Scholarlink

Background <p>Idiopathic normal pressure hydrocephalus (iNPH) is characterized by Hakim’s triad of gait disturbances, cognitive decline, and urinary incontinence. However, atypical presentations complicate diagnosis and remain poorly understood. This systematic review aimed to comprehensively analyze atypical symptoms of iNPH and their response to cerebrospinal fluid (CSF) diversion therapy.</p> Methods <p>Following PRISMA guidelines, a systematic search was conducted on PubMed using the keyword “normal pressure hydrocephalus symptoms.” Articles from 1914 to 2024 were screened. Inclusion criteria encompassed patients of both sexes diagnosed with iNPH presenting with symptoms beyond Hakim’s triad. While iNPH predominantly affects individuals older than 65 years, a strict age threshold was not applied as a primary exclusion criterion, given that several eligible studies did not systematically report patient age. Studies describing atypical symptoms appearing singularly or linked to one or two known symptoms were included.</p> Results <p>Of 3,814 initially retrieved articles, 27 studies met inclusion criteria. Neuropsychiatric manifestations were most frequent, including psychosis (6 studies), depression (6 studies), apathy (3 studies), aggressiveness (2 studies), and mania (2 studies). Neurological presentations included dysphagia (86% preoperatively), speech impairment (75%), tremor, palinopsia, and Parinaud’s syndrome. Autonomic symptoms included fecal incontinence (32% of cases). Post-shunt outcomes varied considerably: complete resolution rates ranged from 0% (apathy, aggressiveness) to 100% (palinopsia, systemic manifestations), highlighting symptom-specific therapeutic responsiveness.</p> Conclusions <p>Atypical symptoms in iNPH extend beyond the conventional triad, encompassing diverse neuropsychiatric, neurological, autonomic, and systemic manifestations. Recognition of these presentations is essential for early diagnosis and optimized therapeutic intervention. Variable response to CSF diversion suggests that while some symptoms reflect reversible hydrocephalic pathophysiology, others may indicate irreversible neural damage or concurrent pathology. Expanded clinical awareness and further research are needed to establish comprehensive diagnostic criteria.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Beyond Hakim’s triad: a systematic review of atypical clinical manifestations in idiopathic normal pressure hydrocephalus

  • 期刊论文

  • 发表日期：

    2026年4月6日

查看全文
  • Giuseppe Demichele,
  • Graziano Taddei,
  • Edvige Iaboni,
  • Mariasole Gagliano,
  • Alfredo De Vito,
  • Angelo Pompucci,
  • Gianpaolo Petrella

摘要

Background

Idiopathic normal pressure hydrocephalus (iNPH) is characterized by Hakim’s triad of gait disturbances, cognitive decline, and urinary incontinence. However, atypical presentations complicate diagnosis and remain poorly understood. This systematic review aimed to comprehensively analyze atypical symptoms of iNPH and their response to cerebrospinal fluid (CSF) diversion therapy.

Methods

Following PRISMA guidelines, a systematic search was conducted on PubMed using the keyword “normal pressure hydrocephalus symptoms.” Articles from 1914 to 2024 were screened. Inclusion criteria encompassed patients of both sexes diagnosed with iNPH presenting with symptoms beyond Hakim’s triad. While iNPH predominantly affects individuals older than 65 years, a strict age threshold was not applied as a primary exclusion criterion, given that several eligible studies did not systematically report patient age. Studies describing atypical symptoms appearing singularly or linked to one or two known symptoms were included.

Results

Of 3,814 initially retrieved articles, 27 studies met inclusion criteria. Neuropsychiatric manifestations were most frequent, including psychosis (6 studies), depression (6 studies), apathy (3 studies), aggressiveness (2 studies), and mania (2 studies). Neurological presentations included dysphagia (86% preoperatively), speech impairment (75%), tremor, palinopsia, and Parinaud’s syndrome. Autonomic symptoms included fecal incontinence (32% of cases). Post-shunt outcomes varied considerably: complete resolution rates ranged from 0% (apathy, aggressiveness) to 100% (palinopsia, systemic manifestations), highlighting symptom-specific therapeutic responsiveness.

Conclusions

Atypical symptoms in iNPH extend beyond the conventional triad, encompassing diverse neuropsychiatric, neurological, autonomic, and systemic manifestations. Recognition of these presentations is essential for early diagnosis and optimized therapeutic intervention. Variable response to CSF diversion suggests that while some symptoms reflect reversible hydrocephalic pathophysiology, others may indicate irreversible neural damage or concurrent pathology. Expanded clinical awareness and further research are needed to establish comprehensive diagnostic criteria.