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.

Original languageEnglish (US)
Pages (from-to)540-547
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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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.",
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