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

John A. Cowan, Matthew J. McGirt, Graeme Woodworth, Daniele Rigamonti, Michael A. Williams

Research output: Contribution to journalArticle

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 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. 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.

LanguageEnglish (US)
Pages540-547
Number of pages8
JournalNeurological Research
Volume27
Issue number5
DOIs
StatePublished - Jul 2005
Externally publishedYes

Fingerprint

Hydrocephalus
Gait Apraxia
Gait
Headache
Cognition
Ventriculostomy
Ventriculoperitoneal Shunt
Urinary Incontinence
Nausea
Vomiting
Dementia
Urinary Bladder
Pressure

Keywords

  • Adults ages 18-55 years
  • Endoscopic third ventriculostomy
  • Headache
  • Hydrocephalus
  • Syncope
  • Ventriculoperitoneal shunt

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

The syndrome of hydrocephalus in young and middle-aged adults (SHYMA). / Cowan, John A.; McGirt, Matthew J.; Woodworth, Graeme; Rigamonti, Daniele; Williams, Michael A.

In: Neurological Research, Vol. 27, No. 5, 07.2005, p. 540-547.

Research output: Contribution to journalArticle

Cowan, John A. ; McGirt, Matthew J. ; Woodworth, Graeme ; Rigamonti, Daniele ; Williams, Michael A. / The syndrome of hydrocephalus in young and middle-aged adults (SHYMA). In: Neurological Research. 2005 ; Vol. 27, No. 5. pp. 540-547.
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