Normal Pressure Hydrocephalus

Original Editor - David Olukayode

Top Contributors - David Olukayode

Introduction[edit | edit source]

Normal Pressure Hydrocephalus (NPH) occurs when cerebrospinal fluid accumulates due to obstructed flow or excess production, resulting in gait and memory problems as well as urinary incontinence.[1] These classic triad of symptoms (gait disturbance, urinary incontinence, and dementia) were first described by Hakim and Adams. The mnemonic "wet, wobbly and wacky" was also given to these symptoms [2]To consider the diagnosis, gait disturbance plus one other characteristic is usually required.[3]

Etiology[edit | edit source]

NPH is of two types:

  • Idiopathic or primary NPH (without any identifiable cause)
  • Symptomatic or secondary NPH (caused by risk factors like traumatic brain injury, brain infection, subarachnoid haemorrhage, tumor, or radiation contributing to hydrocephalus) [4][1]

Both idiopathic NPH and secondary NPH share the fact that they are communicating types of hydrocephalus with similar prognoses. The key distinction between them is that secondary NPH affects people of all ages, whereas idiopathic NPH is mostly a disease of the elderly.[4]Since the causes of secondary NPH has been made clear, this article will focus of idiopathic NPH.

Epidemiology[edit | edit source]

The most extensive population-based investigation on the prevalence of idiopathic NPH in Western Sweden reported that 0.2% of people aged 70 to 79 years and 5.9% of those aged 80 and older met guideline criteria for probable idiopathic NPH. The majority of idiopathic NPH patients were over the age of 80.[5]Men and women are equally afflicted, and the average age of onset is around 70 years. It is estimated that idiopathic NPH accounts for 6% of all dementia cases. The estimated prevalence of normal pressure hydrocephalus is reported to be 0.003% in people under the age of 65 and 0.2% to 2.9% in people 65 and older. The estimated incidence of normal pressure hydrocephalus is 0.2 to 5.5 per 100000 person-years.[6][7][8]

Pathogenesis[edit | edit source]

The actual pathogenesis process of idiopathic NPH remains cloudy.

Resources[edit | edit source]

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References[edit | edit source]

  1. 1.0 1.1 Patel S, Ditamo M, Mangal R, Gould M, Ganti L. Normal Pressure Hydrocephalus. Cureus. 2023 Feb 18;15(2):e35131. doi: 10.7759/cureus.35131. PMID: 36949988; PMCID: PMC10026533.
  2. Hakim S, Adams RD. The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci. 1965;2:307–327.
  3. Shprecher D, Schwalb J, Kurlan R. Normal pressure hydrocephalus: diagnosis and treatment. Curr Neurol Neurosci Rep. 2008 Sep;8(5):371-6. doi: 10.1007/s11910-008-0058-2. PMID: 18713572; PMCID: PMC2674287.
  4. 4.0 4.1 M Das J, Biagioni MC. Normal Pressure Hydrocephalus. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-.
  5. Jaraj D, Rabiei K, Marlow T, Jensen C, Skoog I, Wikkelsø C. Prevalence of idiopathic normal-pressure hydrocephalus. Neurology. 2014 Apr 22;82(16):1449-54.
  6. Brean A, Fredø HL, Sollid S, Müller T, Sundstrøm T, Eide PK. Five-year incidence of surgery for idiopathic normal pressure hydrocephalus in Norway. Acta Neurol Scand. 2009 Nov;120(5):314-6.
  7. Tanaka N, Yamaguchi S, Ishikawa H, Ishii H, Meguro K. Prevalence of possible idiopathic normal-pressure hydrocephalus in Japan: the Osaki-Tajiri project. Neuroepidemiology. 2009;32(3):171-5.
  8. Krauss JK, Halve B. Normal pressure hydrocephalus: survey on contemporary diagnostic algorithms and therapeutic decision-making in clinical practice. Acta Neurochir (Wien). 2004 Apr;146(4):379-88; discussion 388.