Rinshō shinkeigaku = Clinical neurology
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Case Reports
[Progressive dysarthria and bilateral sensory disturbance in a case of bilateral ventrolateral pontine infarction].
A rare case of bilateral ventrolateral pontine infarction in a 70-year-old man who developed progressive dysarthria and bilateral sensory disturbance is reported with literature review. He had been diagnosed with hypertension, dyslipidemia, and impaired glucose tolerance 10 years earlier. ⋯ High resolution three-dimensional MRI revealed bilateral ventrolateral pontine infarction with a large atherosclerotic plaque in the ventral side of the basilar artery, which led to a diagnosis of atherothrombotic brain infarction. The atherosclerotic plaque in the basilar artery was thought to be responsible for simultaneous occlusion of the bilateral short circumflex arteries of the pons.
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A 20-year-old woman first developed acute disseminated encephalomyelitis (ADEM) at 11 years of age. At 17 years of age, she was hospitalized due to generalized seizure and diagnosed with encephalitis. Brain MRI revealed a FLAIR-hyperintense lesion in the unilateral cerebral cortex. ⋯ MDEM has been observed in patients that are seropositive for the anti-MOG antibody. More recently, unilateral cerebral cortex encephalitis with epilepsy has also been reported in such patients. The co-occurrence of MDEM and cortical encephalitis in the same patient has important implications for the pathogenesis of anti-MOG antibody-associated autoimmune diseases.
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Case Reports
A case of hereditary neuropathy with liability to pressure palsies due to push-up exercise.
A 17-year-old man with no familial history developed motor and sensory disturbance of the left upper limb a few days after starting push-up exercise. Neurological examination revealed broad weakness and radial sensory disturbance of the left upper limb and magnetic resonance neurography showed laterality of brachial plexus intensity signals. ⋯ Genetic tests revealed a diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP). HNPP should be included in the differential diagnosis for neuropathy due to slight exercise or nerve compression even when familial history is negative.
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A 33-year-old woman was admitted due to disturbance of consciousness, dysarthria, dysphagia, sensory disturbances and weakness of the left upper limb after mycoplasma infection. She was treated with intravenous immunoglobulin and intravenous high-dose methylprednisolone as Bickerstaff brainstem encephalitis (BBE). ⋯ The transient reflex myoclonus may be originated from brainstem lesion which was affected by BBE. Reflex myoclonus is thought to be rare symptom in patient with BBE.
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A 78-year-old man presented with subacute progressive proximal weakness and dysphagia. A biopsy specimen from the left biceps femoris revealed evidence of necrotic and regenerating muscle fibers, but lymphocyte infiltration was not noted. The patient was diagnosed with necrotizing myopathy with anti-signal recognition particle (SRP) antibodies. ⋯ Immunoelectrophoresis of serum revealed IgG M protein, and muscle pathology revealed amyloid deposits in numerous blood vessels and at the periphery of a few muscle fibers, and deposits stained positive for anti-λ light chain antibody. The patient was diagnosed with amyloid myopathy, and therapy for systemic amyloid light chain amyloidosis caused muscle weakness to diminish. Amyloidosis is believed to be the primary pathology in this case based on the patient's response to treatment reaction, but the significance of a case involving both amyloid myopathy and necrotizing myopathy warranted examination.