Respiratory insufficiency associated with acute hepatic porphyria.

We reported two patients with acute hepatic porphyria with acute respiratory failure. The acute porphyrias are characterized biochemically by increased excretion of porphyrins and porphyrin precursors, ALA and PBG. It has been shown to be due to partial enzyme blockage along the haeme biosynthetic pathway which results in secondary increased ALA synthetase activity. They are characterized clinically by episodes of acute neurological involvement. Neurological manifestations could be related to either a decrease in essential haemeproteins, or to a toxic effect of ALA and PBG. The first patient illustrated a diagnosis of either variegated or hereditary coproporphyria. The second patient had acute intermittent porphyria. Eventhough, the disease is uncommon in Thailand, many drugs can aggravate an acute attack. Thus, we should be careful not to use such drugs in these patients.

Intermittent exophthalmos.

A 28-year-old woman is reported with intermittent exophthalmos and acute orbital hemorrhage which developed after a warning symptom of recurrent positional headache. The cause was proven to be a primary orbital varix. Conservative measure was considered in this case. The characteristics of the primary orbital varix are presented and the problems of diagnosis and treatment are discussed.

Serum antibodies to HTLV-I in Thai patients with chronic progressive myelopathy, multiple sclerosis, myopathy and in HIV-seropositive intravenous drug abusers.

Antibodies to HTLV-I were assayed in sera of 9 patients with progressive myelopathy, 11 with multiple sclerosis, 5 with myopathy and in 10 HIV-seropositive intravenous heroin abusers. Clinical features in 9 cases with progressive myelopathy were not different from those previously described in tropical spastic paraparesis associated with HTLV-I infection. No detectable HTLV-1 antibody was found in the sera of any of the 35 patients studied.

Enhanced antibody response to rabies virus in patients with neurologic complications following brain tissue-derived rabies vaccination.

Sera from 26 patients with major neurologic complications, 19 patients with minor complications and 23 patients with no complications induced by Semple rabies vaccine were assayed for neutralizing antibody to rabies virus. Seroconversion to the protective level (greater than or equal to 0.5 IU/ml) was found in 21 of 22 patients (95%) with major complications, and in 7 of 19 (37%) with minor complications, occurring during the first 14 days of vaccination. This was in contrast to 42% (5/12) seroconversion in uncomplicated recipients after 14 daily injections. After initiation of dexamethasone treatment, 13 of 18 patients with major complications showed a diminution in their rabies antibody levels on day 4 or 7.