Faculty Bibliography

We tested patients with celiac disease (CD) for the presence of serum anti-ganglioside antibodies. Six of twenty-seven patient sera were reactive against brain gangliosides by an agglutination immunoassay. Neurological examination in all six revealed the presence of distal sensory loss, consistent with the diagnosis of peripheral neuropathy. When tested by ELISA for antibodies to isolated GM1, GM2, GD1a, GD1b, GT1b, and GQ1b gangliosides, all six were positive for IgG antibodies to at least one. The neuropathy of celiac disease may be autoimmune and associated with anti-ganglioside antibodies. The presence of IgG reactivity furthermore implicates a T cell-mediated response to ganglioside antigens

We evaluated the diagnostic sensitivity of quantitative sensory testing (QST), using the CASE IV system, in 14 patients with clinically diagnosed small-fiber neuropathy and normal traditional electrodiagnostic studies. All patients had at least 1 abnormal threshold, 13 patients had abnormal heat-pain thresholds, 8 had abnormal cold thresholds, and 7 had abnormal vibration thresholds. QST is therefore highly effective in documenting dysfunction in small fiber neuropathy patients

OBJECTIVES: Proximal myotonic myopathy (PROMM) is a multisystem disorder that may mimic myotonic dystrophy (MD). Previously we demonstrated that the 60 s exercise test was normal in two siblings with PROMM. The test enabled distinction of PROMM from MD, as there is a well documented immediate post-exercise compound muscle action potential (CMAP) amplitude decline in MD. METHODS: We now performed exercise testing using several exercise durations in 8 PROMM patients from 6 kinships, and one MD patient, extending our previous observations. Repetitive stimulation and needle electromyography findings were also recorded. RESULTS: The 10 (n = 8), 30 (n = 5), and 60 (n = 5) s, and the 5 min (n = 1) exercise tests were normal in all PROMM patients. Specifically, the maximum post-exercise CMAP amplitude decline was 8%. In contrast, the MD patient had CMAP amplitude declines of 48% (10 s exercise test) and 26% (30 s exercise test). The distribution of repetitive stimulation and motor unit duration abnormalities were variable and less diagnostically useful. CONCLUSIONS: The 10, 30, and 60 s exercise tests help distinguish PROMM from MD. As the 10 s exercise test is rapid and easily tolerated, we recommend this test for clinical testing

Current Perception Threshold (CPT) evaluation quantifies the sensory threshold to transcutaneous electrical stimulation of three sensory fiber subtypes: A-beta (2,000 Hz), A-delta (250 Hz) and C fibers (5 Hz). Demyelinating polyneuropathies tend to affect larger myelinated fibers before smaller unmyelinated fibers, and they usually begin at the proximal nerve roots or terminal axons, due to relative weakness of the blood-nerve barrier in these locations. Axonal polyneuropathies tend to affect smaller fibers before larger fibers, in a distal to proximal gradient. Ten patients with demyelinating polyneuropathy and ten patients with axonal polyneuropathy underwent CPT testing. CPT comparisons were made with regard to side-to-side asymmetries, fiber type involvement, and the ratio of fiber types involved. The C2, lateral antebrachial cutaneous, and sural distributions were examined bilaterally. Demyelinating polyneuropathies were detected with 50% sensitivity and 100% specificity. This diagnostic sensitivity is similar to that of published criteria based upon motor nerve conduction. CPT testing can distinguish demyelinating from axonal polyneuropathies. It may be particularly helpful in patients with predominantly sensory symptoms in whom EMG/NCS data may be equivocal, or in patients who decline EMG/NCS studies

We describe two modified methods for median-to-ulnar motor conduction comparison in the diagnosis of median neuropathy at the wrist: the median-thenar to ulnar-thenar latency difference (TTLD), and the median-thenar to ulnar-hypothenar latency difference (THLD). We also describe an F-wave ulnar-to-median comparative test, the F-wave latency difference (FWLD). The abnormal cutoffs based upon 34 normal controls are: TTLD, 0.8 ms; THLD, 1.2 ms; FWLD, 0.6 ms. In 50 patients (79 hands) with clinically defined carpal tunnel syndrome and electrophysiological evidence of median neuropathy at the wrist (based upon a prolonged median nerve palm-wrist latency), the diagnostic sensitivities were: 95-98%, 85-88%, and 75-78%, respectively. These tests are therefore highly sensitive. They are easily performed and require minimal additional effort to incorporate into commonly used clinical electrodiagnostic routines. They may be advantageous when a concomitant polyneuropathy is present, and they may also help avoid technical pitfalls and aid in identification of anatomic variants

In accessory neuropathy electrodiagnosis, upper trapezius compound muscle action potential (CMAP) latencies and amplitudes are commonly measured. The few prior reports describing middle and lower trapezius recording have traditionally emphasized latency value determination. The utility of amplitude measurement with middle and lower trapezius recording has not, to our knowledge, been previously described in individual patients with accessory neuropathy. We report three patients (A-C) who developed unilateral accessory neuropathy following surgical procedures. Accessory nerve conduction studies were performed with surface recording over the upper, middle, and lower trapezius muscles. Latency values were normal except for a prolonged lower trapezius latency value in patient B. Side-side trapezius amplitude comparisons revealed striking asymmetries from all three recording sites in patients A and B (71-95% CMAP amplitude decrements) and in the lower trapezius recording of patient C. Middle and lower trapezius side-side CMAP amplitude comparisons may increase the sensitivity of accessory neuropathy electrodiagnosis