Benign Intracranial Hypertension: A case report and Review of Literature

摘要

This is a case report of benign intracranial hypertension. Her CT scan head was normal and cerebrospinal fluid (CSF) study at the time of admission only revealed high CSF pressure (47 cm of water). She improved with conservative management and papilledema was resolved at the time of discharge. A brief review of literature is also included. Introduction Benign intracranial hypertension was first described by Quincke in 1896, but the cause of this disorder continues to be a source of speculation. The prevalence of benign intracranial hypertension has been estimated to range from 1 to 19 cases per 100,000 population.1 Women are affected eight times more frequently than men.2 The pathophysiologic basis of benign intracranial hypertension remains unclear3 but is associated with the conditions such as aberrations in intracranial volume regulation, metabolic diseases, medication-associated abnormalities and miscellaneous disorders. Diagnosis is primarily clinical and requires radiographic exclusion of an intracranial mass and measurement of cerebrospinal fluid pressure. Treatment is directed at reducing intracranial pressure in idiopathic cases or correcting associated conditions. Case Report A 14 years old girl presented to National Institute of Neurological and Allied Sciences, Kathmandu with complains of headache and vomiting for 5 days. There was no history of fever, seizures, loss of consciousness. On examination, the child wass conscious and well oriented to time, place and person. Her vitals along with higher mental functions were normal and cranial nerves intact. Her systemic examinations were normal and no evidence of focal neurological deficit except bilateral papillidema. Haematology and biochemistry were within normal limits. CT scan head revealed no abnormality. Cerebrospinal fluid (CSF) study at the time of admission only revealed high CSF pressure (47 cm of water). She was kept in the hospital with the diagnosis benign intracranial hypertension and managed with acetazolamide and tapering dose of steroids. She improved with conservative management and papilledema was resolved at the time of discharge. Discussion Benign intracranial hypertension (BIH) also known as idiopathic intracranial hypertension (IIH) or pseudotumor cerebri, is a cause of progressive visual loss in children and young adults. It is a neurological disorder that is characterized by increased intracranial pressure , in the absence of a tumor or other diseases affecting the brain or its lining. The diagnosis of benign intracranial hypertension is in large part clinical, but radiologic and laboratory studies have a role in confirming the diagnosis. Pediatric ICP very often is found in association with an underlying causative factor, such as ear infection, dural sinus thrombosis, steroid withdrawal, malnutrition associated with refeeding, hypervitaminosis A, minocycline, and others.4 Headache is the most common symptom 5 and most frequently described as throbbing, episodic and without localization. It is exacerbated by the Valsalva maneuver and head movement and is most severe in the morning. Visual changes are a frequent clue to the diagnosis. The most frequently reported visual changes are episodic horizontal diplopia or tangential visual obscuration.6 Patients may report pulsatile tinnitus and pain in the shoulders, neck, back and arms. Finally, children may be entirely asymptomatic and present only with papilledema during a routine eye examination.7 Loss of visual acuity more commonly is a late finding in IIH. Uni- or bilateral sixth nerve palsy is frequent (40% to 48%) in children who have IIH, 8 and the incidence seems to be higher in children than in adults. The original criteria for IIH were described by the American neurosurgeon Walter E. Dandy in 1937.9 They were modified by Smith in 1985 to become the “modified Dandy criteria” 10 (Table:1).