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Título

CHRONIC IMMUNE MOTOR POLYRADICULOPATHY AT A YOUNG AGE: A CASE REPORT

Apresentação do Caso

A 14-year-old boy presented with progressive distal muscle weakness for one year, which started with distal left lower limb paresis, followed by right side involvement after six months. He was previously healthy, with no recent infection. Neurological exam showed preserved mental status, cranial nerves, and upper limbs motor evaluation. There was distal muscle hypotrophy, and weakness, described as 0/5 to 3/5 on the left, and 2/5 to 3/5 on the right side (MRC Scale). Ankle reflexes were abolished. Lasègue sign was present. Sensation and coordination were normal. Nerve conduction study revealed a bilateral absence of fibular and tibialis anterior potentials, fibrillation, positive sharp waves, fasciculations, and rarefaction of motor units in the lower limb and paraspinal muscles. Sensory conduction was unaltered. This suggested a relatively symmetrical motor axonal neuropathic process in L5-S1 myotomes bilaterally, with signs of severe denervation. MRI revealed contrast enhancement in L5 and S1 ventral nerve roots. Cerebrospinal fluid analysis showed 2 cells/mm3, 91 mg/dL proteins, and normal glucose; VDRL, herpes panel, and cultures were negative. Oligoclonal bands were negative and protein electrophoresis was normal. Additional laboratory investigation was unaltered, including blood cell count, erythrocyte sedimentation rate, C-reactive protein, fasting glucose, glycated hemoglobin, TSH, free T4, CK, transaminases, and vitamin B12. There was no evidence of infectious diseases (VDRL, COVID-19, HIV, hepatitis B, and C testing were negative). Immunological tests were negative (ANA, ANCA, anti-SSA/SSB, anti-SRP, and anti-Scl70). PET-CT discarded neoplastic lesions.

Discussão

The progressive symmetric distal motor neuropathy, electromyography features, radiculitis, and albuminocytological dissociation suggested a chronic immune motor polyradiculopathy (CIMP), which was recently described as an inflammatory process restricted to the ventral nerve roots. Differential diagnoses are multifocal motor neuropathy and motor neuron disease. Steroid response rate is usually high, thus methylprednisolone 3g was monthly administered for three months, followed by prednisone tapering off, and, at last, by methotrexate as steroid-sparing. He progressed with clinical improvement.

Comentários Finais

Despite uncommon, CIMP should be considered as a differential diagnosis of motor neuropathies in children.

Referências (se houver)

Silwal A, Pitt M, Phadke R; et al. Clinical spectrum, treatment and outcome of children with suspected diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy. Neuromuscul Disord 2018; 28(9): 757-765.

Menon D, Katzberg HD, Bril V. Treatment Approaches for Atypical CIDP. Front Neurol 2021; 15;12:653734.

Fonte de Fomento (se houver)

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Área

Doenças neuromusculares