Cerebrospinal Fluid Pressure Studies in Normal Pressure Hydrocephalus and Cerebral Atrophy

1976 ◽  
Vol 14 (2) ◽  
pp. 119-128 ◽  
Author(s):  
C. Di Rocco ◽  
G. Maira ◽  
G.F. Rossi ◽  
A. Vignati
1984 ◽  
Vol 21 (2) ◽  
pp. 195-203 ◽  
Author(s):  
John S. Meyer ◽  
Hisao Tachibana ◽  
Jeffrey P. Hardenberg ◽  
Richard E. Dowell ◽  
Yasuhisa Kitagawa ◽  
...  

Author(s):  
M. D. Cusimano ◽  
D. Rewilak ◽  
D. T. Stuss ◽  
J. C. Barrera-Martinez ◽  
F. Salehi ◽  
...  

Background:Normal-pressure hydrocephalus (NPH) is characterized by gait disturbance, cognitive impairment, with or without urinary incontinence, enlarged ventricles with or without cerebral atrophy and normal cerebrospinal fluid pressure.Methods:We report two sisters with NPH who lived together their entire lives and whose natural history might provide insights into genetic and environmental mechanisms underlying this disorder. Both patients were in their early seventies, single, had similar daily habits and hypertension. No other family members had NPH.Results:They both underwent shunt placement and showed improvement documented by history and neuropsychological assessment. Both showed a delayed deterioration due to vasculopathy. Both patients were homozygous for the apolipoprotein E (ApoE) e3 allele on chromosome 19. No environmental factors that might have influenced the development of NPH were identified.Conclusion:Our report of two sisters with NPH may indicate the presence of genetic predisposition and further studies involving genetics and environmental factors are necessary to elucidate their role in the pathogenesis of NPH.


2015 ◽  
Vol 55 (8) ◽  
pp. 657-662 ◽  
Author(s):  
Naokazu HAYASHI ◽  
Mitsunori MATSUMAE ◽  
Satoshi YATSUSHIRO ◽  
Akihiro HIRAYAMA ◽  
Afnizanfaizal ABDULLAH ◽  
...  

Author(s):  
BC Shettar ◽  
S Mirsattari

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


2021 ◽  
pp. 096777202110540
Author(s):  
Daniel Jaramillo-Velásquez ◽  
Fernando Hakim ◽  
Andreas K Demetriades

Salomón Hakim (1922–2011) was a Colombian neurosurgeon and brain scientist This biography examines the social and cultural background through which he emerged as an inquisitive and multi-dimensional surgeon-scientist, and his lifelong contributions to the specialty of neurosurgery. With empirical knowledge in applied medical physics, electronics, electricity and chemistry, he understood the paradoxical phenomenon of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. This ultimately led Hakim to describe in exquisite detail the physics of the cranial cavity and brain hydrodynamics. His name is intertwined with the identification of the entity of a syndrome which had not previously been addressed in the medical literature: Normal Pressure Hydrocephalus (Hakim's syndrome). Additionally, he designed and built various models of valved shunting devices to treat the condition (eg the Hakim programmable valve). Through his selflessness and cogent work, Hakim left a legacy and intellectual heritage that has allowed many colleagues worldwide to save thousands of lives who would be otherwise condemned to oblivion.


Author(s):  
C. M. Fisher

Abstract:Background:The pathogenesis of symptoms in normal pressure hydrocephalus (NPH) is unclear. One theory is that in the presence of enlarged lateral ventricles, the ventricular fluid exerts increased force on the ventricular walls, the cerebrospinal fluid pressure remaining normal. This is in accordance with Pascal’s principle of F = PxA. It has not been possible to obtain direct evidence for this view.Method:The opportunity to gain insight into the matter arose when it was observed that two patients with symptomatic NPH were relieved of their symptoms when they developed bilateral subdural collections, one after head trauma, the other after ventriculo-peritoneal shunting for NPH.Result:In each case, concomitant with the subdural collections and the reversal of symptoms, the ventricular system became smaller. In the first patient, symptoms returned when the subdural collections were resorbed. In the second patient, symptoms returned when the subdural collections became excessive.Conclusion:Although documentation was less thorough than desired, it was concluded that relief of symptoms was related to the decrease in the ventricular size rather than lowering of the intraventricular pressure, thus providing evidence for operation of the principle F = PxA as the mechanism of symptoms in NPH.


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