Neuromyelitis optica (NMO) is an inflammatory-demyelinating disease of the central nervous system classically characterized by optic neuritis and severe myelitis. New diagnostic criteria defined neuromyelitis optica spectrum disorder as limited forms of NMO or diverse neurologic presentations in the presence of specific antiaquaporin-4 antibodies. We report the case of a 57-year-old woman admitted in our department for recurrent attacks of optic neuritis, tetraparesis with severe painful tonic spasms of the left limbs and brainstem involvement. Painful tonic spasms have been described as movement disorders associated with multiple sclerosis, but a growing number of reports describe them in cases of NMO.
Therapeutic plasma exchange (TPE) is an extracorporeal blood purification technique, which removes large molecular weight particles such as autoantibodies from plasma. TPE is accepted by the American Society for Apheresis as first line treatment for some severe neuroimmune disorders. Double filtration plasmapheresis (DFPP) is a newer technique in which plasma is not entirely removed, only the antibodies, using special filters. High-dose intravenous immunoglobulins are an alternative treatment for these patients but are much more expensive. We reviewed medical records of 20 patients with severe neurological diseases requiring TPE or DFPP. We analyzed the indications, complications and efficacy of these procedures. After completing the procedures, neurological improvement was recorded in 80% of the patients, 5% had no improvement, and the mortality was 15%. The rate of neurological improvement was similar to other studies. None of the patients presented catheter related complications. Systemic complications were mild, transient and completely reversible.
Guillain-Barre syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide, having an incidence of about 1/100,000 across several studies in a number of countries. We present the case of a 60-year-old female patient, with known hypertension, admitted to our department for paresthesia and muscle weakness predominantly in the distal upper and lower limbs. Symptomatology had an acute onset after 14 days from influenza vaccine administration. Lumbar puncture revealed CSF glucose (91 mg/dl), CSF protein (0.508 g/l) and no pleocytosis. Electromyography supported the presumptive diagnosis of polyradiculoneuritis. The patient underwent three sessions of double filtration and the final diagnosis was Guillain-Barre polyradiculoneuritis secondary to influenza vaccination. Approximately 80% of patients with polyradiculoneuritis recover completely within a few months to one year; however, 5-10% of these patients experience one or more recurrences. It should be emphasized that acute-phase rehabilitation must start immediately and include an individualized program of gentle strengthening, and manual resistive and progressive resistive exercises.
Key words: polyradiculoneuropathy, influenza vaccine, neurorehabilitation,
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