Ataxia and ophthalmoplegia: an atypical case of Miller Fisher syndrome (MFS) with anti-GAD antibody.

BACKGROUND: Miller Fisher syndrome (MFS) is frequently encountered variant of Gillian Barre Syndrome (GBS). It has distinct clinical and serological features. Here we describe an atypical GQ1b seronegative case with significantly elevated anti-glutamic acid decarboxylase antibody (GAD-Ab). CASE DESCRIPTION: A 24-year-old previously healthy Caucasian male presented with rapidly progressive ascending weakness, binocular diplopia and autonomic instability for 2 days. Examination was remarkable for asymmetrical facial weakness (L > R), opthalmoplegia and truncal ataxia without areflexia. MRI brain was normal. CSF analysis showed elevated protein. Electromyography/Nerve Conduction Study (EMG/NCS) within the first week was normal. Antiganglioside antibodies were negative. Extended serological and neoplastic workup revealed negative anti-GQ1b antibody, but significant increase of GAD-Ab, Voltage Gated Calcium Channel (VGCC) Ab, and mild elevation of TPO Ab IgG and Thyroglobulin (Tg) Ab IgG. Clinical diagnosis of partial MFS was made. He received a course of IVIg (2 g/kg over 5 days) and had complete recovery in 3 months. CONCLUSION: There are incomplete or atypical forms of MFS. Recognition of its various clinical presentations is essential for early diagnosis and optimal management. Further investigation is needed to elucidate the role of anti-GAD-ab and other autoimmune antibodies in the pathogenesis of GQ1b-seronegative MFS patients.

Effects of aging on action-intentional programming.

BACKGROUND: Action-intentional programs control "when" we initiate, inhibit, continue, and stop motor actions. The purpose of this study was to learn if there are changes in the action-intentional system with healthy aging, and if these changes are asymmetrical (right versus left upper limb) or related to impaired interhemispheric communication. METHODS: We administered tests of action-intention to 41 middle-aged and older adults (61.9 ± 12.3 years). RESULTS: Regression analyses revealed that older age predicted a decrement in performance for tests of crossed motor response inhibition as well as slower motor initiation with the left hand. CONCLUSION: Changes in action-intention with aging appear to be related to alterations of interhemispheric communication and/or age-related right hemisphere dysfunction; however, further research is needed to identify the mechanisms for age-related changes in the brain networks that mediate action-intention.

Comparison of three risk assessment methods for venous thromboembolism prophylaxis.

Venous thromboembolism (VTE) is a major problem for hospitalized patients. Nevertheless, adherence to VTE prophylaxis guidelines is suboptimal, partly because of physicians' neglect due to excessive workload. Simplified risk assessment methods (RAMs) facilitate timely risk stratification and thromboprophylaxis. However, the accuracy of such RAMs has not been extensively studied. Using the prospectively collected data from hospitalized patients of Masih-Daneshvari Hospital, we tested the accuracy of the Goldhaber RAM and the Harinath and St. John RAM for VTE prophylaxis, compared with the eighth edition of the American College of Chest Physicians (ACCP) recommendations. We evaluated 1091 patients. With reference to the ACCP recommendations, both RAMs had high specificities for detection of patients at risk of VTE (97.33 and 99.11%, respectively); however, we found significant interdepartment differences. The Goldhaber RAM had superior accuracy in medical oncology patients (P = 0.03), whereas the Harinath and St. John method was superior among surgical patients (P < 0.001). Overall accuracies of Goldhaber RAM for appropriate VTE risk assessment and for proper detection of at-risk patients were close to 60%. Corresponding figures were close to 70% for the Harinath and St. John method. Simplified VTE prophylaxis RAMs are valuable, especially for transmitting electronic alerts and for timely risk assessment and thromboprophylaxis. Both of the studied RAMs had high specificities and positive-predictive values, minimizing the risk of overprophylaxis. Improving the sensitivity of such RAMs can help for timely risk assessment for a greater array of real-world patients.