Multiperspective and multimeasurement assessment, even when using a validated patient-oriented tool, provided interesting information that confirmed and clarified the clinical neurophysiologic dissociation often observed in carpal tunnel syndrome (CTS) patients. Furthermore, CTS appeared to be an ideal model for evaluating the importance of patient-oriented measurement.

Following the AAEM electrodiagnostic guidelines, we developed a neurophysiological classification of carpal tunnel syndrome (CTS). Six hundred hands with clinical CTS (mean age 51.4 yr., female/male ratio 5.5/1, right/left ratio 1.8/1) were prospectively evaluated and divided into six classes of severity only on the basis of median nerve electrodiagnostic findings: extreme CTS (EXT-absence of thenar motor responses), severe CTS (SEV-absence of sensory response and abnormal distal motor latency-DML), moderate CTS (MOD-abnormal digit-wrist conduction and abnormal DML), mild CTS (MILD-abnormal digit wrist conduction and normal DML), minimal CTS (MIN-exclusive abnormal segmental and/or comparative study), and negative CTS (NEG-normal findings at all tests). Using this neurophysiological classification, the CTS groups appeared normally distributed (EXT 3% of cases, SEV 14%, MOD 36%, MILD 24%, MIN 21%, NEG 3%), and the age of patients and clinical findings appeared to be related to neurophysiological abnormalities. Significant differences in median neurophysiological parameters not included in the classification (such as palm-wrist sensory conduction velocity) were observed in the different CTS groups. The analysis of the groups showed that: 1) the majority of advanced cases (SEV and EXT) occurred in older patients (60-80 years), 2) most of the milder cases (MIN and MILD) occurred in young female patients. The aim of this study was to standardise the neurophysiological evaluation of CTS.

In 43 patients (50 hands) with clinical manifestations of mild‐moderate carpal tunnel syndrome (CTS) and 36 healthy volunteers (40 hands), orthodromic sensory nerve conduction velocity (SNCV) was measured with surface electrodes in the median nerve between the third digit and palm and between the palm and wrist. These figures were used to calculate the ratio of distal to proximal conduction (distoproximal ratio). All 90 hands were also subjected to other nerve conduction studies used for diagnosis of CTS. All control hands presented distoproximal ratios <1.0, reflecting higher conduction rates in the proximal segment. In contrast, 49 of 50 CTS hands (98%) presented reversed ratios (>1.0) indicating compromised proximal conduction. The sensitivity of this test was significantly greater than that of other methods evaluated, including comparative studies and segmental study of the palm‐wrist portion of the median nerve. Segmental study of median SNCV with calculation of the distoproximal ratio is a sensitive technique for diagnosis of CTS in patients with normal findings in standard nerve conduction studies. © 1996 John Wiley & Sons, Inc.

Our findings show that young-adult amyotrophic lateral sclerosis with the predominant upper motor neuron phenotype represents a distinctive clinical variant characterized by a unique clinical pattern, longer survival, and male prevalence.

Forty-eight patients with purely ocular myasthenia were studied. Tensilon test was positive in 46 patients (95%); decremental response from limb muscles was present in 24 patients (50%); anti-AChR antibodies were detected in 20 patients of 44 (45.5%). Twenty-two patients underwent thymectomy, 18 were given corticosteroids, 42 received AChE drugs. At the end of the observation period, 8% of the patients were in remission, 67% were improved, 25% were unchanged. In our experience, the diagnosis of ocular myasthenia relies mainly on clinical data; AChE drugs are not very effective in extrinsic ocular muscles; indications for thymectomy should be restricted to thymoma cases and, perhaps, to patients in the early stages of the disease, within the first year of onset; corticosteroids are effective in most cases, but relapses after withdrawal are not uncommon.

We studied the long-term outcome of prednisone therapy in 104 patients with myasthenia gravis (MG). At the end of the follow-up period, good therapeutic results were recorded in 85 patients (81.7%), poor results in 13 (12.5 %) and no significant change of MG status in 6 (5.8%). We found a correlation between the duration of treatment and the incidence of steroid side effects. The presence of thymoma and severe forms of MG were associated with relapsing disease requiring prolonged corticosteroid regimens. The age at the start of therapy did not influence significantly the response to treatment.

We studied 59 children with myasthenia gravis (MG). Disease onset was pre-pubertal in 26 patients and post-pubertal in 33. The male to female ratio was 0.62 in the early- and 0.17 in the late-onset groups. The frequency of ocular MG was higher in patients with prepubertal onset. Patients with generalized MG generally showed a good response to thymectomy and corticosteroid therapy proved effective with no major side-effects. In our experience early-onset MG has the same course as in adult life. We recommend thymectomy for generalized disease in childhood, except in very young children on account of possible long-term effects on immunological development. Immunosuppressive therapy should be considered in severely affected patients who do not respond adequately to other therapies.

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