normal pressure hydrocephalus 정상압 뇌수종 이란 무엇인가

정상압 뇌수종.

 

흔한 질병은 아니다.  대학교 ENT 실습나가서 한번 봤고 

2주전에 2번째로 보게 되었다. 1년정도 거동이 불편해지고 인지 저하가 오면서 동시에 소변을 가리지 못하게 되었다. 결국 정상압 뇌수종진단을 받고, neurosurg 에 입원해서 VP shunt 수술을 받고 , 안정되자 geri병동으로 이송됐다.

2주정도의 재활후 거동은 좀 나아졌고, short term memory loss 는 있고 delirium 이 아직 있지만, attention 도 좋아지고 좀더 orientation 이 나아졌다. 가족들도 집에서 돌보길 원해서 nursing home 이 아닌 재활 병원으로 이송했다. 

 

 

Definition

-A condition of pathologically enlarged ventricular size with normal opening pressures on lumbar puncture. 

-a form of communicating hydrocephalus 

-is associated with a classic triad of dementia, gait disturbance, and urinary incontinence

-is potentially reversible by the placement of a ventriculoperitoneal (VP) shunt - > it is important to recognize and diagnose accurately

 

Epidemiology 

-The incidence of NPH has varied in different studies from 2 to 20 per million per year

- idiopathic NPH increases in prevalence with age and is most common in adults over the age of 60 years 

 

Pathophysiology

-Cerebrospinal fluid (CSF) is produced by the choroid plexus in the lateral ventricles and flows from the lateral ventricles to the third and fourth ventricles, and then through the basal cisterns, tentorium, and subarachnoid space over the cerebral convexities to the area of the sagittal sinus. CSF is absorbed into the systemic circulation primarily across the arachnoid villi into the venous channels of the sagittal sinus.

 

NPH is classically described as having three cardinal features: gait difficulty, cognitive disturbance, and urinary incontinence [26]. These manifestations are believed to arise from dysfunction of supplementary motor areas of the frontal lobe and periventricular white matter tracts, particularly those subserving frontal lobe connections

 

Clinical features 

-three cardinal features: gait difficulty, cognitive disturbance/impairment, and urinary incontinence

    >These manifestations are believed to arise from dysfunction of supplementary motor areas of the frontal lobe and periventricular white matter tracts, particularly those subserving frontal lobe connections

   >gait must be the predominant problem

1) Gait dysfunction 

-a magnetic or "glue-footed" gait, gait apraxia, or a frontal ataxia

-Patients with NPH move slowly and take small steps, often with a wide base

-Long tract signs may be observed, with lower-extremity spasticity, hyperreflexia, and extensor plantar responses. In late stages, frontal release signs, akinetic mutism, and quadriparesis may occur.

2)Cognitive impairment

-The cognitive disturbance of NPH evolves over months to years and usually develops after the onset of gait dysfunction. Patients typically have both subcortical and frontal features, including:

  >Psychomotor slowing

  >Decreased attention and concentration

  >Impaired executive function

  >Apathy

3)Urinary incontinence

-Urinary urgency rather than incontinence may be present at early stages. Also, the gait disorder of NPH delays the patient reaching the bathroom in time.

-In later stages, urinary incontinence is accompanied by a lack of concern, reflecting its probable origin in frontal lobe impairment.

Notable negatives — By definition, patients with NPH have a normal opening pressure at the lumbar cistern. The clinical presentation is therefore notable for an absence of signs and symptoms related to diffusely increased intracranial pressure (ICP), such as:

  >Headaches

  >Nausea and vomiting

  >Visual loss

  >Papilledema

 

Differential diagnosis 

-Dementia with Lewy bodies 

-Parkinson disease dementia 

-Progressive supranuclear palsy 

-Multiple system atrophy 

-Corticobasal syndrome 

-Alzheimer disease 

-Cerebrovascular disease 

 

Diagnostic evaluation 

-cognitive evaluation 

   >Vit B12 , TFT 

   >formal neuropsychological assessment 

-Magnetic resonance imaging 

   >MRI is superior to CT in the evaluation of patients with possible NPH because it allows visualization of other markers of NPH and provides additional information that can exclude other potential etiologies in the differential diagnosis.

-Ventriculomegaly 

    > The hallmark finding of NPH on CT or MRI is ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement, with no evidence of obstruction at the level of the third or fourth ventricles.

-Confirmatory tests 

  >high-volume lumbar puncture 

     : 30-50ml of CSF is removed with documentation of the patient's gait function before and within 30 to 60 minutes after the procedure. Common parameters measured before and after CSF removal include measures of gait speed, stride length, and number of steps it takes to turn 180 or 360 degrees.

 

Ventricular shunting 

-The treatment for NPH is an implanted ventricular shunt.

-Most shunts divert cerebrospinal fluid (CSF) from a catheter in the lateral ventricle into the abdomen (ventriculoperitoneal [VP]) or, less commonly, into the heart (ventriculoatrial). 

 

Complications 

-Intracranial infection 

-Seizures 

-Intracerebral haemorrhage from catheter placement 

-Mechanical shunt failures or blocked shunts 

-Abdominal injury (ascitities, peritonitis, abdominal perforations, volvulus) in VP shunts 

-Arrhythmias from incorrect distal catheter placement, systemic emboli in ventriculoatrial shunts