Myasthenia Gravis in HIV Positive Girl

Excerpt

To the Editor: Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission [1]. MG in association with HIV infection is rare [2‐4]. We discuss a 5-y-old girl with perinatally acquired HIV infection developing MG, and highlight challenges faced in her management. A 5-y-old girl presented with history of bilateral fluctuating ptosis from five months. Consultation was sought after she developed dysarthria, dysphagia, and bulbar weakness for the past 2 wk. Other than myasthenia she was asymptomatic and was not on combination antiretroviral therapy (cART). On examination she had bilateral ptosis, external ophthalmoplegia, bulbar and proximal muscle weakness. In view of her oculo-bulbar symptoms, the differentials considered were, myasthenia gravis, or a space occupying or inflammatory lesion of the brain stem. The repetitive nerve stimulation test (RNST) at 3 Hz frequency at left abductor digiti minimi showed a decrimental response of 31% (Fig. 1, normal <10%). She had prompt reversal of ptosis and improvement in speech after intramuscular neostigmine challenge test establishing the diagnosis of MG; however, serum anti-acetylcholine receptor (AChR) antibodies were negative. Her CD4+ T cell counts were 1289/μL and CD3+ T cell counts were 2010/μL. She was treated with oral pyridostigmine and prednisolone (2 mg/kg/d) and her symptoms subsided. After 6 wk, on tapering prednisolone she had recurrence of symptoms hence she required hiking dosage. She was initiated on cART (zidovudine, lamivudine, and nevirapine) because of decline in CD4+ T cell counts (516/μL) after initiation of steroid therapy. She remained well during follow-up; gradually she was weaned from steroids and pyridostigmine. At the last follow-up, 2 y after the diagnosis of MG, she remained in clinical remission; with normal CD4+ T cell counts on cART.

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