A Case of Congenital Muscular Dystrophy Associated with Hydrocephalus. CSF Dynamics and Surgical Treatment.:CSF Dynamics and Surgical Treatment

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A case of Fukuyama-type congenital muscular dystrophy (FCMD) associated with hydrocephalus was reported. A 15-month- old male, diagnosed as FCMD at 4 months old, revealed progressing large head. CT scan and MRI indicated not only lissencephaly II classified by Dobyn, but dilated ventricular system and subarachnoid space, and these findings radiologically resembled those of brain atrophy. Intracranial pressure and CSF dynamics monitoring revealed high intracranial pressure and high CSF outflow resistance, indicating a high pressure hydrocephalus. V-P shunt made the patient improved not only symptomatically, but physiologically (increased CBF and decreased intracranial pressure and CSF outflow resistance). We concluded that the early diagnosis and treatment of hydrocephalus superimposing the organic disease may prevent secondary damage of the brain, and the intracranial pressure and CSF dynamics monitoring is essential for its diagnosis.
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