamus without cortical or cerebellar hyperperfusion. Electroencephalographic recordings with depth electrodes implanted in the hamartoma demonstrated focal seizure origin from the hamartoma in 1 patient. Electrical stimulation studies reproduced the typical gelastic events. Stereotactic radiofrequency lesioning of the hamartoma resulted in seizure remission without complications 20 months after surgery. The functional imaging findings, electrophysiological data, and results of radiofrequency surgery indicate that epileptic seizures in this syndrome originate and propagate from the hypothalamic hamartoma and adjacent structures.
(Department of Neurology, University of Alabama at Birmingham Epilepsy Center, USA)

Kuzniecky R, Guthrie B, Mountz J, Gilliam F, Faught E (1995) Hypothalamic hamartomas and gelastic seizures: evidence for subcortical seizure generation by ictal SPECT and cerebral stimulation (Abstract). Epilepsia, 36 (suppl 3):S266.

Lehman RM (1983) Gelastic seizures: case report. Alaska Medicine, 25(2): 50-2.
No abstract available.

Lehtinen L, Kivalo A (1965) Laughter epilepsy. Acta Neurologica Scandinavica, 41: 255-61.
No abstract available.

Liebaldt GP (1971) Hypothalamic hamartoma in a case of uncontrollable exhibitionism. Journal of Neuro-Visceral Relations, 0 (0): suppl 10: 713-9.
No abstract available.

Lin YY, Yiu CH, Kwan SY, Tu YF, Wong TT, Chang KP, Su MS (1995) Hypothalamic hamartoma and gelastic epilepsy: a case report. Chung Hua I Hsueh Tsa Chih – Chinese Medical Journal, 55 (1): 78-82.
We studied a 6-year-old girl who presented with inappropriate and uncontrollable laughing episodes since age 3. Physical examination revealed a precocious puberty. The luteinizing hormone-releasing hormone (LH-RH) stimulation test showed an increased level of follicle-stimulating hormone (FSH). The interictal electroencephalogram (EEG) was normal. Several laughing fits were documented during video/EEG monitoring. During laughing, the ictal EEG showed a diffuse suppression of background rhythm, prominent over the left mesial temporal region. A mass lesion about 2 x 2 cm in size was found over the suprasellar cistern with a broad base attached to the hypothalamus, which was isodense on a computed tomography (CT) scan, isointense to gray matter on T1-weighted magnetic resonance (MR) imaging and hyperintense on T2-weighted MR imaging. The findings were suggestive of a hypothalamic hamartoma. A variety of anticonvulsants had been used with little or no response to the frequency or duration of the laughing seizures.
(Section of Neurology, Veterans General Hospital-Taipei, Taiwan, R.O.C.)
Read the full article here.

Loiseau P, Cohadon F, Cohadon S (1971) Gelastic epilepsy: a review and report of five cases. Epilepsia, 12: 313-23.
No abstract available.

Lono-Soto A, Takahashi M, Yamashita Y, Sakamoto Y, Shinzato J, Yoshizumi K (1991) MRI findings of hypothalamic hamartoma: report of five cases and review of the literature. Computerized Medical Imaging and Graphics, 15 (6): 415-21.
Hypothalamic hamartoma is a relatively rare congenital malformation, associated with the clinical presentation of precocious puberty of central type. Five cases with hypothalamic hamartoma are reported here, with an emphasis on MR appearance. The most common presentation of hypothalamic hamartoma was a small and well defined mass in the inferior aspect of the hypothalamus, showing isointensity on T1 weighted images and hyperintensity on T2 weighted images compared with the gray matter. The previous reports with MRI description are reviewed and compared with the present results.
(Department of Radiology, Kumamoto University School of Medicine, Japan)

Mami C, Tortorella G, Barberio G, Scaffidi M (1983) Gelastic epilepsy. Report of a case. Minerva Pediatrica, 35(18): 899-902 (in Italian)

No abstract available.

Marcuse PM, Burger RA, Salmon GA (1953) Hamartoma of the hypothalamus. Report of two cases with associated developmental defects. Journal of Pediatrics, 43: 301-8.
No abstract available.

Marimuthu C, Prashanth Tharyan A, Prabhakaran K (1997) Gelastic epilepsy. A case study of neurological disorder. Nursing Journal of India, 88(5): 105-6.
No abstract available.

Markin RS, Leibrock LG, Huseman CA, McComb RD (1987) Hypothalamic hamartoma: a report of two cases. Pediatric Neuroscience, 13 (1): 19-26.
Two patients with hypothalamic hamartoma presented with isosexual precocious puberty. LHRH challenge showed a pubertal LH response in both cases. Serum FSH responses to LHRH were pubertal in case 1, but prepubertal for case 2. Computed tomography revealed isodense noncontrast-enhancing retrosellar mass lesions in both cases. The tumors were composed of mature neurons and neuroglial tissue. Electron microscopy of the lesions failed to demonstrate dense core (neurosecretory) granules in either case. Subtotal removal of the harmartomas resulted in decreased LH responsiveness to LHRH in both cases. Serum FSH responsiveness to LHRH was not significantly suppressed postoperatively in case 1, and FSH responsiveness to LHRH in case 2 showed exaggerated levels, more typical of very young prepubertal girls. Postoperative magnetic resonance imaging (MRI) scans of both patients are also presented.
(Department of Pathology, University of Nebraska Medical Center, Omaha, USA)

Marliani AF, Tampieri D, Melanccon D, Ethier R, Berkovic SF, Andermann F (1991) Magnetic resource imaging of hypothalamic hamartomas causing gelastic epilepsy. Canadian Association of Radiologists Journal, 42(5): 335-39.
Hypothalamic hamartomas may cause a peculiar epileptic syndrome characterized by seizures of laughter and precocious puberty. Four mentally handicapped patients suffering from gelastic epilepsy were referred to our institution for investigation; three of them also presented with precocious puberty. In all four cases magnetic resonance imaging (MRI) revealed a space-occupying lesion of the hypothalamus that was considered to be a hamartoma. Biopsies were not performed. Hamartomas appear isodense in plain computed tomography scans, and they do not enhance. Such lesions display an isointense signal in T1-weighted magnetic resonance images and a hyperintense signal in proton density and T2-weighted images. MRI is the procedure of choice for detecting such lesions at the base of the brain.

Martijn A (1984) Radiologic findings in a hypothalamic hamartoma. Diagnostic Imaging in Clinical Medicine, 53(4):182-5.
This report presents a case of a hypothalamic hamartoma in a 4-month-old boy. Ultrasound demonstrated the tumor and its effect on the ventricular system. Computed tomography and ventriculography confirmed these findings and were complementary in the diagnosis. To the author's best knowledge, this is the first ultrasound documentation of a hypothalamic hamartoma.

Matsuzaki M, Izumi T, Ebato K, Suzuki H, Shishikura K, Osawa M, Fukuyama Y, Shimizu N (1991) Hypothalamic GH Deficiency and gelastic seizures in a 10 year old girl with MELAS. No to Hattatsu (Brain & Development), 23(4): 411-6 (in Japanese).
A case of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes, in which a pituitary growth hormone (GH) secretion deficiency of hypothalamic origin was revealed through neuro-endocrinological examinations, was described. The case was a 10-year-old girl, who had been suffering from generalized tonic seizures since age 5, four episodes of alternating hemiplegia since age 6, stunted growth since age 7, and simple partial motor seizures as well as gelastic seizures since age 8. Marked elevation of lactate and pyruvate in both serum and CSF, abundant ragged red fibers in biopsied muscle, and low density areas in the left occipital lobe and bilateral globus pallidus in addition to diffuse brain atrophy on CT scan and MRI of the head were demonstrated, although the activities of muscle enzymes complex I-IV were within normal ranges. Pituitary GH secretion was deficient under the loadings with insulin, L-DOPA, sleep, and a single growth hormone releasing factor (GRF) administration, but normal GH response was registered under the repetitive stimulation with GRF. Activities of other hormonal axes were normal. It is likely that short stature commonly observed in MELAS patients is due to hypothalamic dysfunction, which might be brought out by chronic ischemia and energy deficiency of the diencephalon based upon mitochondrial abnormality of that region. It is likely that gelastic seizure in this case is due to hypothalamic dysfunction.

Mori K, Handa H, Takeuchi J, Hanakita J, Nakano Y (1981) Hypothalamic hamartoma. Journal of Computer Assisted Tomography, 5 (4): 519-21.
Hypothalamic hamartoma is a rare tumor with onset of symptoms in infancy or early childhood. Clinical presentation includes precocious puberty, laughing spells, and seizures. Computed tomography of two cases of hypothalamic hamartomas revealed a mass lesion in the suprasellar--interpeduncular cisterns (with the density of) the surrounding normal brain. The mass was not enhanced by injection of contrast material.

Munari C, Kahane P, Francione S, Hoffman D, Tassi L, Cusmai R, Vigevano F, Pasquier B, Betti O (1995) Role of the hypothalamic hamartoma in the genesis of gelastic fits (a video-stereo-EEG study). Electroencephalography and Clinical Neurophysiology, 95: 154-60.
Patients having a hypothalamic hamartoma frequently present epileptic attacks of laughter, and they later experience multiple additional seizure types, which invariably lead to a severe drug-resistant epilepsy. If this association is now well-known, relationships between the hypothalamic mass and the different types of seizures remain still mysterious. We report the case of a 16-year-old girl suffering from this peculiar epileptic picture, in whom a stereo-EEG study was performed, allowing us to record both the hamartoma, the neighboring hypothalamic structures, and other bilateral cortical areas. It showed that gelastic fits were strictly linked to ictal discharges which began and remained well localized in the hamartoma. Conversely, atonic seizures, which might result from a secondary epileptogenesis, admitted a widely extended bilateral frontal cortical origin, sparing the lesion, and slightly involving the posterior hypothalamus. Stereotactic radiosurgery of the hamartoma proved to be ineffective on both types of seizures, probably because of the too low dose of X-rays delivered (18 grays), as suggested by the absence of hypothalamic mass changes on MRI. Such data, never reported to our knowledge, seem able to contribute to a better understanding of this very peculiar epileptic syndrome, and perhaps to a better adapted therapeutic management.
(Neurosciences Dpt, CHRU Grenoble, France)

Mutani R, Agnetti V, Dureilli L, et al (1979) Epilepyic laughter: electroclinical and cinefilm report of a case. Journal of Neurology, 220: 215-22.
No abstract available.

Nakagawa N, Takahashi M, Kobrogi Y, Kodama T, Matsukado Y (1986) Neuroradiologic findings of hypothalamic hamartoma with emphasis on computed tomography. Journal of Computed Tomography, 10 (1): 77-83.
Hypothalamic hamartoma is a relatively rare congenital malformation. Five new cases and 31 cases in the literature were evaluated in regard to neuroradiologic findings with emphasis on computed tomography. Five important computed tomography findings were observed: oval-shaped, isodense suprasellar mass; clear demarcation; no enhancement effect; absence of cystic component or calcification; and no effacement of the third ventricle. Clinical features, mechanism of the precocious puberty, and differential diagnosis are also discussed.

Nishio S, Fujiwara S, Aiko Y, Takeshita I, Fukui M (1989) Hypothalamic hamartoma. Report of two cases. Journal of Neurosurgery, 70 (4): 640-5.
Two cases of hypothalamic hamartoma are presented. The first patient was a 4-year-old boy with precocious puberty, and the second was a 6-year-old boy with epileptic seizures. In both patients, clinical symptoms and signs appeared at the age of 2 years and progressed thereafter. Computerized tomography and magnetic resonance imaging in both cases disclosed a suprasellar mass lesion in continuity with the hypothalamus. Removal of the lesions affected the endocrinological status and/or seizure control. Pathological examination revealed the lesions to be composed of well-differentiated neuronal and glial cells. Immunohistochemical study demonstrated the presence of beta-endorphin, corticotropin-releasing factor, oxytocin, and neurofilament protein (210 kD) in the neuronal cells of the first patient, but no neuropeptides were detected in the second. Electron microscopic examination on the second patient disclosed the presence of many nonmyelinated and some myelinated neuronal processes containing dense-core and clear vesicles. The morphological characteristics and the role of surgery for this lesion are discussed.
(Department of Neurosurgery, Faculty of Medicine, Kyushu University, Fukuoka, Japan)

Nuevo Bono FJ, Nieto Barrera M (1984) A case of gelastic epilepsy in a child (epileptic crisis with affective semiology of laughing). Anales Espanoles de Pediatria, 21(9): 858-60 (in Spanish).

No abstract available.

Nurbhai MA, Tomlinson BE, Lorigan-Forsythe B (1985) Infantile hypothalamic hamartoma with multiple congenital abnormalities. Neuropathology and Applied Neurobiology, 11: 61-70.
No abstract available.

Paillas Je, Roger J, Toga M, Soulayrol R, Salamon G, Dravet C, et al (1969) Hamartome de l'hypothalamus: etude clinique, radiologique, histologique. Resultats de l'exerese. Revue Neurologique, 120: 177-94.
No abstract available.

Pendl G (1975) Gelastic epilepsy in tumors of the hypothalamic region. In Penzholz H, Brock M, Hamer J, Klinger M, Spoerri O (eds) Advances in Neurosurgery 3. Berlin, Springer-Verlag, pp 442-49.
No abstract available.

Pilo L (1990) Gelastic epilepsy – a case report. Singapore Medical Journal, 31(1): 78-9.
Gelastic epilepsy is an uncommon phenomenon and it is particularly uncommon in adults. This paper describes a case of gelastic epilepsy in a middle-aged woman presented in a psychiatric hospital. A short history of the condition, clinical and electroencephalographic findings in gelastic epilepsy and causes of pathological laughter are discussed.

Ponsot G, Diebler C, Plouin P, Nardou M, Dulac O, Chaussain JL, Arthuis M (1983) Hamartomes hypothalamiques et crises de rire. A propos de 7 observations. Archives Francaises de Pediatrie, 40 (10): 757-61 (in French).
Seven cases of hypothalamic hamartomas with gelastic seizures are reported. A precocious puberty was found in 4 cases. The normal neurologic examination and lack of sign of intracranial hypertension were in contrast with the severity of the epileptic seizures, of the mental impairment and of the behavioral disorders. The fact that the presenting symptom may be gelastic seizures is stressed. CT scan is the best means to assess the diagnosis and to follow the evolution of these tumors. Except for the management of the precocious puberty, the treatment is disappointing and neurosurgical indications are quite exceptional.

Robben SG, Tanghe HL, Drop SL (1994) Hypothalamic hamartoma. Journal Belge de Radiologie, 77 (5): 221.
No abstract available.

Roger J, Lob H, Waltregny A, Gastaut H (1967) Attacks of epileptic laughter. Report on 5 cases. Electroenceph clin Neurophysiol, 22: 279P.
No abstract available.

Sackheim HA, Greenberg MS, Weiman AL, Gur RC, Hungerbuhler JP, Geschwind N (1982) Hemispheric asymmetry in the expression of positive and negative emotions. Neurologic evidence. Archives of Neurology, 39(4): 210-8.
Three retrospective studies were conducted to examine functional brain asymmetry in the regulation of emotion. In the first study, reports of 119 cases were collected of pathological laughing and crying associated with destructive lesions. Pathological laughing was associated with predominantly right-sided damage, whereas pathological crying was associated with predominantly left-sided lesions. In the second study, 19 reports detailing mood following hemispherectomy were collected; right hemispherectomy was associated with euphoric mood change. In the third study, lateralization of epileptic foci was assessed in reports of 91 patients with ictal outbursts of laughing (gelastic epilepsy). Foci were most likely to be predominantly left-sided. The findings are congruent with studies of the effects of unilateral brain insult on mood, and a general model of hemispheric asymmetry in the regulation of emotion is presented.

Sato H, Ushio Y, Arita N, et al (1985) Hypothalamic hamartoma: report of two cases. Neurosurgery, 16: 198-206.
Two histologically confirmed hypothalamic hamartomas, one in a 7-year-old boy and another in a 10-year-old boy, are reported. One patient had precocious puberty, epileptic laughter, and abnormal behavior; the other had cerebral seizures. Partial removal of the tumors had no effect on precocious puberty; however, behavior improved in the first patient, and seizure control improved in the second patient.

Sebit MB, Suleman MI (1998) A case of gelastic seizures in Harare, Zimbabwe. Central African Journal of Medicine, 44 (4): 109-10.
We describe a very rare case of a frontal lobe epilepsy that presented with gelastic seizures. It occurred in a 19 year old male and the gelastic seizures were controlled by carbamazepine 400 mg/day.
(Department of Psychiatry, Faculty of Medicine, University of Zimbabwe, Harare)

Sethi PK, Surya Rao T (1976) Gelastic, quiritarian and cursive epilepsy: a clinicopathological appraisal. Journal of Neurology, Neurosurgery and Psychiatry, 39: 823-8.
No abstract available.

Shar P, Patkar D, Patankar T, Shah J, Srinivasa P, Krishnan A (1999) MR imaging features in hypothalamic hamartoma: a report of three cases and review of literature. Journal of Postgraduate Medicine, 45 (3): 84-6.
Hypothalamic hamartomas are rare tumours of particular interest because of their unusual symptoms. Three cases of hypothalamic hamartomas are reported in children, who presented with precocious puberty and gelastic seizures.
(Department of Radiology, Seth G. S. Medical College and K.E.M Hosital, Parel, Mumbai, India)

Sharma MC, Gaikwad S, Mahapatra AK, Menon PS, Sarkar C (1998) Hypothalamic hamartoma: report of a case with unusual histologic features. American Journal of Surgical Pathology, 22 (12): 1538-41.
A rare case of hypothalamic hamartoma with unusual radiologic and histopathological features is described, possibly the first of its type in English literature. A 1.5-year-old female child presented with precocious puberty. MR scan of the brain revealed a pedunculated hypothalamic mass, most of which was isointense with normal brain on T1- and T2-weighted images. However, a sizeable component of the lesion was hyperintense on T1-weighted images, suggestive of adipose tissue. Microscopically, the lesion was a hamartoma composed of an admixture of neuroectodermal elements, namely glial cells, neurons, and nerve bundles along with mesenchymal elements in the form of fibroadipose tissue.
(Department of Pathology, All India Institute of Medical Sciences, New Delhi)

Sher PK, Brown SB (1976) Gelastic epilepsy: Onset in neonatal period. American Journal of Diseases in Children, 130(10): 1126.
The phenomenon of gelastic epilepsy was first described in 1873, yet fewer than 100 patients with this disorder have been reported on to date. The purpose of this article is to report on the first two patients to our knowledge with the onset of these seizures in the immediate neonatal period. Both patients have been shown to have posterior hypothalamic mass lesions presumably of congenital origin, and have remained free of neurologic progression of the disease with conservative treatment.

Sisodiya SM, Free SL, Stevens JM, Fish DR, Shorvon SD (1997) Widespread cerebral structural changes in two patients with gelastic seizures and hypothalamic hamartoma. Epilepsia, 38 (9): 1008-10.
We tested the hypothesis that widespread extralesional abnormalities of cerebral structure exist in association with apparently isolated hypothalamic hamartomata, providing a structural basis for the poor response of seizures to removal of the hamartoma or other apparently focal epileptogenic zones present. High-resolution magnetic resonance imaging (MRI) brain scans of 2 patients with hypothalamic hamartomata were quantified by determination of regional distribution and symmetry of distribution of cortical gray matter and subcortical matter volumes. The results were compared with normal ranges for the distribution of such tissues in 33 controls. Both patients had abnormalities of distribution of gray and subcortical matter, whereas control subjects did not. These abnormalities were beyond the hamartoma itself, in areas of cerebrum that on visual inspection alone appeared completely normal. We conclude that extralesional abnormalities of cerebral structure are present in the cerebrum of patients with hypothalamic hamartoma, as in most patients with other dysgeneses. These abnormalities may explain the poor outcome of epilepsy surgery in patients with this form of dysgenesis. These preliminary findings require further investigation.
(Epilepsy Research Group, Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom)

Sugama S, Ito F, Eto Y, Maekawa K (1992) A case of frontal lobe epilepsy presenting with gelastic seizures. No to Hattatsu (Brain & Development), 24(5): 475-9.
We described a 9-year-old boy with frontal lobe epilepsy presenting with gelastic seizures. CT-scan showed mild widening of the left sylvian fissure. Abnormal findings in the left frontal operculum were detected by both MRI and SPECT. Attacks mainly consisted of gelastic seizures with comfortable feeling followed by screaming with fear. Administration of anticonvulsants resulted in reducing the frequency and severity of seizures. Finally the patient had brief laughter attacks only. In the present case, the clinical course suggests that the gelastic seizures does not occur by way of the spreading of epileptic discharges to the temporal or hypothalamic region; rather it might occur as a focal symptom of the frontal region.

Tasch E, Cendes F, Li LM, Dubeau F, Montes J, Rosenblatt B, Andermann F, Arnold D (1998) Hypothalamic hamartomas and gelastic epilepsy: a spectroscopic study. Neurology, 51 (4): 1046-50.
Patients with hypothalamic hamartomas present with epileptic attacks of laughter and later experience multiple seizure types and cognitive decline, suggestive of secondary generalized epilepsy. It has been suggested in the past that gelastic seizures originate in the temporal lobes rather than in the hamartoma, but temporal resections have been ineffective. Recent electrophysiologic evidence suggests that the epileptogenic discharges may originate in the hamartoma itself.  We used proton magnetic resonance spectroscopic imaging to quantify the amount of neuronal damage in the temporal lobes and hamartomas of patients with hypothalamic hamartomas and gelastic seizures. Five patients were studied and the relative intensity of N-acetylaspartate to creatine (NAA/Cr) was determined for both temporal lobes as well as for the hamartoma. These values were compared with signals from the temporal lobes and hypothalami of normal control subjects. Our results show that NAA/Cr was not significantly different from normal control subjects for either temporal lobe, nor was there a significant asymmetry between the two temporal lobes for any of the patients. NAA resonance signals were present in the hamartomas, and the ratio of NAA to Cr was decreased in the hamartomas compared with the hypothalami of normal control subjects (t = 4.5, p = 0.005). We found no detectable neuronal damage in the temporal lobes of patients with hypothalamic hamartomas and gelastic epilepsy. This is further evidence that gelastic seizures do not originate in the temporal lobes of these patients.
(Department of Neurology and Neurosurgery, McGill University, Montreal Neurological Hospital and Institute, Quebec, Canada)

Tonami H, Higashi K, Okamoto K, Akai T, Iizuka H, Nojima T, Takahashi H, Yamamoto I (2001) Report of changing signal intensity on follow-up MRI in a case of hypothalamic hamartoma. Journal of Computer Assisted Tomography, 25 (1): 130-2.
We present a case of hypothalamic hamartoma in which the signal intensity of the lesion significantly changed during the course of follow-up. To date, stability of the lesion morphology over time has been considered an important diagnostic criterion of hypothalamic hamartoma. Radiologists should be aware that in hypothalamic hamartoma, signal intensity can change during its natural course.
(Department of Radiology, Kanazawa Medical University, Ishikawa, Japan)

Weissenberger AA, Dell ML, Liow K, Theodore W, Frattali CM, Hernandez D, Zametkin AJ (2001) Aggression and psychiatric comorbidity in children with  hypothalamic hamartomas and their unaffected siblings. Journal of the American Academy of Child & Adolescent Psychiatry, 40 (6):696-703.
Our objective was to assess aggression and psychiatric comorbidity in a sample of children with hypothalamic hamartomas and gelastic seizures and to assess psychiatric diagnoses in siblings of study subjects. Children with a clinical history of gelastic seizures and hypothalamic hamartomas (n = 12; age range 3-14 years) had diagnoses confirmed by video-EEG and head magnetic resonance imaging. Structured interviews were administered, including the Diagnostic Interview for Children and Adolescents-Revised Parent Form (DICA-R-P), the Test of Broad Cognitive Abilities, and the Vitiello Aggression Scale. Parents were interviewed with the DICA-R-P about each subject and a sibling closest in age without seizures and hypothalamic hamartomas. Patients were seen from 1998 to 2000. Children with gelastic seizures and hypothalamic hamartomas displayed a statistically significant increase in comorbid psychiatric conditions, including oppositional defiant disorder (83.3%) and attention-deficit/hyperactivity disorder (75%). They also exhibited high rates of conduct disorder (33.3%), speech retardation/learning impairment (33.3%), and anxiety and mood disorders (16.7%). Significant rates of aggression were noted, with 58% of the seizure patients meeting criteria for the affective subtype of aggression and 30.5% having the predatory aggression subtype. Affective aggression was significantly more common (p < .05). Unaffected siblings demonstrated low rates of psychiatric pathology on semistructured parental interview and no aggression as measured by the Vitiello Aggression Scale. We conclude that children with hypothalamic hamartomas and gelastic seizures had high rates of psychiatric comorbidity and aggression. Parents reported that healthy siblings had very low rates of psychiatric pathology and aggression.

Yamada H, Yoshida H (1977) Laughing attack: a review and report of nine cases. Folia Psychiatrica et Neurologica Japonica, 31: 129-37.
No abstract available.

Zampolio A, Adami A, Pedeferri M, Petrone M, Zacchetti O (1982) Apropos of gelastic epilepsy. Description of a clinical case and general observations. Rivista di Neurobiologia, 28(1-2): 98-109 (in Italian).
No abstract available.

Feilberg Jorgensen N, Brock Jacobsen B, Ahrons S, Starklink H (1998) An association of hypothalamic hamartoma, central precocious puberty and juvenile granulosa cell tumour in early childhood. Hormone Research, 49 (6): 292-4