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Neurology India, Vol. 59, No. 1, January-February, 2011, pp. 128-130 Letter to Editor Hemispheric intracranial lipoma with seizure: Look under the carpet Ajith Cherian, Neeraj N Baheti, Ramshekhar Menon, Rajesh S Iyer Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India A 13-year-old female consulted us for drug-resistant partial seizures of six years duration. She had multiple episodes of febrile seizures in her early childhood. The semiology of her habitual seizures were characterized by an aura of fear and rising epigastric sensation, verbalization followed by scurrying around with tonic posturing of both upper limbs and pedal automatism, culminating in micturition. The events were brief, and she used to get two to three seizures a month. Based on the magnetic resonance imaging (MRI, 0.5 tesla) findings, she underwent an incomplete right frontal lipoma resection at the age of six years elsewhere. Histology confirmed the presence of lipomatous tissue. She had no benefit post-operatively and later had a run up to daily seizures with occasional clustering of the same semiology. She had already been tried on seven drugs. She had video electroencephalograph which revealed focal epileptiform abnormality maximally over the right frontal region. Four complex partial seizures of right frontal origin were recorded. MRI (1.5 tesla) revealed a well-defined lobulated lesion along the right frontal cortical sulcal space and sylvian fissure suppressed on fat saturation sequence suggesting lipid components [Figure - 1]. There was thickening of right middle and inferior frontal gyrus extending from cortex through subcortical white matter to the frontal horn of right lateral ventricle suggestive of lipoma with transmantle focal cortical dysplasia (FCD) and polymicrogyria. Thus there was a good electroclinical-imaging concordance and re-surgery is being planned. Intracranial lipomas (ICLs) are congenital malformations composed of mature adipocytes that do not multiply but hypertrophy like other normal fat cells. [1] Intracranial lipomas account for only 0.1 to 1.3% of brain lesions [2] and are usually located in the pericallosal region, tuber cinereum, quadrigeminal plate and ambient cistern. [3] They may rarely be associated with more severe congenital anomalies such as corpus callosum agenesis. [2] Hemispheric location accounts for only 3 to 7% of all cases of ICLs. [1],[2],[3] ICLs are often incidental findings and rarely symptomatic. The presenting features include epilepsy, headache, and cranial nerve palsies. [3] Hemispheric cerebral lipomas are more likely to be symptomatic than midline lipomas [1] and the most common symptom is seizure. Our patient had hemispheric lipoma with underlying focal cortical dysplasia and epilepsy. Hemispheric sylvian fissure lipomas are still rare and there are about ten such cases reported in the literature often presenting with seizures. [1],[4],[5] Several associated cortical abnormalities reported in sylvian fissure lipomas include pachygyria, micropolygyria, and heterotopia of gray matter. [5] Various postulates are proposed for this rather unique lipoma -cortical dysplasia association. [6] Intracranial lipomas are considered to be due to persistence of the primitive meninx (mesenchymatous origin) followed by transformation into mature adipose cells during the initial weeks of intrauterine life. The presence of the lipoma could have secondary or concomitant effects on the development of adjacent cortical structures, which takes place between the sixth and twentieth weeks. [7] It may act as an obstacle to migration of gray matter, cause gyration abnormalities, or vascular defects resulting from the lesion and/or its hyper-vascularization. Ischemia could account for the polymicrogyria-like abnormalities that have been observed. [8] These indicate that the malformations associated with hemispheric ICLs are not limited to the lipoma itself. In our case the associated FCD was responsible for the seizures and this should have been born in mind before the option of epilepsy surgery was initially contemplated. Thus, in consideration of this complexity, hemispheric lipomas should be scrutinized more carefully so as not to miss associated FCD, and thus avoid incomplete lesion resection and burden of re-surgery for the patient. References
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