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Título

Severe immune neuropathy with respiratory distress and locked-in syndrome

RESUMO

Case Report: A 58-years-old man, presented with tingling and needles in both hands, progressed to weakness. Over 5 weeks his symptoms progressed to the lower limbs, and he also complains of difficulty to go up and down stairs. In a few months, he was unable to walk without support. On neurological examination he presented with proximal and distal asymmetrical weakness, areflexia and sensory impairment distaly. CSF was normal. NCS revealed a sensory and motor demyelinating polyradiculoneuropathy. A hypothesis of CIDP was made and he was put on high doses of intravenous methylprednisolone for 5 consecutive days. Despite that he rapidly deteriorated, and he was treated with IvIG with no response. He evolved with respiratory insufficiency requiring invasive mechanical ventilation. In addition, he had bilateral eyelid ptosis and restriction of eye movement in all positions. A bedside test with pyridostigmine was performed, with a slight improvement in ptosis in one eye. Then, PLASMEX was done and he started to recover. Based on the poor response to treatment IgG-4 nodo-paranodopathy was considered and rituximab was done, and he is improving.

Discussion: CIDP is an acquired immune inflammatory polyneuropathy characterized by a progression over at least 2 months with a progressive or relapsing course. Our case had several unusual features that raised the possibility of a different diagnosis, including onset of weakness from upper limbs, cranial neuropathy, poor response to IVIg and methylprednisolone. The incidence of ventilatory failure in CIDP is very rare (1-9%), its mechanism is believed to be via phrenic nerve demyelination. Cranial neuropathy, ptosis and ophthalmoplegia are not common in patients with CIDP, raising the possibility of an overlap of myasthenic syndrome.

Final Comments: We identified a patient with a distinct phenotype characterized by severe sensorimotor neuropathy presenting with tetraplegia, cranial nerve involvement, respiratory failure and locked-in syndrome and poor response to steroids and IvIG. There are some case reports of anti–pan-neurofascin-associated neuropathy with similar phenotype. Unfortunately, we were not able to test specific antibodies.

Palavras Chave

CIPD, severe sensorimotor neuropathy , treatment PLASMEX and rituximab