scholarly journals Challenges in the surgical treatment of epilepsy: hypothalamic hamartoma in infancy - case report

2012 ◽  
Vol 18 (1) ◽  
pp. 12-15 ◽  
Author(s):  
Larissa Alessandra Mehl ◽  
Paulo Breno Noronha Liberalesso ◽  
Mônica Jaques Spinosa ◽  
Sílvio Machado ◽  
Alfredo Löhr Júnior
Keyword(s):  
Case Report ◽  
Seizure Control ◽  
Background Activity ◽  
Hypothalamic Hamartoma ◽  
Gelastic Seizures ◽  
Neurosurgical Procedure ◽  
Tuber Cinereum ◽  
Neurosurgical Treatment ◽  
Atonic Seizures ◽  
Infant Case

INTRODUCTION: Hypothalamic hamartoma is a rare congenital malformation, characterized by epilepsy, especially gelastic seizures, psychomotor developmental delay, mental retardation, behavioral disorders and precocious puberty. Epilepsy has early onset and is usually medically refractory. Etiology and pathophysiological mechanisms are unclear. The EEG can present disorganization and slowing of background activity and multifocal and/or generalized epileptogenic discharges. OBJECTIVE: To report the difficulties and challenges of neurosurgical treatment of a hypothalamic hamartoma in an infant. CASE REPORT: Infant with seizures since eight months old of age. The neurological investigation revealed a lesion in tuber cinereum suggestive of hamartoma. The epilepsy evolved with resistance to antiepileptic drugs, requiring neurosurgical procedure. The endoscopic resection could not be performed because the hamartoma was firmly attached to the hypothalamus. Currently, the child remains with tonic, clonic and atonic seizures. DISCUSSION: Lesionectomy performed by microsurgery or radiosurgery seems to be the most effective treatment for seizure control in patients with hypothalamic hamartomas who do not respond to clinical treatment. Callosotomy may be effective in selected cases, and lobectomy/cortical resections are not related to seizure control. In some patients, particularly in infants, lesionectomy and radiosurgery may be technically unfeasible.

Stereotaxy for Hypothalamic Hamartoma with Intractable Gelastic Seizures: Technical Case Report

Neurosurgery ◽  
1999 ◽  
Vol 44 (6) ◽  
pp. 1347-1350 ◽  
Author(s):  
Masafumi Fukuda ◽  
Shigeki Kameyama ◽  
Manabu Wachi ◽  
Ryuichi Tanaka
Keyword(s):  
Case Report ◽  
Hypothalamic Hamartoma ◽  
Gelastic Seizures

Dysembryoplastic neuroepithelial tumor presenting as a hypothalamic hamartoma in a child with gelastic seizures: case report

2020 ◽  
Vol 26 (1) ◽  
pp. 34-37
Author(s):  
Yuxiang Cai ◽  
Yanjin Wang ◽  
Zhiquan Yang
Keyword(s):  
Case Report ◽  
Clinical Manifestation ◽  
Neurological Examination ◽  
Histopathological Examination ◽  
Hypothalamic Lesion ◽  
Hypothalamic Hamartoma ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Intracranial Tumors ◽  
Gelastic Seizures ◽  
Neurosurgical Department

Dysembryoplastic neuroepithelial tumors (DNETs) are benign intracranial tumors of neuroglial origin, mostly located in the supratentorial regions and particularly in the temporal lobe. Few cases of DNETs in the hypothalamus have been described. The authors present the case of a DNET in the hypothalamus. The 5-year-old girl with complaints of limb and gelastic seizures was admitted to the neurosurgical department of Xiangya Hospital. Neurological examination findings were unremarkable. MRI showed isointensity without significant enhancement on T1- and T2-weighted images. The lesion exhibited clearly defined borders on the sagittal, coronal, and axial images. The preliminary diagnosis was hypothalamic hamartoma (HH); however, the lesion was surgically removed, and histopathological examination confirmed the diagnosis of a DNET. Hypothalamic DNETs are extremely rare. Based on their clinical manifestation and imaging, DNETs are easily misdiagnosed as HHs. Diagnoses apart from HHs must be entertained when a hypothalamic lesion is being investigated.

Epilessia gelastica ed amartomi del tuber cinereum

1997 ◽  
Vol 10 (2_suppl) ◽  
pp. 159-162
Author(s):  
F. Caranci ◽  
E. Castellano ◽  
G. Esposito ◽  
G. Zona ◽  
S. Cirillo ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Seizure Control ◽  
Third Ventricle ◽  
Clinical Manifestations ◽  
Review Of The Literature ◽  
Gelastic Seizures ◽  
Tuber Cinereum ◽  
The Third ◽  
Clinical Associations

A detailed study of three patients with gelastic seizures and a review of the literature are presented. Hamartoma of the tuber cinereum was diagnosed in two cases, subependymoma of the floor of the third ventricle in the third patient. The mechanism of ictal laughter, clinical associations and prognosis for seizure control are discussed. The resolution of MR imaging provides a basis to correlate some of the clinical manifestations with the anatomical disposition of the lesions within the hypothalamus.

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