Dados do Trabalho

Título

Chronic inflammatory demyelinating polyneuropathy (CIPD) with unilateral facial and hypoglossal involvement: A case report

RESUMO

Case report: A 31-year-old female with a two-months history of progressive weakness and paresthesia in both upper limbs, mainly distal, followed by a two-day right foot numbness. She referred transient and recovered weakness in the right hand three years prior to admission, with normal exams e no follow-up needed. She had no comorbidities, antecedent infection or recent immunization. She underwent hospitalization and extensive laboratory and radiological work-up. Cerebrospinal fluid analysis revealed high protein content (>90,8mg/dL), normal cell count (WBC 1/mm3, 100% lymphocytes) and normal glucose level (54 mg/dL). Needle electromyography (EMG) and nerve conduction velocity (NCV) study showed diffuse, symmetric, sensorimotor, predominantly primary segmental demyelinating polyradiculoneuropathy. No other relevant findings were found. Initial treatment included intravenous methylprednisolone and immunoglobulin that shown immediate clinical improvement. Outpatient management identified facial asymmetry two months later, besides recurrency of incipient weakness and numbness in extremities. A new curse of medication was carried out also with improvement in symptoms and bimestrial sessions were scheduled. On her next return, she complained of difficulty speaking and eating. Neurological exam evidenced right hypoglossal palsy that determined dysarthria and impaired mobility of the bolus within the mouth. No atrophy or tongue fasciculation were found. New laboratory and radiological exams did not show any abnormalities and 45-day interval course of medication was restated with no further significant neurological deterioration. Discussion: The usual phenotype of CIDP is a sensorimotor disorder with symmetric proximal and distal affectation, areflexia and conduction lowing, temporal dispersion and/or conduction block. Cranial nerve findings are not common: facial muscle weakness may be present in up to 13% and hypoglossal involvement has been reported in only 0–3.8%. Final comments: CIDP is an immune-mediated chronic neuropathy with a wide spectrum of clinical manifestations, lasting for more than two months. Although cranial nerve manifestation is infrequent (7%-16%), we describe this uncommon finding in a patient with facial and hypoglossal involvement. Therefore, therapeutic strategies and prognostic implications of the presence of cranial neuropathy have yet to be determined and active examination to search this affection must be carefully accomplished.

Palavras Chave

Chronic inflammatory demyelinating polyneuropathy; cranial nerves; facial nerve; hypoglossal nerve

Área

Doenças Neuromusculares