, Volume 27, Issue 6, pp 1001-1005
Date: 06 Apr 2011

Fourth ventricle hamartoma presenting with progressive myoclonus and hemifacial spasms: case report and review of literature

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Cerebral hamartomas are rare tumor-like lesions composed of disorganized but mature cells, mostly a combination of neuronal or ganglion cells [1]. These lesions are commonly located in the cortex of temporal and frontal lobes, where may determine chronic or medically intractable epilepsy [2]. Hypothalamic hamartomas represent a rare but important model of subcortical epilepsy. Recent clinical studies, primarily based on intracranial seizure recordings, have established that the hypothalamic hamartoma is intrinsically epileptogenic. In some patients, however, the hamartoma contributes to secondary epileptogenesis affecting the neocortex [3]. Hamartomas of the floor of fourth ventricle (HFFV) are extremely rare. To date, only five cases have been reported in literature [47]. Common clinical signs are hemifacial spasms, eye blinking, nystagmic eye movements, and autonomic manifestations. It has been suggested that such signs represent a rare form of cerebellar epilepsy [57].