Challenges in Diagnosing Normal Pressure Hydrocephalus: Complicating Factors in a Third-World Country
Ana Gomes1, Sephora Almeida1, Diogo Haddad Santos2, Yngrid Ferreira1, Alexandre Shoji1
1Irmandade da Santa Casa de Misericórdia de São Paulo, 2Moema
Objective:

The Centro de Cognição Paulista (CECOP) has seen a rise in referrals for suspected NPH. This study, covering patients evaluated from January 2022 to December 2023, explores their final diagnoses and the challenges in diagnosing NPH. The goal is to identify factors leading to misdiagnosis and highlight the need for thorough evaluations to improve diagnostic accuracy.

Background:

Normal Pressure Hydrocephalus (NPH), described in 1965, is marked by cognitive impairment, gait apraxia, and urinary incontinence, with ventriculomegaly on neuroimaging and normal cerebrospinal fluid pressure. Diagnosing NPH is difficult due to the incomplete presentation of its triad and symptom overlap with other neurodegenerative diseases. In third-world countries, diagnosis is further complicated by limited healthcare access, reliance on advanced imaging, and a high prevalence of comorbidities, leading to delayed diagnosis.

Design/Methods:

We retrospectively analyzed 46 patients referred to the CECOP with suspected NPH. Patients were evaluated for imaging findings, age, gender, presence of NPH-compatible symptoms, and their final diagnosis.

Results:

Among 46 patients, 25 exhibited at least one symptom of Hakim’s triad but had no CT or MRI evidence of NPH. They were diagnosed with other conditions: 14 with Alzheimer’s disease, 5 with vascular dementia, 3 with frontotemporal dementia, and 3 remain under investigation. Only 7 patients had both clinical and imaging findings consistent with NPH and were referred for a tap test. Another 7 showed signs of NPH but were diagnosed with Alzheimer’s disease (4) or vascular dementia (3).

Conclusions:

Our analysis showed an overestimation of the diagnosis, with 15.22% having both clinical and imaging findings consistent with NPH. Despite neuroimaging abnormalities, 54.3% had alternative diagnoses. Biomarkers and the Radscale score may improve accuracy. Diagnostic errors often stem from clinical overestimation and imaging misinterpretation, especially in Brazil, where access to healthcare is limited.


10.1212/WNL.0000000000212127
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