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Proximal-distal motor unit number estimationdynamic changes in patients with amyotrophic lateral sclerosis A one-year follow-up

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Neural Regeneration Research 2010年第8期5卷 597-601页

作者： Sartucci Ferdinando Moscato Gianluca Rossi Chiara Caleo Matteo Bocci Tommaso Murri Luigi Giannini Fabio Rossi Alessandro Department of Neuroscience Unit of NeurologyPisa University Medical School Institute of Neuroscience unit of neurophysiopathology Pontedera Hospital Pisa Department of Neurological Neurosurgical and Behavioural Sciences Siena University Medical School

BACKGROUND：Amyotrophic lateral sclerosis （ALS） is the most common of all the motor neuron diseases and the absence of a biologic marker has made both diagnosis and tracking evolution of the disease difficult, Electrodiagnostic tests play a fundamental role in quantifying pathological changes in the motor unit ***：We assessed distal-proximal Motor unit （MU） loss and changes using the method of motor unit number estimation （MUNE）.DESIGN, TIME AND SETTING：A case-control study was performed at the Department of Neuroscience, Pisa University Medical School, Italy from December 1999 to November 2009. PARTICIPANTS：A total of 50 ALS patients were recruited, 30 males：mean age （59.6 ± 13.3） years; 20 females：mean age （63.9 ± 11.7） years; range （30-82） years; all patients had probable or definite ALS. Thirty healthy volunteers were recruited from department staffs, including 20 males and 10 females; mean age （57.7 ± 13.8） years served as ***：MUNE was performed for both the biceps brachii and abductor digiti minimi muscles of the same side. The technique used relayed substantially on manual incremental stimulation of the motor nerve, known as the McComas technique （50 ms sweep duration, a gain of 2 mV/Div for M wave, 0.5 mV/Div for each step; filters 10-20 kHz）.MAIN OUTCOME MEASURES：MUNE results were ***：Functioning MU numbers, measured by MUNE, decreased in the biceps brachii and abductor digiti minimi muscles over the entire one-year follow-up period （one assessment every three months） compared with baseline determination, the rate of MU decrease was similar in both muscles, but steeper ***：MUNE is a feasible method for ALS patients both proximally and distally to track changes over time in muscle MUs during the disease＇s evolution.

关键词： motor unit number estimation amyotrophic lateral sclerosis motor neuron disease time changes denervation reinnervation

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左乙拉西坦在MERRF综合征中的抗肌阵挛作用

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世界核心医学期刊文摘(神经病学分册) 2006年第8期 30-31页

作者： Mancuso M. Galli R. Pizzanelli C 张玉春 unit of neurophysiopathology Hospital Lotti Pontedera Italy

The treatment of progressive myoclonic epilepsy (PME)is largely empirical, even though valproic acid (VPA) is usually considered the drug of first choice. However, VPA should be used with caution in PME due to mitochondrial dysfunction, i.e. in MERRF (myoclonic epilepsy with ragged red fibers) syndrome, because of its interaction with mitochondrial respiration and metabolism. Levetiracetam (LEV) treatment was started in combination with VPA in a patient with typical clinical, histological, and biochemical features of MERRF due to a mutation on the tRNA of Phenilalanine gene. The average myoclonus score improved dramatically, as well as the quality of life and no side effects were observed, even after having withdrawn VPA. LEV may benefit myoclonus in PME of mitochondrial origin without altering mitochondrial function, and it could be considered the drug of first choice for the treatment of myoclonus in MERRF.

关键词：肌阵挛患者神经肌肉疾病西坦 MERRF 综合征综合病症

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