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. Can be idiopathic with genetic
Hakim Syndrome; Normal-Pressure Hydrocephalus;
Normotensive Hydrocephalus; Occult Hydrocephalus.
Syndrome characterized by the presence of slowly
developing normal-pressure hydrocephalus. Hydrocephalus was first described
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.
Not defined in the literature.
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.
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.
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
Assessment of general considerations
in the premature neonate. Assessment of previous medical or surgical
management; if the hydrocephalus developed slowly, it may have been treated
conservatively as many spontaneously resolve with time. Other therapies,
such as use of drugs to reduce formation of CSF or repeated lumbar punctures
to remove CSF, may have been given. ...