Idiopathic normal pressure hydrocephalus (INPH) is a treatable cause of gait disturbance, cognitive impairment, and urinary incontinence… Not all INPH patients exhibit clinical improvement after shunting, and it is challenging to identify patients who are more likely to benefit from shunting.
…discrepancies in treatment outcomes are the result of controversies in 3 distinct but interrelated domains: the underlying pathophysiology of INPH, the diagnosis of INPH, and the identification of likely shunt-responders.
…shunting is a safe and effective means of achieving meaningful clinical improvement in most patients with INPH.
…Normal pressure hydrocephalus (NPH) was first reported in 1965 as a triad of dementia, gait disturbance, and urinary incontinence, with associated ventricular dilatation and normal cerebrospinal fluid (CSF) pressure.
…NPH can be classified as idiopathic NPH (INPH) or secondary NPH (SNPH), the latter of which occurs most commonly after subarachnoid hemorrhage, trauma, or meningitis.
…surgical outcomes in INPH have been consistently less successful than those in SNPH.
…the reported rates of postoperative clinical improvement in INPH patients have varied from 24% to 96%.
…INPH typically occurs during the seventh decade of life.
…the prevalence is from 0.41% to 1.4% for persons aged 65 years or older.
…To date, no clear pathophysiological mechanism for INPH has been established.
…CSF diversion can improve INPH symptoms
…In their cohort of 93 patients with INPH, 65% of the immediate surgery group demonstrated an improvement in modified Rankin scale of at least 1 point at 3 months compared with 5% in the postponed treatment group.
Cerebrospinal fluid diversion is a procedure that is used to drain fluid from the brain and spinal cord. A shunt is placed in a ventricle of the brain and threaded under the skin to another part of the body, usually the abdomen. It is used to treat hydrocephalus and idiopathic intracranial hypertension.
…Diagnosing INPH is difficult, and the accepted gold standard for diagnosis is clinical improvement after shunt surgery. This implies a circular argument: shunt-responsive INPH (SR-INPH) is defined by a clinical response to CSF diversion.
…INPH is characterized by a slow, short-stepped shuffling with a slightly broad base, reduced step-height, and associated gait freezing. …It may also include postural instability and difficulty turning. These gait disturbances are consistent with subcortical deficits involving the basal ganglia and frontal periventricular pathways.
Although INPH was originally reported as a reversible dementia, dementia is in fact the triad symptom least likely to improve postoperatively. The typical cognitive deficits of INPH are “subcortical,” with psychomotor retardation, apathy, difficulty in executive functions, and impaired recall memory yet relatively preserved recognition memory.
Urinary symptoms in INPH usually begin as increased frequency and urgency, only developing into incontinence in later stages. Urinary symptoms respond well to shunting but only predict a functional improvement in 31% to 33% of patients.
Despite classically being a triad of symptoms, INPH can be diagnosed in the presence of gait disturbance and 1 other cardinal symptom. This is in light of findings revealing that the complete triad often represents prolonged symptom duration, more advanced disease, and a poorer prognosis.
…Neuroimaging evidence of hydrocephalus on computerized tomography (CT) or magnetic resonance imaging (MRI) is essential for the diagnosis of INPH.
…Much of the diagnostic uncertainty surrounding INPH relates to difficulties distinguishing INPH from other diagnoses common in the elderly. It is also important to note that multiple comorbidities are common in the elderly and can mimic INPH…
…Programmable valves have now been adopted, as they permit noninvasive pressure adjustments that can optimize clinical improvement and ameliorate drainage-related complications.