Contents

Search

normal pressure hydrocephalus (NPH)

Etiology: 1) idiopathic 50% 2) prior subarachnoid hemorrhage, meningitis, trauma or intracranial surgery [4] Epidemiology: 1) generally occurs in older patients (4th-6th decade [4]) 2) no sex predilection 3) rare disorder [4] Pathology: - failure of CSF absorption by the pacchonian granulations Clinical manifestations: 1) progressive dementia less prominent than gait disorder a) cognitive slowing, forgetfulness b) agnosia, aphasia typically absent 2) unsteady gait (initial symptoms) a) difficulty initiating gait "magnetic feet" (as if the patient's feet were stuck to the floor) b) repeated falls c) parkinsonian features: 1] short, shuffling steps 2] stooped posture 3) urinary incontinence (proximate to unsteady gait) [2] 4) presentation may be complicated by comorbidities (case presentation) [10] Laboratory: - lumbar puncture (CSF analysis) a) generally normal CSF pressure b) gait, incontinence & cognition may improve with large volume (> 20 mL) CSF removal c) improvement of symptoms with CSF removal may predict response to VP shunt d) tau in CSF & phospho-tau in CSF may predict response to CSF removal [9] Radiology: - neuroimaging - enlargement of the ventricles out of proportion to the cortical atrophy* - computed tomography (CT) of the brain - magnetic resonance imaging may be superior to CT imaging [7] * essential for diagnosis [2] Management: 1) ventriculo-peritoneal (VP) shunt a) unproven benefit [2] b) must be response to high-volume CSF removal or lumbar drainage [2] c) patients with clear cause of hydrocephalus most likely to benefit [2] d) gait & incontinence more likely to improve than cognition [3]* e) better outcomes with early shunting [4] 2) ventricular shunt with an externally controlled programmable valve that allows regulation of cerebrspinal fluid pressure [2] * videos showing gait improvement [7]

Interactions

disease interactions

General

hydrocephalus neurodegenerative disease

Database Correlations

OMIM 236690

References

  1. Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018, 2021 - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
  4. Ferri's Clinical Advisor, Instant Diagnosis and Treatment, Ferri FF (ed), Mosby, Philadelphia, 2003, pg 420
  5. Lee WJ, Wang SJ, Hsu LC et al Brain MRI as a predictor of CSF tap test response in patients with idiopathic normal pressure hydrocephalus. J Neurol. 2010 Oct;257(10):1675-81 PMID: 20512347
  6. Williams MA, Relkin NR. Diagnosis and management of idiopathic normal-pressure hydrocephalus. Neurol Clin Pract. 2013 Oct;3(5):375-385. PMID: 24175154
  7. AbdelRazek MA, Venna N Ventriculoperitoneal-Shunt Placement for Normal-Pressure Hydrocephalus. N Engl J Med 2017; 377:e35. Dec 28, 2017 PMID: 29281574 http://www.nejm.org/doi/full/10.1056/NEJMicm1701226
  8. Halperin JJ, Kurlan R, Schwalb JM et al Practice guideline: Idiopathic normal pressure hydrocephalus: Response to shunting and predictors of response: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2015 Dec 8;85(23):2063-71. Review. Erratum in: Neurology. 2016 Feb 23;86(8):793. PMID: 26644048 Free PMC Article
  9. Muller-Schmitz K et al. Normal pressure hydrocephalus associated with Alzheimer's disease. Ann Neurol 2020 Oct; 88:703 PMID: 32662116 https://onlinelibrary.wiley.com/doi/10.1002/ana.25847
  10. Marouf F, Glover M, Wininger B, Curry WT Case 10-2021: A 70-Year-Old Man with Depressed Mood, Unsteady Gait, and Urinary Incontinence. N Engl J Med 2021; 384:1350-1358. April 8. PMID: 33826823 https://www.nejm.org/doi/full/10.1056/NEJMcpc2027090
  11. NINDS Normal Pressure Hydrocephalus Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Normal-Pressure-Hydrocephalus-Information-Page