Spinal Epidural Venous Plexus Pathology in Hirayama Disease.

Hirayama disease is a rare juvenile amyotrophy that is often misdiagnosed as an unrelated, relentlessly progressive disease. We present the case of an 18-year-old man who presented with weakness and atrophy of the right forearm and hand. Dynamic cervical magnetic resonance imaging was used, revealing the classic findings of epidural venous plexus dilation and anterior displacement of the dural sac. In addition, dilation of the external vertebral venous plexus was visualized. We discuss the clinical utility of dynamic magnetic resonance imaging and the underlying pathophysiology of these findings in Hirayama disease.

Spinal stenosis, back pain, or no symptoms at all? A masked study comparing radiologic and electrodiagnostic diagnoses to the clinical impression.

OBJECTIVE: To assess the relations between clinically recognized lumbar spinal stenosis and the conclusions of masked radiologists and electrodiagnosticians. DESIGN: Prospective, masked, double-controlled trial. SETTING: University spine center. PARTICIPANTS: One hundred fifty persons age 55 to 80 years with or without back pain and with or without magnetic resonance imaging (MRI)-demonstrated stenosis, screened for neuropathy risk, previous surgery, or cancer. INTERVENTIONS: Questionnaires on pain and function; ambulation testing and physical examination; and masked electrodiagnotics and MRI. MAIN OUTCOME MEASURE: Diagnostic impressions of the examining clinician, radiologist, and electrodiagnostician. RESULTS: Following application of post hoc exclusion criteria and elimination of patients due to incomplete or inadequate test data, the clinical diagnosis was lumbar stenosis in 50 subjects, back pain in 44 subjects, and no pain in 32 subjects. Radiologic and clinical impression had no relation (P = .80 vs asymptomatic, P = .99 vs back pain controls). Electrodiagnostic impression trended to relate to clinical impression (P = .14 vs asymptomatic, P = .09 vs back pain). Retrospective application of age-related electrodiagnostic norms for paraspinal electromyographic and limb motor unit changes, established in this study, reclassified 13 of the 17 asymptomatic persons whom the electrodiagnostician thought had stenosis. The clinical impression did correspond to history and physical examination findings typically associated with spinal stenosis and to the independent impression of a neurosurgeon who examined MRI and clinical, but not to the electrodiagnostic data. CONCLUSIONS: The impression obtained from an MRI scan does not determine whether lumbar stenosis is a cause of pain. Electrodiagnostic consultation may be useful, especially if age-related norms obtained in this study are applied.

Assessment of the validity of masking in electrodiagnostic research.

OBJECTIVES: Performed by thousands of specialists and researched for >60 yrs, electrodiagnostic testing rates poorly in evidence-based guidelines in part because there are no valid masked research studies. This study assesses whether the electrodiagnostic examination can be adequately masked. DESIGN: Prospective, controlled, masked trial. A total of 150 persons aged 55-80 yrs, thought clinically to be asymptomatic (n = 32), with low back pain (n = 46), or with spinal stenosis (n = 63), underwent needle electromyography and nerve conduction studies by specialists in physical medicine and rehabilitation who were not permitted to ask any clinical information or to perform a formal examination. Examiners rated potential bias and recorded clues that might bias the examination. RESULTS: Examiners noted potential for bias in conversation in 17 and due to pain behavior in one. Half of these potential unmaskings pointed away from the correct clinical diagnosis, leaving potential for bias in <6% of the subjects. The clinical diagnosis had no relationship to electrodiagnostician rating of pain behavior in response to testing or repositioning, to limb atrophy, and to arthritis (P > 0.05 all instances). CONCLUSIONS: The electrodiagnostic examination can be successfully masked. This information will help future research in electrodiagnostics meet the criteria of evidence-based medicine.