engleski

Early onset progressive chronic inflammatory demyelinating polyneuropathy (CIDP) associated with CNS demyelination and inflammatory myopathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic disorder, which manifests usually with monophasic or relapsing course. We present a boy, now at the age of 6, with progressive generalized muscle weakness since the age of 2, developed after viral infection. On examination, generalized hypotonia and hypotrophy of small muscles, peroneal gait, positive Gower's sign, areflexia, and right abducens palsy were registered. Electromyoneurography showed severe neurogenic lesion, low compound muscle action potentials, slowed motor nerve conduction and myopathic pattern in proximal muscles. Increased protein content was obtained in cerebrospinal fluid. Muscle biopsy showed neurogenic atrophy. Sural nerve biopsy revealed demyelination and onion bulbs. Inflammatory perivascular CD 3 positive infiltrates were present in both biopsies. Brain MRI showed cortical atrophy with hyperintensities of the white matter and gray matter hypointensities. Increased serum anti-GM1 (IgM) and anti GD 1b (IgG) antibodies were registered. Very long chain fatty acids, B12, folic acid, lactate, and immunologic test were normal. He rapidly improved on IVIG and methylprednisolone treatment, while upon tapering off the steroids complete deterioration occurred. Demyelination might develop in central and peripheral nervous system associated with inflammatory myopathy in patients with progressive course of CIDP.

CIDP

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