The syndrome of hydrocephalus in young and middle-aged adults (SHYMA).
OBJECTIVE
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 only 14.3%, 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.


CONCLUSIONS
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.
Cowan
John A
JA
Department of Neurosurgery, Baltimore, MD, USA.
McGirt
Matthew J
MJ
Woodworth
Graeme
G
Rigamonti
Daniele
D
Williams
Michael A
MA
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D014695:Cerebral Ventriculography / Q000379:methods; D015170:Cerebrospinal Fluid Pressure / Q000502:physiology*; D003071:Cognition / Q000502:physiology; D005260:Female; D005684:Gait / Q000502:physiology; D006261:Headache / Q000503:physiopathology; D006339:Heart Rate / Q000502:physiology; D006801:Humans; D006849:Hydrocephalus / Q000145:classification / Q000175:diagnosis / Q000503:physiopathology* / Q000601:surgery; D006850:Hydrocephalus, Normal Pressure / Q000503:physiopathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D014549:Urinary Incontinence; D017287:Ventriculoperitoneal Shunt / Q000379:methods