An unusual Guillain-Barré Syndrome mimic: a case of Spinal ADEM and review of the literature

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Case reportThe patient and her two children had onset of gastroenteritis (GE) with diarrhea and vomiting eight weeks prior to the initial presentation at our emergency room.The GE had lasted for 24 hours.The day after onset of GE, the patient began to feel a burning sensation on the right side of the scalp as well as slight pain in the lower back.Two days later, she sought a general practitioner at her nearby community medical center, due to the lumbar discomfort and numbness in the feet and hands.Infection parameters and antibodies to Lyme disease in blood were normal.Due to increase in the intensity of the sensory AbstractBackground: Acute disseminated encephalomyelitis (ADEM) is one the most common inflammatory demyelinating brain disorders in children and adolescence and it is considered to constitute a third of all encephalitis.To date only few cases of ADEM confined to the spinal cord have been reported in the literature, though this may in part be due to the fact that patients with suspicion of encephalitis are usually not worked-up with a spinal MRI.However, the pathogenesis of spinal ADEM is similar to that of brain ADEM, including a similar relationship to antecedent infections and vaccinations, making it necessary for the clinician to recognize the fact that some cases of ADEM may be restricted merely to the spinal cord.Case presentation: Here, we describe a 45 year old female with a post-infectious sub-acute ascending sensorymotor disturbance eventually affecting all four limbs, hyporeflexia and a long-stretched multilevel intraspinal inflammatory lesion suggesting spinal ADEM.Brain magnetic resonance imaging (MRI) was normal, though the spinal MRI revealed intraspinal hyperintensity contrast-enhancing lesions in the entire spinal cord and the cauda equina.The differential diagnosis based on clinical signs, results of cerebrospinal fluid analysis and imaging included Guillain-Barré syndrome (GBS), Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP), Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), Multiple Sclerosis, Neuromyelitis Optica and spinal cord malignancy.A good clinical response as well as complete radiological resolution was achieved after high-dose corticosteroid therapy and six plasma exchanges leading to an improvement from wheel-chair dependence due to paraparesis, to an ability to walk outdoors without need for any walking aids, upon last follow-up eleven months after onset of neurological symptoms.Conclusions: This case of isolated spinal ADEM presented here had similar CSF findings and clinical, as a well as radiological outcome after immunomodulatory treatment as cerebral ADEM.Further studies are needed to elucidate if spinal ADEM is often misdiagnosed as mere transverse myelitis, and to estimate the long term outcome and mortality rate of spinal cord ADEM.symptoms during the following four weeks, a brain MRI was planned for to exclude multiple sclerosis (MS).On day 33 after onset of the initial neurological symptoms, she experienced onset of weakness in the legs and an increase in level of numbness in the hands and feet.The weakness and numbness were most pronounced in the right foot but was also present in both lower legs.The numbness then spread to the thighs and the dorsal side of the hands and elbows.Eight weeks after onset of neurological symptoms the patient was unable to walk without aid due to weakness of the right leg.She also experienced pinand-needle pain in the right arm and leg and felt weak in both legs.She could not walk more than 2-5 meters.She also had a dull pain sensation around the pelvis and lumbar spine, which radiated to the right thigh, where she also felt a burning sensation.At this stage, there Roshanisefat H (2016) An unusual Guillain-Barré Syndrome mimic: a case of Spinal ADEM and review of the literature Clin Case Rep Rev, 2016

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