Management of Central Precocious Puberty in Children with Hypothalamic Hamartoma

Hypothalamic hamartoma (HH) is a rare, congenital, and benign lesion of the tuber cinereum, typically presenting with central precocious puberty (CPP), gelastic seizure, and developmental delay. This study aimed to investigate CPP in HH patients and compare clinical features between before and after gonadotropin-releasing hormone (GnRH) agonist treatment. A total of 30 HH patients under 18 years of age who visited Severance Children’s Hospital between January 2005 and May 2020 were retrospectively reviewed. Fourteen patients were male (46.7%) and sixteen (53.3%) were female, with a mean age at diagnosis was4.2 ± 2.9 years. During follow-up, 24 patients (80.0%) were diagnosed with CPP, 15 patients (50.0%) had gelastic seizure, and 13 patients (43.3%) had developmental delay. The gelastic seizure was significantly associated with sessile type HH rather than pedunculated type HH (85.7% vs. 18.8%, p = 0.001). After GnRH agonist treatment, discrepancies between bone age and chronological age decreased (3.3 ± 1.3 years to 2.0 ± 1.7 years, p = 0.002). Additionally, height standard deviation score for bone age was increased, and predicted adult height increased significantly in females, while males showed an increasing trend. Clinical symptoms of HH were closely associated with the location of HH, and GnRH agonist treatment was safe and effective in the management of CPP caused by HH.

Hypothalamic hamartoma with gelastic seizures: a case report

Hypothalamic Hamartoma (HH) may have diverse clinical manifestations. Its hallmark association is with gelastic seizures. Gelastic epilepsy is characterized by episodes of loud, hollow, mirthless, stereo-typed, forced laughter. The patient may stare and giggle briefly without any other motor manifestations. Hypothalamic hamartoma is most often the cause of gelastic seizures. Here, authors report a case of gelastic seizure with hypothalamic hamartoma in a 14-month-old boy with an associated tonic clonic seizure. This case highlights the possibility of underdiagnosed hypothalamic hamartoma in younger age groups among pediatric population.

A Girl Who is Smiling for no Reason: Gelastic Seizure- A Case Report

Being a rare disorder, Gelastic seizure primarily presents as episodes of sudden occurrence of emotions usually associated with laugh or giggle due to facial contractions. A child presented with abnormal body movement in the form of smiling and uprolling of eyes, which was clinically diagnosed as gelastic seizure, a rare form of a very common presentation, epilepsy. The case is presented to highlight abnormal posturing and smiling episodes in a child can be a form of seizure episode, which generally presents with a diagnostic dilemma and therapeutic nightmare to the clinician.

Cervical spine hangman fracture secondary to a gelastic seizure

A 42-year-old man reported to our service with a 1-week history of vague cervical neck pain on a background history of a gelastic seizure disorder. Radiological imaging confirmed a type II hangman’s fracture through the C2 pedicle. A CT angiogram of carotid and vertebral arteries was normal, and the patient was managed with an occipitocervical fusion. The patient had no neurological insult and was discharged on day 5 with a Miami-J collar for 6 weeks. Although there are case report evidence of lumbar and thoracic vertebral fractures secondary to seizures, this is the first report of a spine injury resulting from a gelastic seizure.

SYMPTOMATIC LAUGHTER IN A PATIENT WITH ORBITOFRONTAL SEIZURE: A SURGICAL CASE WITH INTRACRANIAL ELECTROENCEPHALOGRAPHIC STUDY

OBJECTIVE A rare case of orbitofrontal lobe epilepsy manifesting gelastic seizure is reported. CLINICAL PRESENTATION A 49-year-old woman had developed weekly complex partial seizures consisting of nonverbal vocalization and unresponsiveness followed by laughter. Magnetic resonance imaging revealed a round tumorous lesion at the posterior side of the right rectal gyrus and medial orbitofrontal gyrus. Neuroimaging studies and electrophysiological examinations, including intracranial electroencephalographic monitoring, suggested the existence of an epileptogenic zone in the ipsilateral orbitofrontal gyrus, including the lesion. INTERVENTION After partial right prefrontal lobectomy including lesionectomy, the patient became seizure-free during a follow-up period of 33 months. We speculated that the limbic system, including the orbitofrontal lobe and temporal structures, which have a strong connection with the pontine nuclei, might be involved in this patient's gelastic seizure. CONCLUSION Except for impaired consciousness, the clinical manifestations did not correspond to the characteristics of orbitofrontal seizure described by the International League Against Epilepsy. Symptomatic laughter in epilepsy that originates from the orbitofrontal lobe is very rare. Intracranial electroencephalographic findings and ictal symptomatology associated with epileptogenesis in this rare case are discussed.