IngentaConnect The syndrome of hydrocephalus in young and middle-aged adults (SH...

Abstract:

Objectives: Currently, headache, nausea/vomiting, visual changes, and altered mental status are accepted as indications for the evaluation of hydrocephalus in children; while dementia, gait apraxia, and urinary incontinence remain indications in the elderly. The clinical presentation of hydrocephalus in young and middle-aged adults remains poorly described. Hence, middle-aged patients with mild gait, cognitive, or urinary symptoms unaccompanied by clear exam findings often remain undiagnosed and untreated.

Methods: We report the clinical presentation, treatment, and outcomes of 46 adults (ages 16–55 years) presenting with congenital, acquired, or idiopathic hydrocephalus with imaging-documented ventriculomegaly and elevated CSF pressure.

Results: Primary symptoms were related to gait (70%), cognition (70%), urinary urgency (48%), and headaches (56%). Eighty-four percent complained of impaired job performance. The exam findings were subtle or absent (no gait apraxia, minor gait changes in 42.9%, mildly abnormal Mini Mental State exams in only14.3%, and incontinence in only 3.6%). Twenty-nine patients underwent ventriculoperitoneal (VP) shunting, and 11 endoscopic third ventriculostomy, of whom six subsequently required a VP shunt. Symptomatic improvement was observed in 93% of patients 16± 11 months after shunting (56% complete resolution, 37% partial resolution). Patients had been followed for their symptoms an average of 6 years (range, 1–30) prior to diagnosis.

Discussion: We propose that there exists a clinically distinct syndrome of hydrocephalus in young and middle-aged adults (SHYMA) that comprises hydrocephalus of all etiologies. SHYMA is characterized by complaints of impaired gait, cognition, bladder control, and headaches, with a discrepancy between the prominence of symptoms and the subtlety of clinical signs. Despite the subtlety of clinical signs, CSF diversion treatment is effective at resolving symptomatology.

Keywords: HYDROCEPHALUS; ADULTS AGES 18-55 YEARS; HEADACHE; SYNCOPE; VENTRICULOPERITONEAL SHUNT; ENDOSCOPIC THIRD VENTRICULOSTOMY

Document Type: Research article

DOI: 10.1179/016164105X17242

Affiliations: 1: Department of Neurosurgery, Baltimore, MD, USA 2: Department of Neurosurgery, Baltimore, MD, USA; Department of Neurology, Baltimore, MD, USA

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The syndrome of hydrocephalus in young and middle-aged adults (SHYMA)