Objectives:Motor neuron disease is a progressive neurodegenerative disease involving upper and lower motor neurons. Nonmotor symptoms (NMS) are part of disease manifestation. We aim to report the prevalence and severity of NMS in patients with motor neuron disease (MND) in Lebanon.
Methods:Fifty-eight patients diagnosed with MND at the American University of Beirut Medical Center were interviewed using the NMS Scale. The prevalence of these symptoms was assessed and correlated with disease progression.
Results:All our patients had at least 2 NMS with an average total score of 15.8. Symptoms reported in more than half of the patients were fatigue, depression, dysphagia, lack of motivation, pain, change in weight, anxiety, constipation, and lack of pleasure. A significant correlation was found between the total NMS score and Amyotrophic Lateral Sclerosis Functional Rating Scale score (P ¼ 0.002) and between the NMS score corresponding to mental health and Amyotrophic Lateral Sclerosis Functional Rating Scale score (P ¼ 0.012). Patients with bulbar symptoms had a significantly higher NMS score corresponding to gastrointestinal symptoms (P , 0.0001). It is important to note that NMS such as dysphagia could be related to motor neuron involvement.
Conclusions:NMS are commonly reported in patients with MND and seem to positively correlate with disease progression. Treating NMS is a critical aspect of the clinical care delivered to patients with MND to maintain a good quality of life.
associated anti-3-hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) myopathy: Imaging findings on thighmuscle magnetic resonance imaging (MRI) in six patients.
Introduction/Aims
Most patients with myasthenia gravis (MG) develop ocular manifestations during their illness and up to 22% may have isolated ocular myasthenia gravis (OMG). Apraclonidine elevates the eyelid by activating alpha‐2 receptors on Muller's muscle, an accessory eyelid elevator muscle. In this study we evaluate the effect of apraclonidine in alleviating ptosis secondary to MG.
Methods
This clinical trial (NCT05045248) was done at the American University of Beirut Medical Center. Patients with ptosis secondary to MG were administered two drops of apraclonidine 0.5% solution to the most affected eye. We measured palpebral fissure height (PF), marginal reflex distance‐1 (MRD1), marginal reflex distance‐2 (MRD2), and levator function (LF) before drug administration and at 1, 5, 30, and 60 minutes after administration.
Results
Ten participants were enrolled in the study. Improvement in all eyelid measurements was noted in all participants as early as 1 minute after apraclonidine administration. From baseline to 60 minutes after administration, average PF increased from 8.8 ± 1.9 mm to 14.2 ± 2.6 mm, MRD‐1 from 1.7 ± 1.4 mm to 5.4 ± 2.9 mm, MRD‐2 from 7.1 ± 1.3 mm to 8.8 ± 1.7 mm, and LF from 13.4 ± 2.9 mm to 17.5 ± 2.4 mm. All increases were statistically significant.
Discussion
Apraclonidine may alleviate ptosis secondary to MG and may be an effective alternative treatment for this group of patients.
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