Case report of idiopathic normal pressure hydrocephalus: a challenging diagnosis
DOI:
https://doi.org/10.2340/jrmcc.v6.11631Keywords:
idiopathic normal pressure hydrocephalus, ventriculomegaly, triad, gait impairments, gait ataxia, urinary incontinence, cognitive impairment, ventriculoperitoneal shunt, delay in diagnosis, missed diagnosisAbstract
Introduction: This report concerns the case of a 70-year-old man with idiopathic normal pressure hydrocephalus (iNPH). The diagnosis in the current case took more than 2 years. iNPH is characterised by ventriculomegaly with a known triad of symptoms: gait disturbance, cognitive impairments and urinary incontinence. Although this is a difficult diagnosis and other conditions must be ruled out, several points in the process could lead to a correct diagnosis. The aim of the report is to identify several reasons why the diagnosis was delayed for such a long time, as well as lessons for the future.
Case: This patient developed several symptoms over time. First, he presented with depressive mood and altered behaviour. He later developed gait difficulties and, finally, urinary incontinence. Multiple consultations and examinations failed to provide an exact explanation for all his symptoms. After 2 years, a new doctor at the hospital started from scratch and recognised the iNPH triad, and the diagnosis was confirmed by the radiologist.
Conclusion: The diagnosis of iNPH is difficult, as symptoms may manifest over time. In this case, the delay of diagnosis exceeded estimations. A broader view through interdisciplinary consultation could provide new insights and lead to earlier diagnosis.
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References
Williams MA, Malm J. Diagnosis and treatment of idiopathic normal pressure hydrocephalus. Continuum (Minneap Minn) 2016; 22(2 Dementia): 579-599.
https://doi.org/10.1212/CON.0000000000000305 DOI: https://doi.org/10.1212/CON.0000000000000305
Factora R. When do common symptoms indicate normal pressure hydrocephalus? Cleve Clin J Med 2006; 73: 447-456.
https://doi.org/10.3949/ccjm.73.5.447 DOI: https://doi.org/10.3949/ccjm.73.5.447
Wang Z, Zhang Y, Hu F, Ding J, Wang X. Pathogenesis and pathophysiology of idiopathic normal pressure hydrocephalus. CNS Neurosci Ther 2020; 26: 1230-1240.
https://doi.org/10.1111/cns.13526 DOI: https://doi.org/10.1111/cns.13526
Gavrilov GV, Gaydar BV, Svistov DV, Korovin AE, Samarcev IN, Churilov LP, et al. Idiopathic normal pressure hydrocephalus (Hakim-Adams Syndrome): clinical symptoms, diagnosis and treatment. Psychiatr Danub 2019; 31: 737-744.
Nakajima M, Yamada S, Miyajima M, Ishii K, Kuriyama N, Kazui H, et al. Research committee of idiopathic normal pressure hydrocephalus. Guidelines for Management of Idiopathic Normal Pressure Hydrocephalus (Third Edition): Endorsed by the Japanese Society of Normal Pressure Hydrocephalus. Neurol Med Chir (Tokyo) 2021; 61: 63-97.
https://doi.org/10.2176/nmc.st.2020-0292 DOI: https://doi.org/10.2176/nmc.st.2020-0292
Isaacs A, Williams M, Hamilton M. Current update on treatment strategies for idiopathic normal pressure hydrocephalus. Curr Treat Options Neurol 2019; 21: 65.
https://doi.org/10.1007/s11940-019-0604-z DOI: https://doi.org/10.1007/s11940-019-0604-z
Miyajima M, Kazui H, Mori E, Ishikawa M. on behalf of the SINPHONI-2 Investigators. One-year outcome in patients with idiopathic normal-pressure hydrocephalus: comparison of lumboperitoneal shunt to ventriculoperitoneal shunt. J Neurosurg 2016; 125: 1483-1492.
https://doi.org/10.3171/2015.10.JNS151894 DOI: https://doi.org/10.3171/2015.10.JNS151894
Nikaido Y, Urakami H, Okada Y, Akisue T, Kawami Y, Ishida N, et al. Rehabilitation effects in idiopathic normal pressure hydrocephalus: a randomized controlled trial. J Neurol 2023; 270: 357-368.
https://doi.org/10.1007/s00415-022-11362-x DOI: https://doi.org/10.1007/s00415-022-11362-x
Rydja J, Kollén L, Hellström P, Owen K, Nilsson AL, Wikkelsø C, et al. Physical exercise and goal attainment after shunt surgery in idiopathic normal pressure hydrocephalus: a randomised clinical trial. Fluids Barriers CNS 2021; 18: 51.
https://doi.org/10.1186/s12987-021-00287-8 DOI: https://doi.org/10.1186/s12987-021-00287-8
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