normal pressure hydrocephalus (NPH)
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Etiology
- idiopathic 50%
- prior subarachnoid hemorrhage, meningitis, trauma or intracranial surgery[4]
Epidemiology
Pathology
- failure of CSF absorption by the pacchonian granulations
Clinical manifestations
- progressive dementia less prominent than gait disorder
- cognitive slowing, forgetfulness
- agnosia, aphasia typically absent
- unsteady gait (initial symptoms)
- difficulty initiating gait "magnetic feet" (as if the patient's feet were stuck to the floor)
- repeated falls
- parkinsonian features:
- short, shuffling steps
- stooped posture
- urinary incontinence (proximate to unsteady gait)[2]
- presentation may be complicated by comorbidities (case presentation)[10]
Laboratory
- lumbar puncture (CSF analysis)
- generally normal CSF pressure
- gait, incontinence & cognition may improve with large volume (> 20 mL) CSF removal
- improvement of symptoms with CSF removal may predict response to VP shunt
- 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]
Complications
Management
- ventriculo-peritoneal (VP) shunt
- unproven benefit[2]
- must be response to high-volume CSF removal or lumbar drainage[2]
- patients with clear cause of hydrocephalus most likely to benefit[2]
- gait & incontinence more likely to improve than cognition[3][13]*
- better outcomes with early shunting[4]
- long-term outcomes of VP shunting are not influenced by preoperative comorbidities, age, or responses to CSF samplings
- ventricular shunt with an externally controlled programmable valve that allows regulation of cerebrospinal fluid pressure[2]
* videos showing gait improvement[7]
More general terms
References
- ↑ Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
- ↑ Jump up to: 2.0 2.1 2.2 2.3 2.4 2.5 2.6 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 - ↑ Jump up to: 3.0 3.1 UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
Andren K, Wikkelso C, Hellstrom P, Tullberg M, Jaraj D. Early shunt surgery improves survival in idiopathic normal pressure hydrocephalus. Eur J Neurol. 2021 Apr;28(4):1153-1159. PMID: https://www.ncbi.nlm.nih.gov/pubmed/33316127 PMCID: PMC7986742 Free PMC article. https://onlinelibrary.wiley.com/doi/10.1111/ene.14671 - ↑ Jump up to: 4.0 4.1 4.2 4.3 4.4 Ferri's Clinical Advisor, Instant Diagnosis and Treatment, Ferri FF (ed), Mosby, Philadelphia, 2003, pg 420
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/20512347
- ↑ Williams MA, Relkin NR. Diagnosis and management of idiopathic normal-pressure hydrocephalus. Neurol Clin Pract. 2013 Oct;3(5):375-385. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24175154
- ↑ Jump up to: 7.0 7.1 7.2 AbdelRazek MA, Venna N Ventriculoperitoneal-Shunt Placement for Normal-Pressure Hydrocephalus. N Engl J Med 2017; 377:e35. Dec 28, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/29281574 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1701226
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/26644048 Free PMC Article
- ↑ Jump up to: 9.0 9.1 Muller-Schmitz K et al. Normal pressure hydrocephalus associated with Alzheimer's disease. Ann Neurol 2020 Oct; 88:703 PMID: https://www.ncbi.nlm.nih.gov/pubmed/32662116 https://onlinelibrary.wiley.com/doi/10.1002/ana.25847
- ↑ Jump up to: 10.0 10.1 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: https://www.ncbi.nlm.nih.gov/pubmed/33826823 https://www.nejm.org/doi/full/10.1056/NEJMcpc2027090
- ↑ NINDS Normal Pressure Hydrocephalus Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Normal-Pressure-Hydrocephalus-Information-Page
- ↑ Jump up to: 12.0 12.1 12.2 Bianconi A, Colonna S, Minardi M, et al. Prognostic Factors in Idiopathic Normal Pressure Hydrocephalus Patients After Ventriculo-Peritoneal Shunt: Results From a Single-Institution Observational Cohort Study With Long Term Follow-Up. World Neurosurgery. 2024;187:e1089-e1096. PMID: https://www.ncbi.nlm.nih.gov/pubmed/38759789 https://www.sciencedirect.com/science/article/abs/pii/S1878875024008234
- ↑ Jump up to: 13.0 13.1 Ghosh S, Lippa C. Diagnosis and prognosis in idiopathic normal pressure hydrocephalus. Am J Alzheimers Dis Other Demen. 2014 Nov;29(7):583-9. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24550545 PMCID: PMC10852782 Free PMC article. Review. https://journals.sagepub.com/doi/10.1177/1533317514523485
- ↑ Hallqvist C, Gronstedt H, Arvidsson L. Gait, falls, cognitive function, and health-related quality of life after shunt- treated idiopathic normal pressure hydrocephalus-a single-center study. Acta Neurochir (Wien). 2022 Sep;164(9):2367-2373. PMID: https://www.ncbi.nlm.nih.gov/pubmed/35831723 PMCID: PMC9427897 Free PMC article. https://link.springer.com/article/10.1007/s00701-022-05309-4
Patient information
normal pressure hydrocephalus patient information