[A comparative study of the clinical and histological characteristics between classic nemaline myopathy and that associated with the human immunodeficiency virus].

BACKGROUND

Several cases of nemaline myopathy (NM) have been described in patients with human immunodeficiency virus (HIV) infection. The clinical and histological characteristics of classical NM are known, but remain to be defined in HIV positive patients.

METHODS

A retrospective review of the NM over a 12-year period (1982-1993) was carried out. The clinical and histological data of the classical NM were compared with those associated to the HIV.

RESULTS

Eight cases of NM were collected: five in HIV negative patients (classical NM) and three in HIV positive patients. The latter patients with NM were all males with no family history of myopathy. Motor development had been normal and was not associated with disorders in other systems and the muscular symptoms were slight. On muscle biopsy a lower percentage of fibers with nemaline bodies and type 2 muscle fiber atrophy were observed, as well as slight alterations in the oxidative reactions. A second muscle biopsy performed two years after in one of these patients was normal. Females predominated in the group of HIV negative patients with family history, motor retardation during infancy and different extramuscular alterations being frequent. The muscle symptoms were more marked and severe in the two cases with respiratory musculature involvement. Histologic study demonstrated a higher percentage of nemaline bodies, predominance of type 1 muscle fiber atrophy and frequent alterations in the oxidative reactions.

CONCLUSION

Nemaline myopathy presented in patients with HIV infection differs from the classically described form. The presence of nemaline bodies on muscle biopsy in HIV positive patients may be interpreted more as an epiphenomenon in relation with HIV infection than as a primary myopathy.