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Electrophysiologic Examination (electrophysiologic + examination)
Selected AbstractsCMT1A Associated With The 17p11.2 Duplication: Differential Features And CorrelationJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2001D Pareyson A duplication on chromosome 17p11.2 encompassing the gene coding for the peripheral myelin protein-22 (PMP22) is the most common genetic abnormality underlying Charcot-Marie-Tooth disease (CMT). We report clinical and electrophysiologic features of our series of CMT1A patients harboring the duplication. There were 92 patients from 53 families representing 42% of all CMT index cases (n = 125) and 64% of CMT1 probands (n = 83). In CMT1A patients, mean age at onset was 9.7 ± 11.4 and was significantly lower than in non-duplicated CMT1 cases (12.6 ± 9.7, p < 0.01) and CMT2 cases (21.5 ± 17, p < 0.001). Clinical severity was similar to that of CMT2 patients, but significantly milder than in non-duplicated CMT1 cases. Pes cavus, upper limb involvement, deep tendon reflexes abnormalities, and sensory loss were more frequent compared to CMT2. Electrophysiologic examination revealed motor and sensory conduction velocity (MCV, SCV) slowing below 32 m/s in upper limbs. MCV and SCV were significantly lower than in non-duplicated CMT1 patients. Amplitudes of upper limb compound muscle action potentials (CMAPs) and of upper and lower limb sensory action potentials (SAPs) were significantly lower than in CMT2, paralleling clinical differences. Clinical severity correlated with CMAP amplitudes and with disease duration. On the other hand, MCV slowing was not correlated with either severity or duration of the disease. We found a direct correlation between age at onset and upper limb MCV slowing. Conclusions: CMT1A is an early-onset but slowly progressive disorder, on average milder than other CMT1. Axonal loss rather than demyelination per se underlies disease progression. [source] SUB-CLINICAL PERIPHERAL NERVE INVOLVEMENT IN PSORIATIC ARTHRITISJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2000C. Di Girolamo Immunological studies document the role of HLA in psoriasis and the correlation between neuropeptides, psoriasis, and related arthritis. Some anecdotal case reports, moreover, describe a noncasual association between peripheral neuropathy and psoriatic manifestations. To verify a possible subclinical peripheral nerve involvement in this disimmune pathology, we started a pilot study in twenty patients with psoriatic arthritis and in whom other common causes of peripheral neuropathies had been ruled out. We performed a complete clinical neurological examination and a neurophysiological examination (orthodromic sensory and motor nerve conduction velocity in median and tibial nerves; antidromic sensory nerve conduction velocity in sural nerve). In 40% of the patients there was a mild but definite "glove-stocking" hypoesthesia, while hypopallesthesia was detected in only 20%. Electrophysiologic examinations were less informative borderline distal conduction velocities in 30% of patients. These preliminary data suggest a peripheral nerve involvement in this pathology, mainly affecting the small nerve fibres. [source] Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 81JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2003S Lori Symptomatic neuropathy in young patients with type 1 Diabetes Mellitus (t1DM) is rare but subclinical peripheral alterations can be assessed by electroclinical evaluation. This study aimed to assess prevalence of clinical and subclinical peripheral neuropathy in patients with t1DM. Motor and/or sensory nerve conduction studies of both median, ulnar, peroneal, tibial and sural nerves and standard clinical examination of peripheral nervous system were performed in 83 patients (27 females and 56 males) with diabetes onset since five years. The mean age of patients was 19.89 (range 9,28.3) years, the mean disease duration was 9.61(range 4.4,19.3) and the mean age at the onset of diabetes was 9.02 (range 0.8,23.5). Five patients (6.02 %) had both symptomatic (light clinical abnormalities as paresthesias and mild reduction of vibratory sensibility) and electrophysiologic neuropathy and six (7.2 %) with mild abnormal nerve conduction studies were totally asymptomatic (subclinical neuropathy). The majority of symptoms and electrophysiological alterations were found on the lower limbs. Only two patients had a minimal distal neuropathy of median nerve. No patients showed laboratory evidence of early renal complications or systemic hypertension; 5 (6.02 %) had early diabetic retinal abnormalities as microaneurisms, seen by fundus examination. Analysis of sex, age of onset, duration of diabetes, age at the date of electrophysiologic examination, Hemoglobin A1c (mean level of the last two years), association with retinal abnormalities and clinical assessment was performed (Fisher Exact Test, ANOVA). No correlation was found with the age at the onset, retinal abnormalities and glycaemic control index. Peripheral neuropathy was significantly related with patient age at the date of electrophysiological study and duration of t1DM. [source] A Novel Drug Therapy for Recurrent Laryngeal Nerve Injury Using T-588,THE LARYNGOSCOPE, Issue 7 2007Yuko Mori MD Abstract Objectives/Hypothesis: We have previously shown that gene therapy using Insulin-like growth factor (IGF)-I, glial cell line-derived neurotrophic factor (GDNF), and brain-derived neurotrophic factor (BDNF), or a combination of these trophic factors, is a treatment option for recurrent laryngeal nerve (RLN) palsy. However, there remain some difficulties preventing this option from becoming a common clinical therapy for RLN injury. Thus, we need to develop novel treatment option that overcomes the problems of gene therapy. R(,)-1-(benzothiophen-5-yl)-2-[2-N,N-diethylamino]ethoxy]ethanol hydrochloride (T-588), a synthetic compound, is known to have neuroprotective effects on neural cells. In the present study, the possibility of new drug treatments using T-588 for RLN injury was assessed using rat models. Study Design: Animal study. Methods: Animals were administered T-588 for 4 weeks. The neuroprotective effects of T-588 administration after vagal nerve avulsion and neurofunctional recovery after recurrent laryngeal nerve crush were studied using motoneuron cell counting, evaluation of choline acetyltransferase immunoreactivity, the electrophysiologic examination, and the re-mobilization of the vocal fold. Results: T-588 administration successfully prevented motoneuron loss and ameliorated the choline acetyltransferase immunoreactivity in the ipsilateral nucleus ambiguus after vagal nerve avulsion. Significant improvements of motor nerve conduction velocity of the RLN and vocal fold movement were observed in the treatment group when compared to controls. Conclusion: These results indicate that oral administration of T-588 might be a promising therapeutic option in treating peripheral nerve injury. [source] | ||||||||||