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Título

NEUROMYELITIS OPTICA – TONIC SPASMS AS ASSOCIATED CLINICAL MANIFESTATION

RESUMO

CASE PRESENTATION:
DNG, female, 26 years old, with inappetence, asthenia and paresthesia in the lower limbs, started 40 days after cesarean section and with progressive worsening. She had painful tonic limb spasms of short duration provoked by voluntary movements and emotional factors. Former alcoholic/addict for 6 months, she reported reduced visual acuity for 4 years after first pregnancy, with partial improvement.
On physical examination, in addition to spasms, there was dysesthesia and paresis of limbs, hyperreflexia, bilateral reduced visual acuity with diffuse left papilla atrophy. Magnetic resonance imaging (MRI) of the orbits showed atrophy and hypersignal in the left optic nerve, suggesting inflammatory optic neuropathy, cervical spine MRI with hypersignal in T2 bulbomedullary, in C2-C3 and C6-C7, corresponding to demyelinating lesion, increased CSF of proteins and positive anti-aquaporin 4 antibodies (anti-AQP4).
Pulse therapy with 1g Methylprednisolone was performed for 5 days and 40 mg of prednisone was maintained with gradual weaning. There was a good response with complete recovery. Currently on Azathioprine 50mg 2 times a day. For the treatment of spasms, we opted for the use of benzodiazepine and carbamazepine, with good control.

DISCUSSION:
Neuromyelitis optica (NMO) is an autoimmune inflammatory disease of the central nervous system (CNS), characterized by simultaneous or consecutive cases of optic neuritis and transverse myelitis. The majority (80%) is caused by IgG autoantibodies anti AQP4– a protein that forms a water channel in CNS. The clinical manifestations are diverse, being normally present the reduction of visual acuity and paresis/paresthesia in limbs with sensitive level. Tonic spasms are not common and are characterized by sudden onset of tonic postures, short duration, triggered by voluntary movements, sensory stimulation or hyperventilation, with or without pain and are due to axonal irritability due to the release of inflammatory mediators. On MRI there is hypersignal in the optic nerve and spinal cord, in addition to anti-AQP4 in blood/liquor. Treatment is with pulsetherapy, followed by immunosuppressive medication. Tonic spasms respond well to carbamazepine.

FINAL COMMENTS:
NMO is a disease with multiple clinical manifestations, sometimes presenting atypical symptoms as in the case above. It is up to the physician to make an early diagnosis for appropriate treatment, seeking control of symptoms and a favorable outcome.

Palavras Chave

Neuromyelitis optica; tonic spasms

Área

Neuroimunologia