Atypical Presentation of Acute Disseminated Encephalomyelitis (ADEM) in a Middle-Aged Adult.

BACKGROUND Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease that usually presents in pediatric patients, usually following a viral or bacterial infection. The clinical findings in ADEM include acute neurologic decline that typically presents with encephalopathy, with some cases progressing to multiple sclerosis. An atypical case of ADEM is reported that presented in a middle-aged adult. CASE REPORT A 46-year-old Caucasian man, who had recently emigrated to the US from Ukraine, presented with gait abnormalities that began four days after he developed abdominal cramps. Magnetic resonance imaging (MRI) of the brain with contrast, fluid-attenuated inversion recovery (FLAIR), and T2-weighting showed contrast-enhancing, patchy, diffuse lesions in both cerebral hemispheres. Cerebrospinal fluid (CSF) was negative for oligoclonal bands. On hospital admission, the patient was treated with intravenous (IV) methylprednisolone, 500 mg twice daily. He responded well and was discharged from hospital after a week, with resolution of his presenting symptoms and signs. CONCLUSIONS This report is of an atypical presentation of ADEM in a middle-aged patient who presented with spastic paresis. Although there are no set guidelines for the diagnosis of ADEM in adults, this diagnosis should be considered in patients with acute onset of demyelinating lesions in cerebral MRI. As this case has shown, first-line treatment is with high-dose steroids, which can be rapidly effective.

Effect of inpatient electroencephalography on clinical decision making.

CONTEXT: Routine inpatient electroencephalography (EEG) is commonly used as a diagnostic and therapeutic decision-making tool in the care of patients with a wide spectrum of conditions. Previous investigations on EEG use have focused on current guidelines or specific clinical presentations. OBJECTIVE: To assess the effect of EEGs on clinical diagnosis and management of disease in adult inpatients in a community hospital. METHODS: Medical records of adult patients who underwent EEG between October 2008 and June 2009 in a single general community hospital were retrospectively reviewed. Data were collected for comorbidities, diagnoses, and management. Findings from EEGs were classified as normal, abnormal, or uninterpretable and according to whether they resulted in a change in diagnosis or management, supported clinical decision making and resulted in no change in diagnosis or management, or did not contribute to diagnosis or management. RESULTS: A total of 200 medical records were reviewed; 110 (55%) were for male patients and 90 (45%) were for female patients, with a mean (range) age of 60 (18-96) years. The most common pre-EEG diagnoses were altered mental status (52 [26%]) and seizure (48 [24%]). Of all EEGs, 115 (57.5%) had findings that were normal, 83 (41.5%) had findings that were abnormal, and 2 (1%) had findings that were uninterpretable. No EEGs had findings that resulted in a change in diagnosis or management, 8 EEGs (4%) had findings that supported clinical decision making and resulted in no change in diagnosis or management, and 192 EEGs (96%) had findings that did not contribute to diagnosis or management. CONCLUSION: In this study, inpatient EEGs rarely contributed to clinical decision making and in no case resulted in a change in diagnosis or management. These findings warrant future research on the effectiveness of inpatient EEGs for a wide breadth of clinical inpatient diagnoses.

Modified Phalen's test as an aid in diagnosing carpal tunnel syndrome.

OBJECTIVE: We developed a modified Phalen's test (MPT), which uses sensory testing in Phalen's position, as a diagnostic screening tool for carpal tunnel syndrome (CTS). This study was designed to determine the sensitivity, specificity, and receiver operating characteristic (ROC) curve of the MPT for diagnosis of CTS. METHODS: Electrodiagnostic nerve conduction studies (EDS) were used as the gold standard. MPT was performed by a blinded examiner on patients prior to EDS. MPT was recorded as either positive or negative based on the presence or absence of a median nerve sensory deficit while the hand was in Phalen's position. RESULTS: Sixty-six hands were included in this study. Chi-square for the MPT compared to EDS as the gold standard was 41.449 (P < 0.001), and the validity coefficient (phi) was 0.792 (P < 0.01). The traditional Phalen's test (TPT) compared to the EDS demonstrated a chi-square of 15.349 (P < 0.001) and a phi coefficient of 0.482 (P < 0.01). ROC curve estimates for the MPT revealed a sensitivity of 84.4% compared to 50% for the TPT. The standard error of the estimate for sensitivity was 3.3% for the MPT and 5.8% for the TPT. CONCLUSION: The MPT is a highly useful screening diagnostic tool for CTS. The MPT demonstrates greater accuracy than the TPT for predicting CTS. The MPT also demonstrates greater sensitivity than the TPT in predicting a positive electrodiagnostic test for CTS.

Autoimmune sensorineural hearing loss: clinical course and treatment outcome.

BACKGROUND: Autoimmune sensorineural hearing loss (ASHL) originally was defined both by the presence of progressive hearing loss (with or without vertigo) and a positive response to glucocorticoid therapy. Subsequently, antibodies to a 68-kd inner-ear antigen were identified in a high percentage of patients with ASHL. OBJECTIVE: To analyze the disease progression and treatment outcome of ASHL. METHODS: Retrospective chart review of 30 patients with a diagnosis of ASHL referred to Texas Tech Rheumatology Clinic. All subjects were tested for anti-68-kd antibody, had audiometric evidence of hearing loss, and were treated with glucocorticoids. RESULTS: The median duration from onset of symptoms to audiometric testing was 25.5 weeks (mean, 144 weeks; range, 3-1,040 weeks), and to testing for anti-68-kd antibody was 40 weeks (mean, 157.3; range, 4-1,092 weeks). Ninety percent of subjects tested positive for anti-68-kd antibodies. By audiometric testing, 50% of subjects were steroid-responsive; minimal improvement or no change was recorded in 12%, and worsening occurred in 39% after steroid therapy. The 3 subjects who were anti-68-kd antibody-negative were steroid-unresponsive, and 1 progressed to complete deafness. CONCLUSIONS AND RELEVANCE: This series of patients with ASHL suggests a more variable and benign course with a better prognosis than previously reported. Immunosuppressive therapy other than steroids rarely was required, and rapid progression to complete hearing loss was very uncommon in this cohort.