ABSTRACT
We report a patient with Lewis-Sumner syndrome (LSS) who showed an improvement only with plasma exchange (PE). The patient, 32-yr old man, had progressive multifocal motor-sensory deficits with persistent, multiple conduction blocks and marked slowing of NCVs. Nerve pathology supported a diagnosis of demyelinating neuropathy by revealing marked loss of myelinated fibers with inter- and intrafascicular variation. Although the patient was refractory to treatment with corticosteroid and intravenous immunoglobulin, PE produced a dramatic improvement. Our experience strongly proposes that PE should be tried for refractory LSS.
Subject(s)
Adult , Humans , Male , Adrenal Cortex Hormones/therapeutic use , Demyelinating Diseases/diagnosis , Immunoglobulins, Intravenous/therapeutic use , Neural Conduction/physiology , Peripheral Nerves/pathology , Plasma Exchange , Syndrome , Treatment OutcomeABSTRACT
Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache, diffuse dural thickening, and enhancement in MRI associated with low cerebrospinal fluid pressure. While SIH causes several complications such as subdural hematoma, cranial nerve palsies, and altered consciousness, cerebral venous thrombosis caused by SIH has been rarely described. We report a patient presenting with generalized tonic-clonic seizure that was probably caused by cortical vein thrombosis that had developed after SIH.