Psychiatric symptoms are present in most of the patients with idiopathic normal pressure hydrocephalus

Normal pressure hydrocephalus (NPH) is characterized by gait disturbance, dementia and/or urinary incontinence associated with dilation of ventricular system with normal opening cerebrospinal fluid pressure. Wide scientifical evidence confirms association between NPH and psychiatric symptoms. We selected 35 patients with idiopathic normal pressure hydrocephalus from January 2010 to January 2012 in a Brazilian tertiary hospital and performed a formal psychiatric evaluation to identify psychiatric disorders. Psychiatric disorders were present in 71% of these patients, especially anxiety, depression and psychotic syndromes. NPH patients may develop symptoms with frontal dominance, such as personality changes, anxiety, depression, psychotic syndromes, obsessive compulsive disorder, Othello syndrome; shoplifting and mania. Unusual appearances of NPH symptoms may hinder early diagnosis and consequently proper treatment.

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Lumbar cerebrospinal fluid pressure waves versus intracranial pressure waves in idiopathic normal pressure hydrocephalus

British Journal of Neurosurgery ◽

10.1080/02688690601047312 ◽

2006 ◽

Vol 20 (6) ◽

pp. 407-414 ◽

Cited By ~ 21

Author(s):

P. K. Eide ◽

A. Brean

Keyword(s):

Cerebrospinal Fluid ◽

Intracranial Pressure ◽

Normal Pressure Hydrocephalus ◽

Fluid Pressure ◽

Normal Pressure ◽

Idiopathic Normal Pressure Hydrocephalus ◽

Cerebrospinal Fluid Pressure ◽

Pressure Waves ◽

Lumbar Cerebrospinal Fluid

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Prevalence of C9ORF72 Expansion in a Large Series of Patients with Idiopathic Normal-Pressure Hydrocephalus

Dementia and Geriatric Cognitive Disorders ◽

10.1159/000497306 ◽

2019 ◽

Vol 47 (1-2) ◽

pp. 91-103 ◽

Cited By ~ 1

Author(s):

Ville E. Korhonen ◽

Anne M. Remes ◽

Seppo Helisalmi ◽

Tuomas Rauramaa ◽

Anna Sutela ◽

...

Keyword(s):

Normal Pressure Hydrocephalus ◽

Psychiatric Symptoms ◽

Age At Onset ◽

Normal Pressure ◽

Idiopathic Normal Pressure Hydrocephalus ◽

Study Cohort ◽

C9orf72 Expansion ◽

Personality Changes ◽

Clinically Significant ◽

Shunt Response

Background/Aims: The C9ORF72 expansion is known to cause frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). We aim to identify the prevalence of the C9ORF72 expansion in idiopathic normal pressure hydrocephalus (iNPH). Methods: We analysed the C9ORF72 expansion in a large cohort of patients with possible iNPH (n = 487) and cognitively intact elderly controls (n = 432; age > 65 years). Results: While the C9ORF72 expansion was detected in 1.6% (n = 8/487) of cases with possible iNPH, no control subject was found to carry the mutation. The mean age at onset of symptoms of C9ORF72 expansion carriers was 59 years (range: 52–67 years), 11 years less than non-carriers (p = 0.0002). The most frequent initial/main symptom pertained to gait difficulties. Despite identified mutation, only 3 of the patients fulfilled the criteria for the FTLD-ALS spectrum. Clinically significant shunt response was detected in 6 out of 7 shunted C9ORF72 expansion carriers. Conclusion: This is the first study cohort identifying the underlying C9ORF72 expansion in patients with iNPH providing evidence for the potential comorbidity between iNPH and the FTLD-ALS spectrum. Analysis of the C9ORF72 expansion should be considered for patients with probable iNPH presenting with frontal atrophy and personality changes or other severe psychiatric symptoms.

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P.007 Familial idiopathic normal pressure hydrocephalus in a Canadian family

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2018.109 ◽

2018 ◽

Vol 45 (s2) ◽

pp. S17-S17

Author(s):

BC Shettar ◽

S Mirsattari

Keyword(s):

Cognitive Impairment ◽

Normal Pressure Hydrocephalus ◽

Large Volume ◽

Fluid Pressure ◽

Cerebral Atrophy ◽

Normal Pressure ◽

Idiopathic Normal Pressure Hydrocephalus ◽

Opening Pressure ◽

Vp Shunt ◽

Surgery Patient

Background: Idiopathic Normal-pressure hydrocephalus (iNPH) is characterized by cognitive impairment, gait disturbance, enlarged ventricles with/without cerebral atrophy, with/without urinary incontinence, and normal cerebrospinal fluid pressure. Familial iNPH is very rarely described in the literature. A Canadian family with more than one generation of iNPH has never been described. Methods: Patient 1: 50-year-old female presented with wide-based and magnetic gait, multiple falls with subsequent freezing. LP with large volume tap was performed. Patient had ventriculo peritoneal (VP) shunt surgery. Patient 2: 52 year male (brother): Presented with long-standing cognitive impairment and fatigue. Montreal Cognitive Assessment (MOCA) was performed. Whole exome sequencing(WES) of both siblings as well as an unaffected first cousin was done. The father and grandmother of both patients was diagnosed with iNPH. Results: Patient 1: Opening pressure on LP was 22 cm-H2O. She responded well to large volume tap. She had VP shunt resulting in improved gait. Patient 2: Opening pressure on LP was 16cm-H2O. CSF flow study was slow for age indicative of NPH. MoCA score was 25/30. WES of patients and unaffected first cousin is underway. Conclusions: We present an undescribed Canadian family with iNPH in more than one generation. WES is underway for better understanding of genetic predesposition and inheritance of familial iNPH

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Programmable valve represents an efficient and safe tool in the treatment of idiopathic normal-pressure hydrocephalus patients

Arquivos de Neuro-Psiquiatria ◽

10.1590/0004-282x20130007 ◽

2013 ◽

Vol 71 (4) ◽

pp. 229-236 ◽

Cited By ~ 17

Author(s):

Matheus Fernandes de Oliveira ◽

Felippe Saad ◽

Rodolfo Casimiro Reis ◽

José Marcus Rotta ◽

Fernando Campos Gomes Pinto

Keyword(s):

Normal Pressure Hydrocephalus ◽

Standard Treatment ◽

Fluid Pressure ◽

Normal Pressure ◽

Idiopathic Normal Pressure Hydrocephalus ◽

Test Results ◽

Shunt Surgery ◽

Tap Test ◽

Programmable Valve ◽

Programmable Valves

Idiopathic normal pressure hydrocephalus (iNPH) is characterized by gait disturbance, dementia and /or urinary incontinence, dilation of the ventricular system and normal opening cerebrospinal fluid pressure. Shunt surgery is the standard treatment of iNHP. Diversions with programmable valves are recommended, once drainage pressure can be changed. However, well-defined protocols still lack guiding the steps to attain proper pressure for each patient. Methods: In our study, we reported the experience of shunting 24 patients with iNPH using Strata® (Medtronic) valve, following a protocol based on a positive Tap Test. Results: We observed clinical improvement in 20 patients and stability/worsening in 4 patients. Complications occurred in five patients, including one death. The results display improvement, and complications occurred at a lower rate than reported in other studies. Conclusions: The Strata® valve used in the proposed protocol represents an efficient and safe tool in the treatment of iNPH.

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