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Título

Charcot Marie-Tooth disease (CMT) superimposed on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a patient with a history of childhood poliomyelitis: a case report

RESUMO

Case Presentation: A 68-year-old male patient presented weakness in lower limbs, myalgia, paresthesia in the feet and hands, initiated 9 months ago, with progressive worsening. He denied loss of sphincter control, dyspnea, dysphagia and infections preceding the onset of the condition. History of childhood poliomyelitis with left lower limb atrophy. Neurological examination showed bilateral pes cavus, hammer toes and lower limbs in a characteristic "inverted champagne bottle" appearance. The cranial nerves were all intact. Motor examination showed reduced muscle strength (MRC grades were 4 for lower limbs and 5 for upper limbs) and hyporeflexia in upper and lower limbs. Sensory examination revealed a decreased vibratory sense in the distal lower and in upper limbs. The coordination test assessment showed ataxic gait aggravated by closing eyes and dysmetria on the left side. Cerebrospinal fluid (CSF) revealed albuminocytological dissociation (leukocytes: 1,56 cell/mm3 and proteins: 116,4 mg/dL). Electroneuromyography demonstrated a chronic demyelinating sensory-motor polyradiculoneuritis associated with a bilateral chronic neuropathic process, without current denervative activity, in bilateral myotomes from L2 to S1, consistent with the history of childhood poliomyelitis. The patient has been submitted to monthly pulse therapy with methylprednisolone 1g/day for 5 days, with progressive improvement of the condition. Discussion: In the first evaluation, the patient has lower limbs deformities characteristic of CMT, but due to the presence of progressive loss of muscle strength, the CIDP hypothesis was raised and corroborated by protein-cytological dissociation. Although the coexistence of Charcot- Marie- Tooth disease with CIDP is already established in the literature, the inflammatory diagnosis superimposed on the hereditary neuromuscular process is not always easy. This case was even more challenging from a clinical and electrophysiological perspective because of the history of childhood poliomyelitis. Final Comments: This case report highlights the importance of considering clinical exacerbation of CMT due to the possibility of superimposed CIDP. The recognition of superimposed inflammatory neuropathy, although difficult, is of high clinical importance since with specific treatment the patient's clinical condition can be improved.