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Motor Neurological Examination of the Hand and Upper Limb
Published in J. Terrence Jose Jerome, Clinical Examination of the Hand, 2022
The conditions that can cause motor neurone disease (MND) could be amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), pseudobulbar palsy, progressive muscular atrophy (PMA), primary lateral sclerosis (PLS) and monomelic amyotrophy (MMA), as well as some rarer variants resembling ALS.
Characteristics of amyotrophic lateral sclerosis in Lebanon-a chart review
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
Elia Malek, Helen Ismail, Hassan Doumiati, Johnny Salameh
This is a retrospective, chart review based study, approved by the institutional review board at the American university of Beirut medical center (ID:IM-JS-01). All patients with ALS who presented to the American University of Beirut Medical Center (AUBMC) between June 2015 and March 2020 were included. The medical center is located in Beirut (capital of Lebanon) and is considered the main referral center with the only actively functioning ALS clinic in the country. Subjects were classified as: classic ALS if they had clinical and electromyography (EMG) evidence of upper and lower motor neuron involvement; PLS if they had clinical and motor evoked potentials (MEP) evidence of pure upper motor neuron involvement for a duration of at least 4 years; PMA if they had clinical and EMG evidence of pure lower motor neuron involvement for a duration of more than 1 year; PBP/IBP if they had exclusive involvement of the bulbar motor neuron group for a duration of more than 1 year; and FAS/FLS if their disease was confined to the motor tracts of the upper or lower limbs respectively. One subject that was classified as ALS-FTD had the typical ALS manifestations mentioned above in addition to fronto-temporal dementia (FTD). The classification of familial ALS was solely based on the family history as reported by patients and their family members (no genetic studies were performed, thus the term familial will be replaced by family history in this article). Assessment of cognitive performance was done using the Montreal Cognitive Assessment scale in case there were complaints reported by patients/family members or in case the primary care physician noted any form of cognitive impairment. All enrolled subjects underwent nerve conduction studies (NCS) and needle electromyography (EMG) that confirmed their diagnosis and excluded mimickers in addition to laboratory investigations as well as imaging of the nervous system to rule out other causes of their symptoms. Patients that progressed to develop symptoms and signs of corticospinal tract and/or anterior horn disorders were labeled as classic ALS. Subjects with classic ALS phenotype were classified into slow, moderate and fast progressors based on their ALSFRS-R score progression according to the following criteria: slow: an average decrease of ≤0.3 points/month; moderate: an average decrease of >0.3 points/month and <1.1 points/month; fast: an average decrease of ≥1.1 points/month. Patients with monomelic amyotrophy (MA) were only mentioned in the distribution of our patients but were not included in any other statistics. Correlation between ALS-FRS-R scores and time since symptom onset was obtained by Pearson correlation coefficient, using IBM SPSS Statistics 25.