Clinical, histological and radiological responses to methylprednisolone in HIV-associated rod myopathy

Neuromuscul Disord. 2017 Aug;27(8):756-759. doi: 10.1016/j.nmd.2017.05.008. Epub 2017 May 12.

Abstract

Skeletal muscle involvement as a neurologic manifestation in individuals with HIV is rare, especially as rod myopathy. We describe a 41-year-old male with HIV infection who presented progressive proximal muscle weakness and limb-girdle atrophy. A muscle magnetic resonance image showed bilateral fatty infiltration and post-contrast enhancement in the arm and thigh muscles. The muscle biopsy revealed intracytoplasmic aggregates with appearance of nemaline rod bodies with Gomori trichrome staining and electron microscopy in most fibers. The patient underwent six cycles of intravenous methylprednisolone pulses, presenting clinical improvement. Post-treatment muscle biopsy showed fewer nemaline bodies and muscle magnetic resonance image depicted a pronounced reduction of muscular edema. These findings corroborate that deposition of nemaline bodies in these patients might be related to an immune response triggered by the virus.

Keywords: HIV infection; Inflammatory myopathy; Muscle MRI; Rod myopathy; Sporadic late-onset nemaline myopathy (SLONM).

Publication types

  • Case Reports

MeSH terms

  • Adult
  • HIV Infections / diagnostic imaging
  • HIV Infections / drug therapy*
  • HIV Infections / pathology*
  • HIV Infections / physiopathology
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Methylprednisolone / therapeutic use*
  • Muscle Weakness / diagnostic imaging
  • Muscle Weakness / drug therapy
  • Muscle Weakness / pathology
  • Muscle Weakness / physiopathology
  • Muscle, Skeletal / diagnostic imaging
  • Muscle, Skeletal / drug effects
  • Muscle, Skeletal / pathology
  • Myopathies, Nemaline / diagnostic imaging
  • Myopathies, Nemaline / drug therapy*
  • Myopathies, Nemaline / pathology*
  • Myopathies, Nemaline / physiopathology
  • Neuroprotective Agents / therapeutic use*
  • Treatment Outcome

Substances

  • Neuroprotective Agents
  • Methylprednisolone