Hakim-Adams Syndrome

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 predisposition.

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

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

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 incontinence.

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. If so, these treatments, plus complications such as the presence of infection, must be considered. Also assess for other abnormalities, especially Arnold-Chiari malformation.

Intracranial pressure by definition should be normal. However, a patient presenting for insertion of a ventriculoperitoneal shunt is usually in a worsening state, and general measures for increased intracranial pressure may be indicated, including use of cricoid pressure during laryngoscopy for tracheal intubation after a modified rapid-sequence induction and use of mild hypocapnia. Patients affected with psychotic-like behavior may present a significant challenge at induction of anesthesia. The use of sedative medication should be balanced against the risk of slight hypoventilation in the presence of raised intracranial pressure.

Tracheal intubation following succinylcholine or rocuronium is preferable if progressive hydrocephalus has been confirmed.

Syndromes with Hydrocephalus: Aqueductal stenosis (can be inherited as an X-linked trait; gene map location is Xq28), myelomeningocele, Arnold-Chiari malformation, intraventricular hemorrhage, meningitis, tumor (including X-linked hydrocephalus).

Dandy-Walker Malformation: Congenital hydrocephalus syndrome caused by blockage of foramina of the fourth ventricle. This condition is associated with agenesis of the cerebellar vermis.