Benign intracranial hypertension

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Benign intracranial hypertension (pseudotumour cerebri) is a term applied to patients with raised intracranial pressure and no evidence of any 'mass' lesion or of hydrocephalus. AETIOLOGY: This condition is related to a variety of clinical disorders:

Most demonstrate a direct causal link -venous outflow obstruction to csf absorption

„ Sagittal sinus thrombosis

Lateral sinus thrombosis usually secondary to mastoiditis

Following neck operation —

Congestive cardiac failure

Lateral sinus thrombosis usually secondary to mastoiditis

Intrathoracic mass lesion

CLINICAL FEATURES

Age: any age, but usually in 3rd and 4th decades. Sex: female > male - especially in the idiopathic group.

In the minority, the causal link remains obscure, but a variety of factors are associated -diet - obesity.

- hyper/hypovitaminosis A. endocrine - pregnancy, menarche, menstrual irregularities, Addison's disease. haematological - iron deficiency anaemia.

- polycythaemia vera. drugs - oral contraceptives.

- steroid withdrawal.

- tetracycline.

- nalidixic acid.

Various mechanisms have been postulated.

- brain swelling j Different studies support

- J. csf absorption I different mechanisms. The

- t csf secretion J link with obesity suggests an underlying endocrine basis, but, except in Addison's disease, endocrine assessment has failed to reveal abnormalities.

Symptoms Signs

Headache Obesity

Visual obscurations 1 Impaired visual acuity J ~ Papilledema N Diplopia VI nerve palsy

In women the condition is often associated with - recent weight gain, fluid retention, menstrual dysfunction, the first trimester of pregnancy and the postpartum period.

Investigations

CT scan - ventricles usually small. Visual field charting -K J Enlarged blind spot (often used to monitor \ Peripheral field constriction. progress). Lumbar puncture and pressure measurement. ICP monitoring - if diagnostic doubt persists. MR Venogram - will identify a sinus thrombosis (see page 41)

TREATMENT

Treat the underlying cause if known. Weight reduction diet.

Drugs - acetazolamide (reduces CSF production).

- thiazide diuretics. If above fail -* lumboperitoneal shunt.

PROGNOSIS

Most patients respond rapidly to short-term treatment, but up to one-third develop recurrent attacks. In 10% visual impairment persists.

364 Optic nerve sheath fenestration - if progressive impairment of visual acuity despite treatment.

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