Demyelinating polyneuropathy and polymyositis as prominent neurological features in a case of HIV-infection

HIV infections are often associated with a variety of neurological symptoms. The peripheral nervous system might me affected at various stages of an HIV infection. A distally pronounced sensorimotor polyneuropathy is the most common kind of peripheral nervous system involvement, observed in up to 80% of HIV positive patients. It is characterized by distal dysethesia or parestheisa, often accompanied by neuropathic pain, paresis of different degrees and muscular atrophy. A demyelinating polyneuropathy is much rarer and it might present as an acute inflammatory polyneuroradiculatis (Guillain Barre Syndrome) or as a chronic inflammatory demyelinating polyneuroradiculitis (CIDP). The acute variant is mainly observed in early disease stages, whereas the chronic one may manifest at any stage, even in the terminal phase of AIDS. ╠uscular weakness due to myopathy may develop also at any stage of HIV infection. Myopathies in HIV-infected patients are classified as follows: (i) HIV-associated myopathies , including HIV polymyositis, inclusion-body myositis, nemaline myopathy, diffuse infiltrative lymphocytosis syndrome (DILS), HIV-wasting syndrome, vasculitic processes, myasthenic syndromes, (ii) muscle complications of antiretroviral therapy, including zidovudine and toxic mitochondrial myopathies related to other nucleoside-analogue reverse transcriptase inhibitors (NRTIs), (iii) opportunistic infections and tumor infiltrations of skeletal muscles and (iv) rhabdomyolysis.

We present the case of a male patient who presented due to a since about two years persisting, slowly progressive proximal muscular weakness of the limbs accompanied by elevated CPK values. Electromyography revealed profound myopathic findings. Conduction studies also proved a previously reported, however not in detail evaluated, chronic demyelinating polyneuropathy. Cerebrospinal fluid examination also demonstrated adjacent elevated protein concentration. The patient underwent muscle biopsy which showed myopathic changes with inflammatory infiltrations and eosinophilic inclusion, supporting the diagnosis of polymyositis. Further clinical evaluation and detailed history taking also revealed important features and symptoms, such as fever, severe weight lost, tongue leukoplakia and cutaneous lesions. Based on these an HIV infection was suspected and appropriate serologic examinations were performed (ELISA and Western blot) and finally established the diagnosis of HIV infection.

The manifestation first of demyelinating polyneuropathy followed by polymyositis as presenting neurological features leading to the diagnosis of HIV infections is uncommon. In the present case diagnosis could have been established earlier. The patient's incompliance and incomplete diagnostic evaluations were obviously the main causes of delayed diagnosis and treatment onset. Although the diagnosis of HIV infections is usually set by other medical specialists, neurologists should be aware of HIV-associated neurological manifestations, including the different kinds of HIV-related myopathy, as well as the most commonly observed distal polyneuropathy and the less frequently reported inflammatory demyelinating polyneuroradiculitis.

Key words: ăIV, demyelinating neuropathy, polymyositis.