The Characteristics and Prognosis of Miller Fisher Syndrome

PURPOSE: In the present study, the clinical characteristics and prognosis of patients clinically diagnosed with classic Miller Fisher syndrome were evaluated. METHODS: We retrospectively investigated the clinical and laboratory findings as well as treatment outcomes using the medical records of patients diagnosed with Miller Fisher syndrome. Symptom triad including acute ophthalmoplegia, ataxia, and areflexia were evaluated. RESULTS: This study included 10 patients. Nine patients had antecedent infectious illness which took an average of 11 ± 9.7 days for onset of diplopia from antecedent infectious systemic illness. Seven patients showed bilateral paralytic strabismus. Specifically, 5 patients showed the involvement of vertical and horizontal extraocular muscles. Pupil impairment and blepharoptosis were observed in 4 patients, limb weakness in 3 patients, dysarthria in 3 patients and facial palsy in 1 patient. Two patients showed contrast enhancement of the abducens nerve on brain magnetic resonance imaging (MRI) and 2 patients showed albumin-cell dissociation on cerebrospinal fluid (CSF) analysis. Eight patients had anti-GQ1b antibodies in their blood serum analysis. Six patients were treated with intravenous immunoglobulins and the other patients were observed with regular follow-ups. The duration of diplopia was 2.9 ± 1.2 months in the treatment group and 3.1 ± 1.7 months in the control group (p > 0.05). The duration of ataxia was 1 ± 0.4 months in the treatment group and 1 ± 0.9 months in the control group (p > 0.05). CONCLUSIONS: Miller Fisher syndrome should be considered in patients with antecedent infection; acute ophthalmoplegia, ataxia and areflexia as well as anti-GQ1b antibody can be helpful for diagnosis. Final outcomes in the treated group were not significantly different from the control group and all patients showed good final outcomes.

Distribution of Inflammatory Cells and Expression of PSGL-1 in Infant Brainstem Tissue Related Fatal Brainstem Encephalitis / 法医学杂志

Objective To explore the distribution of inflam m atory cells and positive expression of P-se-lectin glycoprotein ligand-1 (PSG L-1) in infant brainstem tissue from hand-foot-m outh disease related fatal brainstem encephalitis. Methods Tw enty brainstem sam ples from infants suffered from brainstem en-cephalitis w ere collected as the experim ental group. Ten brainstem sam ples from infants died of non-brain diseases and injuries w ere collected as the control group. The distribution of inflam m atory cells and the expression of PSG L-1 in the tw o groups w ere exam ined by im m unohistochem ical m ethod. The characteristics of the positive cells w ere observed. Results In brainstem tissue of the experim ental group, there w ere sleeve infiltrations of inflam m atory cells around the vessels and in the glial nodule. Microglia was the m ost and following was neutrophils around the vessels and in the glial nodule. There was a significant statistical difference am ong m icroglias, neutrophils and lym phocytes (P<0.05). There was no sleeve infiltration in the control group. PSG L-1 protein was expressed w idely in inflam m atory cells in the experim ental group, especially in the inflam m atory cells around the vessels and in the glial nodule. B ut PSG L-1 positive staining could be observed significantly less in the control group com paring with the experim ental group (P<0.05). Conclusion Microglia is the m ain type of inflam m atory cells involved in the progress of the fatal disease. Moreover, PSG L-1 could participate in the pathogenesis of hand-foot-m outh disease related fatal brainstem encephalitis.

Hand-Foot-Mouth Disease Related to Enterovirus 71

Hand-foot-mouth disease (HFMD), one of the more distinctive rash syndromes, is most frequently caused by coxsackievirus A16, but can also be caused by enterovirus 71 (EV71) and other coxsackieviruses. Recently, there have been large outbreaks of simple, neurologically complicated and even fatal HFMD caused by EV71 in Western Pacific Area. However, in the Republic of Korea, despite its location in EV71 endemic Western Pacific Area, published reports on HFMD with EV71 are rare and there are no published reports on fatal cases. After the first fatal case of HFMD caused by EV71 announced in May 2009, much more cases of neurologically complicated HFMD have been announced. Even now, physician's increased awareness about the seriousness of HFMD, viral surveillance and early warning system of HFMD, and early detection and proper management of potentially life threatening HFMD caused by EV71 are required in the Republic of Korea, as in the neighboring countries.

A Case of Bickerstaff's Brainstem Encephalitis with Guillain-Barre Syndrome Presenting Optic Neuropathy and Seizure

Bickerstaff's brainstem encephalitis (BBE), characterized by acute ophthalmoplegia and ataxia, often causes impaired consciousness and hyperreflexia. A 17-year-old man was admitted with an acute meningitic condition including high and neck stiffness. His condition rapidly deteriorated over 2 weeks, and he showed ophthalmoplegia, ataxia, seizure, tetraplegia, comatose mentality, and optic neuropathy. Electroencephalography showed diffuse slow waves. Visual evoked potentials showed no responses in the right eye. This is the first case of BBE with Guillain-Barre syndrome presenting with optic neuropathy and seizure.