Chronic Hypervitaminosis A with Intracranial Hypertension and Low Cerebrospinal Fluid Concentration of Protein

Abstract

An unusually high dietary intake can lead to vitamin A intoxication. These two children had clinical and radiologic evidences of increased intracranial pressure and low cerebrospinal fluid protein concentrations. &dquo;... it made us rzlt sicke, specially three that were exceeding sicke, czrzcl we verily thought we should have lost them all, for all their skins came of from the foote to the head; but yet they recoitred again, fou zz~hich we gave Cod heartie thanks.&dquo; GERRIT DE VEER, 3 historian of an Arctic expedition in 1597, recorded this early account of the toxicity of polar bear liver., Not until this century was the high content of vitamin A found to be responsible for the toxic properties .2 Both acute and chronic vitamin A intoxication are now well documented syndromes. This paper describes two siblings who had chronic hypervitaminosis A which was manifested primarily by signs and symptoms of intracranial hypertension. Of particular interest in these cases were the radiologic features and the finding of low concentration of protein in the cerebrospinal fluid. Their vitamin A intoxication was induced by the administration of large amounts of vitamin by a health faddist mother. Case ReportsGase 1This 30-month-old boy was admitted to the YaleNew Haven Hospital because of anorexia, lethargy, stiff neck and inability to walk. These complaints were accompanied by increasing head size, patchy alopecia, and an exfoliative pruritic rash which had developed over a period of several weeks. His mother, a health faddist, had refused to have her children immunized and had been feeding them a variety of health foods and vitamins for about a year. Retrospectively the daily diet had included estimated minimums of at least 57,000 IU of vitamin A, 1,000 IU of vitamin D, 200 IU of vitamin E, 480 mg of vitamin C, 1,600 mg of calcium, and 750 mg of phosphate.The child appeared chronically ill. His height was 90 cm (90th percentile), weight 14.1 kg (50th percentile) and head circumference 54 cm (greater than 97th percentile). The pertinent physical findings included an obviously large head, stiff neck, slightly enlarged liver and spleen, and patches of alopecia. He refused to walk because of pain in both shins which were tender to palpation.Laboratory studies yielded normal readings for blood count, serum electrolytes, blood urea nitrogen and phosphate. Liver function results were normal except for a transaminase level of 104 units (normal 15-35). The serum calcium was initially 13.5 mg/100 ml but fell to 10.0 mg/100 ml after the calcium and vitamin D supplements were stopped. Blood carotene was normal at 98 pgmn/100 ml (normal '7Q-211U~. The serum vitamin A was markedly elevated at 520 ,ugm/l00 ml (norat UQ Library on March 15, 2015 cpj.sagepub.com Downloaded from

Cerebrospinal fluid absorption in primary hypoparathyroidism.

“…The rate of appearance Cerebrospinal fluid absorption in primary hypoparathyroidism has been established in normal subjects by Abbott and Alksne (1968) who found plasma values 24 hours after injection ranging from 41 to 60% (mean 48%) of the total cisternal dose. These values are similar to those obtained in dogs where the plasma level of 131I RISA increases steadily to a point of equilibrium 16 to 24 hours after injection (van Wart et al, 1960;Abbott and Alksne, 1968). In the patient studied, only 16% of the cisternally injected 1311 RISA had appeared in the plasma after 20 hours and equilibrium was not achieved until 72 hours.…”

“…Nine months after correction of his serum calcium the rate of appearance had increased to 53% with equilibrium at 20 hours. It has been suggested that the CSF volume is increased in patients with benign intracranial hypertension (Davidoff, 1956;Greer, 1968;Siegel and Spackman, 1972) but in this patient computed tomography did not show any detectable change in the ventricular size or prominence of the subarachnoid spaces after treatment. Whilc this does not rule out smaller changes in the size of the basal subarachnoid cisterns it certainly indicates that any change in volume was small and could not account for the marked increase in the rate of appearance of 1311 RISA in the plasma.…”

The neurobiology of retinoic acid in affective disorders

“…Multiple case reports found benign intracranial hypertension, or pseudotumor cerebri, characterized by headache, papilledema, and normal CT/MRI and CSF, in children and adults exposed to high levels of vitamin A (Alemayehu 1995;Gerber et al 1954;Gomber and Chellani 1996;Lombaert and Carton 1976;Marie and See 1954;Oliver and Havener 1958;Seigel and Spackman 1972;Selhorst et al 1984;Spector and Carlisle 1984). Selhorst (Selhorst et al 1984) reported that 5/50 patients with "idiopathic" pseudotumor cerebri were found by dietary history to be ingesting large amounts of Vitamin A in their diet.…”

Hypervitaminosis A as a complication of treatment for neuroblastoma

“…Increased intracranial pressure and splitting of the sutures has also been reported [4]. Radiographically, the most common manifestation is ulnar periostitis.…”