Normal pressure hydrocephalus in a patient with myotonic dystrophy: case report with a 10-year follow-up.

Abstract

Myotonic dystrophy (MD) is the most common adult muscular dystrophy involving multiple organs. Normal pressure hydrocephalus (NPH) is characterized by gait apraxia, urinary incontinence, and dementia. NPH in association with MD has been reported in only three cases. This report provides for the first time the complete evaluation, treatment, and long-term follow-up of a patient with NPH and MD. A 61-year-old man known to have MD complained of 4 years of progressive deterioration of long-term memory and gait disturbance, which was the main clinical feature. Computed tomography scan and magnetic resonance imaging showed ventricular enlargement. Intracranial pressure monitoring showed B waves with amplitude superior to 9 mm Hg in more than 5% of the recording. We performed a ventriculoperitoneal shunt with clear postoperative improvement, confirming the diagnosis of NPH. The clinical improvement has remained stable through prolonged follow-up. Association between MD and NPH could be more than coincidental, and it is important to recognize this potential insidious association because a ventriculoperitoneal shunt can provide excellent long-term clinical improvement.