Abstract Details

Normal pressure hydrocephalus (NPH) is a condition that is potentially reversible, which typically presents with the triad of gait disturbance, urinary incontinence and dementia. Multiple systems atrophy (MSA) is a rare sporadic neurodegenerative disease that is not reversible and is characterized by a variable combination of dysautonomia, parkinsonism and cerebellar signs.

To report a case of a 68-year-old male who presented with rapidly progressing gait instability and was diagnosed as a rare case of Normal Pressure Hydrocephalus (NPH) with Multiple System Atrophy-Cerebellar (MSA-C).

We report a case of a 68-year-old Filipino male, who developed rapidly progressing gait instability, executive dysfunction, associated with frequent falls, erectile dysfunction and urinary incontinence. On neurologic exam, patient had significant impairment of cognitive funtion (MOCA-E: 17/20, MMSE 25/30), no cranial nerve deficits, no motor or sensory deficits with bilateral dysmetria and dysdiadochokinesia, with wide-based stance with hesitancy on initiation, shorter step and stride length and decreased cadence. Cranial MRI showed ventriculomegaly with Evans index of 0.4, widening of the temporal horns of the lateral ventricles and dilated Sylvian fissures, with atrophy of brainstem and bilateral cerebellum.

The clinical presentation as well as imaging findings points out to an overlap between MSA-C and NPH, hence, patient underwent diagnostic and therapeutic lumbar puncture with normal pressure. There was improvement of gait afterwards, and patient subjectively feels comfortable on ambulating. Thus, NPH could be contributory for the patient's gait and memory lapses, therefore, VP shunt insertion was performed, despite underlying MSA. 4 weeks post-operatively, patient was noted to have improvement of gait and cognitive parameters (MOCA-E 22/30, MMSE 27/30).

Normal Pressure Hydrocephalus is potentially reversible and it is important to determine if it co-exist with other neurodegenerative disease that are treatment- resistant. Treatment should be individualized and performing CSF removal for shunt responsiveness should be considered.