Within 2 days he noticed difficulties with speech and swallowing. with additional symptoms such as diplegia facialis, bulbar dysarthria or limb weakness, other causes should be considered in the diagnostic work-up. The presence of serum IgG and IgM antibodies to the ganglioside GQ1b may help to confirm or reject the analysis MFS, particularly when a Nutlin-3 patient with MFS has a recurrence of symptoms. We report a patient with relapsing dysarthria and ataxia in whom dedication of serum anti-GQ1b antibodies helped to make the correct diagnosis. During the 1st show the patient experienced MFS, but during the second show the symptoms were caused by mind stem infarction. CASE Demonstration A vital 80-year-old man with a history of claudicatio intermittens developed double vision and unsteadiness of gait 1 week after a Nutlin-3 slight upper respiratory tract illness. Within 2 days he noticed difficulties with conversation and swallowing. Neurological exam revealed a bilateral external ophthalmoparesis with normal light reactions, diplegia facialis and bulbar dysarthria with paresis of the pharyngeal muscle tissue. Additionally, he had a symmetrical slight weakness of deltoid and biceps muscle tissue, sensory ataxia, almost absent vibration sense and areflexia with normal plantar reflexes. The patient was unable to stand or walk unaided. The medical symptoms were compatible with a analysis of MFS. INVESTIGATIONS This analysis was supported by the presence of an elevated cerebral spinal fluid protein content (0.77 g/litre, normal research 0.58 g/litre) without pleiocytosis and a high serum antibody reactivity to GQ1b (IgG titre 3200 and IgM titre 1600). Cerebral CT scanning showed a small silent mind infarct in the remaining corona radiata. TREATMENT Immediately after admission the patient deteriorated and developed a paralysis of pharyngeal muscle tissue followed by respiratory failure, for which he required mechanical air flow. He was treated with a standard dose of intravenous immunoglobulins (0.4 g/kg/day time for 5 days) after which he gradually improved. End result AND FOLLOW-UP At 4 weeks after admission the Nutlin-3 patient experienced a residual ataxia but was able to walk independently. The oculomotor motions also improved, leaving a slight bilateral ophthalmoparesis. The patient was discharged to a rehabilitation centre. At 5 weeks later the patient developed Rabbit Polyclonal to SLC6A1 a second show with symptoms that were largely similar to the 1st show. The patient again complained of progressive conversation disturbances, double vision and unsteadiness of gait. This time the patient also complained of vertigo and nausea. The onset of this show was probably acute, even though symptoms fluctuated in severity and progressed within several hours. Neurological exam revealed normal consciousness and a residual external ophthalmoplegia with progression of impaired abduction on the right part without nystagmus. There was slight peripheral facial nerve palsy on the right. Bulbar dysarthria experienced worsened compared with the neurological exam at discharge. Visual fields were normal. The patient was unable to walk and showed respiratory stress. Tendon reflexes were absent and plantar reflexes were normal. Based on these findings, basilar artery thrombosis and recurrent MFS were considered as Nutlin-3 differential diagnoses. Cerebral CT scanning showed no fresh abnormalities compared to the CT scan of the previous show. CT angiography showed occlusion of the intradural section of the remaining vertebral artery (V4), compatible with acute thrombosis, and a normal basilar artery. Cerebral MRI showed a hypointensive area on T1 and a hyperintensive area on T2 in the remaining medulla oblongata, compatible with recent ischaemia due to occlusion of the remaining posterior substandard cerebellar artery (fig 1). Moreover, anti-GQ1b serology was bad this time (fig 2). Open in a separate window Number 1 MRI scan of the patient during second show. T2-weighted axial MRI.