Papilledema Gone Wrong (video)

She underwent brain biopsy of the frontal lobe, which revealed non-specific reactive astrocytosis. Dermatology was consulted for areas of skin thickening and discoloration, which were felt to be non-specific thin hyperpigmented, lichenified plaques on her ankles, knees and elbows bilaterally suggestive of retention hyperkeratosis. She was treated with IVIg, steroids, and plasmapheresis for a presumed autoimmune process, but she did not demonstrate improvement.. With directed questioning, the neurology team elicited a history of significant chronic mothball inhalation exposure describing use of multiple mothballs as air fresheners. Serum paradicholorobenzene level was 2800 ug/L with the asymptomatic mean reported to be 2.1 ug/L. Urine dichlorophenol levels were 200,000ug/L with asymptomatic mean reported at 200ug/L. The progressive neurologic decline associated with periventricular white matter hyperintensities on MRI, is consistent with paradichlorobenzene (PDCB) toxicity2. Fifteen cases have been reported in the literature. Additionally, as with our patient, it is not uncommon to have an ichthyosis-like dermatosis early in the presentation.1,2 In retrospect, normocytic anemia and mildly elevated transaminases were also present since admission, findings consistent with systemic effects of PDCB toxicity.3 Her elevated serum paradicholorobenzene and urine dichlorophenol (DCP) levels confirmed the diagnosis. This rare presentation of PDCB toxicity consistent initially with pseudotumor cerebri syndrome/IIH but later characterized by encephalopathy and white matter changes has been reported only once before. This is the first pediatric case presented.