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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Hypothalamic hamartoma. 726 91

Hypothalamic hamartoma are rare lesions. We report a new series of eight patients treated for precocious puberty (six cases) or gelastic seizures (two cases). Surgical resection was total in four cases (three pediculated and one sessile). Precocious puberty was controlled by surgical treatment in all cases. Gelastic seizures were controlled by medical treatment, but the patients did not become seizure free. We observed no mortality and no endocrinological or visual morbidity. The fact that a vascular "rete mirabilis" was observed on the surface of the lesion in our surgical material is an argument favoring a vascular mechanism in precocious puberty. Coagulation of this vascular structure can help control precocious puberty. Our series confirms that the hypothalamic hamartoma can be surgically treated when patients fail to respond to medical treatment, when the length of the treatment cannot be tolerated by the chidren and their families, and when there are uncontrolled gelastic seizures
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PMID:Hypothalamic hamartoma: the role of surgery in a series of eight patients. 1139 42

Hypothalamic hamartoma is a non-neoplastic tumor manifesting as gelastic seizure, precocious puberty, and abnormal behavior. Treatment of it is very complicated due to its location. We report a case of hypothalamic hamartoma treated by neuroendoscopic surgery and stereotactic radiosurgery. A 5-year-old girl presented with violent behavior, precocious puberty, gelastic seizure and atonic seizure. She was diagnosed with hypothalamic hamartoma by CT and magnetic resonance imaging at 11 months of age. Tumor size did not change, but tumor intensity had changed on the MR image at 5 years of age. Magnetic resonance spectroscopy revealed decreased N-acetylaspartate and increased choline and creatine in the tumor. After neuroendoscopic biopsy, she underwent linear accelerator stereotactic radiosurgery. But her symptoms remained unchanged for 6 months. She then underwent partial resection and laser coagulation of the tumor by a neuroendoscopic approach. After the procedure, the frequency of her seizures was remarkably decreased, and her violent behavior improved. The transventricular neuroendoscopic approach to the hypothalamus is less invasive than the radical surgery. Neuroendoscopic surgery can be one of the treatments of choice for hypothalamic hamartoma.
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PMID:Treatments of hamartoma with neuroendoscopic surgery and stereotactic radiosurgery: a case report. 1249 60

Hypothalamic hamartoma presents with precocious puberty, epilepsy or both. There are two epileptic syndromes, one presenting initially in infancy with gelastic seizures evolving rapidly into a syndrome with multiple seizures, developmental delay and a moderate to severe behaviour disorder. The other presents later with a milder epileptic syndrome, again usually including gelastic seizures, but with normal intellect and behaviour. Magnetic resonance imaging identifies and gives a detailed anatomical picture of these lesions. Direct surgery, using microsurgical techniques and neuronavigation guidance has been used for these lesions. Three surgical approaches have been used, one lateral pterional, another midline frontal through the lamina terminalis and a third is a transcallosal interforniceal approach. In addition a disconnection procedure, usually pterional, aims to disconnect the lesion without the risks of major resection. The transcallosal interforniceal approach is the most successful with 69% of patients seizure-free. There are complications in about 24% of patients, the same as other approaches, but the complications are milder and include fewer neurological deficits than the other routes. Alternate strategies include stereotactic radiosurgery and radiofrequency ablation under stereotactic control.
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PMID:Resective surgery for hypothalamic hamartoma. 2470 77

A 19-year old female with intractable gelastic seizures was found to have 0.7 x 1.8 x 1.8 cm elliptical mass on the floor of the third ventricle. The signal intensity on the Magnetic Resonance Imaging (MRI) was consistent with the Hypothalamic Hamartoma (HH). Ictal EEG demonstrated rhythmic 7 Hz waves over Fp2, F4, and C4 with spreading to the right temporal region and then bilaterally. Ictal Single Photon Emission Computerized Tomography (SPECT) showed hyperperfusion at hypothalamic and medial frontopolar regions. The patient underwent surgical resection using Trans Callosal Subchoroidal Approach (TCSA) to the third ventricle. Pathological finding confirmed the diagnosis of hypothalamic hamartoma. Following the operation, she has been seizure free up to 12 months. Thereafter, provoked seizures seldom occurred and there has been improvement in her memory, emotional control and independence. This appears to be the first report of this surgical approach for HH, which is less likely to disturb memory function compared to previously described interfoniceal approach.
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PMID:Successful resection of Hypothalamic Hamartoma with intractable gelastic seizures--by transcallosal subchoroidal approach. 1704 40

Hypothalamic hamartoma (HH) is a congenital malformation of the hypothalamus that may be asymptomatic or manifest with precocious puberty or seizures. Gelastic seizures often begin early in life, even in the newborn period, being manifest by frequent attacks of inappropriate laughter resulting from seizure activity in the HH. The scalp electroencephalogram (EEG) is often normal in children with gelastic seizures, such that the diagnosis of epilepsy and the finding of a HH are often delayed. In a proportion of children with HH, there is an epileptic progression, in which complex partial seizures with frontal, temporal, and lateralized clinical features appear, usually with the appearance of focal slowing and epileptiform activity on the interictal EEG. Further progression may ensue with the appearance of tonic or atonic drop attacks, generalized tonic-clonic seizures, and epileptic spasms; rarely, infantile spasms may be the presenting seizure type. With the appearance of generalized seizures, the interictal EEG shows bilaterally synchronous and generalized epileptiform activity, often in abundance. The mechanism of this evolution is incompletely understood but neocortical seizure propagation and secondary epileptogenesis are believed to be important. Paralleling the development of the focal and generalized electroclinical manifestations in children with HH is usually slowing of development and the appearance of behavioral problems. Fortunately, many of these neurologic manifestations can be arrested, or reversed, with effective surgical treatment directed at the HH.
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PMID:Epilepsy in hypothalamic hamartoma: clinical and EEG features. 1754 48

Hypothalamic hamartoma (HH) syndrome comprises the clinical triad of epilepsy, developmental retardation, and central precocious puberty. A predominant opinion has been that the acquired cognitive and behavioral disorders observed in children with this syndrome are a direct effect of their seizure activity. A review of the recent literature suggests that this opinion needs to be revised because it is only partially supported by the data. The size of the HH and its anatomic attachment/location, in addition to the seizure history, appear to contribute to the cognitive and behavioral disturbances in children with HH. Small sample sizes and the inability to use standard neuropsychological testing scales in more severely affected HH patients complicate the study of causality. The present literature, however, suggests that multiple factors contribute to the cognitive and behavioral problems of these children.
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PMID:Cognitive and behavioral dysfunction in children with hypothalamic hamartoma and epilepsy. 1754 49

Hypothalamic hamartomas (HHs) are associated with a catastrophic form of childhood epilepsy and are intrinsically epileptogenic for the gelastic seizures that are peculiar to this disorder. The cellular mechanisms of seizure generation within HH tissue are unknown. Hypothalamic hamartoma tissue consists of well-differentiated neurons interspersed with glial cells. Based on intrinsic electrophysiologic properties, we hypothesized that small and large HH neurons would have different ultrastructural features. Surgically resected HH tissue samples from 7 patients with refractory epilepsy were studied using electron microscopy. Models of neurons were made from reconstruction of serial sections. Nissl body density, polyribosome density, glycogen density, and the ratio of nuclear area to cytoplasmic area differed significantly between small and large neurons. Abundant small neurons were found in clusters and elaborated terminals that made symmetric, putatively inhibitory, synapses. Symmetric synapses were found predominantly on the soma and proximal dendrites of large projection-like neurons, whereas asymmetric, putatively excitatory, synapses were found primarily on distal dendrites. All samples showed unusual dendritic varicosities. The presence of these morphologically and functionally distinct neurons, the large number of nerve fibers in the neuropil, and the presence of inhibitory and excitatory synapses suggest that HH tissue has the necessary substrate to generate seizures.
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PMID:Hypothalamic hamartomas associated with epilepsy: ultrastructural features. 1859 47

Hypothalamic hamartoma (HH) is the pathologic hallmark of a spectrum of epileptic conditions, ranging from a mild form of epilepsy, whose seizures are an urge to laugh without cognitive defects, to the fully developed syndrome of early onset gelastic seizures (GS) associated with precocious puberty and the evolution to a catastrophic epilepsy syndrome. However, a refractory focal or generalized epilepsy develops during the clinical course in nearly all cases. Neurophysiologic and neuroimaging studies have assessed the role of HH in the generation of the GS as well as in the process of secondary epileptogenesis. Electrophysiologic properties of small gamma-aminobutyric acid (GABA)ergic, spontaneously firing neurons might explain the intrinsic epileptogenicity of HH. Surgical ablation of the HH can reverse both epilepsy and encephalopathy. Gamma-knife radiosurgery and image-guided robotic radiosurgery can be useful and safe approaches for treatment, in particular of small HH.
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PMID:The syndrome gelastic seizures-hypothalamic hamartoma: severe, potentially reversible encephalopathy. 1946 51

Hypothalamic hamartoma (HH) is malformation of the brain manifesting by paroxysmal disorders (gelastic seizures) and endocrine disorders (mostly precocious sexual development). Surgical resection of HH is associated with the risk of severe neurological and neuroendocrine complications. Alternative ways of treatment are being investigasted. Stereotactic radiosurgery is one of the leading techniques in treatment of pharmacoresistant seizures due to high effectiveness and low complication rate. Since 2005 till 2009 7 patients with HH were treated in Burdenko Moscow Neurosurgical Institute. Age of patients varied between 9 months and 20 years 9 mean - 8.5 years). In all cases the disease manifested by gelastic seizures with frequency differing between 2-3 per months and 17 per day. All patients received long-term combined anticonvulsant treatment (using valproates, carbamazepine, lamotrigine, oxcarbazepine, topiramate) with temporary and insufficient effect or without any effect. In 4 patients frequency of seizures increased during anticonvulsant therapy. I patient had been operated (partial resection of HH) with temporary effect which consisted of decrease in frequency of seizures for 8 months; later frequency of paroxysms increased. Main indications to radiosurgical treatment included progressive course of disease, low effectiveness of anticonvulsant therapy and high risk of direct surgical treatment. 6 patients were treated using linear accelerator "Novalis" and 1 using "Cyber-knife". Treatment was performed under pharmacological sedation (midazolam, ketamine). Marginal dose was 11-18 Gy (mean 15.7 Gy). Volume of hamartomas was 0.3-3 cm3. Follow-up period varied between 3 and 50 months. Decreased frequency of seizures was observed in 100% of cases. Stereotactic radiosurgery is an effective and safe method of treatment for HH and may be considered as treatment of choice for pharmacoresistant seizures caused by HH.
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PMID:[Stereotactic radiosurgery in treatment of patients with hypothalamic hamartomas]. 2043 59


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