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At a glance

Syndrome characterized (in adults) by the clinical triad of dementia (memory loss, bradyphrenia), gait ataxia (mimicking Parkinson disease), and urinary incontinence, caused by progressive hydrocephalus (without increase in CSF pressure) following previous trauma, subarachnoid hemorrhage, neoplasm, and infection. Children may present with at least two elements of the triad. This syndrome can be idiopathic with genetic predisposition.

Synonyms

Hakim Syndrome; Normal-Pressure Hydrocephalus; Normotensive Hydrocephalus; Occult Hydrocephalus.

History

Syndrome characterized by the presence of slowly developing normal-pressure hydrocephalus. Hydrocephalus was first described by Hippocrates.

Incidence

Internationally, the estimate in the general population is 1:25,000 cases and as many as 6% of patients affected with dementia. No gender proclivity. Increasing in the premature neonatal population as a result of periventricular/intraventricular hemorrhage. The likelihood of developing hydrocephalus is related to the severity of hemorrhagic lesions. The most common progression is to normal pressure hydrocephalus.

Genetic inheritance

Not defined in the literature.

Pathophysiology

Following intraventricular/periventricular bleed in the newborn, the condition may result from obliterative arachnoiditis affecting mainly the posterior fossa or, less commonly, obstruction of cerebrospinal fluid (CSF) flow within the ventricular system by clots or other debris. The precise relationship between this and the genesis of brain injury in the newborn is unknown. In older children, there is usually clearly identifiable etiology or previous shunt surgery. Clinical symptoms result from stretching of the corona radiata by the distended ventricles. Dementia is attributed to distension of the fornix by the third ventricle, and incontinence is attributed to the involvement of sacral motor fibers. There seems to be an association between Hakim Syndrome and either generalized vascular disease or deep white matter infarctions in adults.

Diagnosis

Usually clinical, initially with progressive dilation of the ventricles that may take days to weeks to reach the classic signs of hydrocephalus (head enlargement in infants, sun-setting eyes, irritability, lethargy, nausea, vomiting, weakness, cognitive difficulties, incontinence, headache). The older child may show signs of subtle psychomotor deterioration. Diagnosis of hydrocephalus is confirmed by ultrasonography through the anterior fontanelle (in infants) and by computerized tomography or magnetic resonance imaging when the fontanelle is closed. Nuclear medicine cisternogram reveals absent (or delayed) passage of tracer over convexities. Most patients undergo ventriculoperitoneal shunt derivation without prior assessment of CSF pressure, so the term chronic hydrocephalus has been proposed as an alternative title.

Clinical aspects

Neurologic examination usually reveals the presence of normal-pressure hydrocephalus, such as bulging fontanelle and inappropriate increase in head circumference. Most children also have at least two elements of the adult triad, consisting of psychomotor retardation, gait disturbance psychotic-like behavior, mild dementia, and urinary or fecal incontinence. The presence of gait instability, urinary incontinence, and slight cognitive impairment constitute ...

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