Nonmotor Symptoms in Amyotrophic Lateral Sclerosis and Their Correlation With Disease Progression.

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 = 0.002) and between the NMS score corresponding to mental health and Amyotrophic Lateral Sclerosis Functional Rating Scale score (P = 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.

Characteristics of Guillain-Barré syndrome in a Lebanese tertiary care center.

Guillain-Barré Syndrome (GBS) is a group of acute inflammatory disorders that share a clinical presentation of progressive polyradiculo-neuropathy. Data on GBS in the Middle East and Lebanon are scarce; hence, we explored the characteristics of patients presenting with GBS to a tertiary care center in Beirut, Lebanon. This was a single-centered retrospective study over a 12-year period. We reviewed the charts of patients presenting with GBS to the American University of Beirut medical center and examined their presentation, management and outcome. 61 patients were included, with the majority being males. 59% of the patients reported an infection prior to admission. 77% had sensory and motor symptoms and 69% were diagnosed with acute inflammatory demyelinating polyneuropathy (AIDP). 57% of patients had initial symptoms in the lower extremities, 25% experienced cranial neuropathies, and 26% complained of pain. 77% were managed by intravenous immunoglobulin with a median hospital stay of 6.5 days. AIDP was noted to be the most prevalent GBS variant in Lebanon. More than 50% had an unfavorable outcome at discharge, which raises the need for better treatment and management approaches.

Absent sural responses in tethered cord syndrome.

Context: Tethered cord syndrome is a progressive condition usually diagnosed early in life, and leads to a diversity of manifestations including neurologic, urologic, and orthopedic dysfunction.Findings: We report two cases of tethered cord syndrome associated with a unilaterally absent sural sensory response on nerve conduction studies while other causes of this finding being excluded.Conclusion/clinical relevance: We believe that this finding is caused by a selective injury of sensory fibers at the level or distal to the dorsal root ganglia.

Kennedy's disease: an under-recognized motor neuron disorder.

Kennedy's disease or spinal bulbar muscular atrophy is a rare, inherited and slowly progressive multisystem disease mostly manifesting with a motor neuron disease phenotype leading to disability. The slow progression, partial androgen insensitivity, electrophysiological evidence of sensory neuronopathy, and relatively spared central nervous system pathways help differentiate it from amyotrophic lateral sclerosis. To date, there is no treatment or cure with clinical care mainly focused on accurate diagnosis, symptom management, patient education, and genetic counselling.

Characteristics of amyotrophic lateral sclerosis in Lebanon-a chart review.

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder primarily manifesting as motor deficits. It is caused by motor neuron death and leads to progressive disability and demise. It can present at any age, manifest as several phenotypes, and may have a variable progression pattern. Methods: This retrospective study is based on chart review of subjects presenting to the American University of Beirut Medical Center from June 2015 till March 2020. It aims to describe the characteristics of ALS in Lebanon. Results: Out of 140 subjects identified, 113 had classical ALS. The mean age in classical and atypical ALS were 55.5 and 55.6 years, male gender was predominant in both groups, and the mean duration from disease onset to diagnosis was 10 months in classic ALS compared to 22 months in atypical ALS. The median survival in subjects with classical ALS was 31 months which was significantly lower than atypical ALS phenotypes of 41.5 months. Additionally, more than half of patients (57%) were found to have a moderate ALSFRS-R progression rate. Conclusions: The study is the first to report the characteristics of amyotrophic lateral sclerosis in Lebanon. Moreover, we were able to categorize patients with classical phenotype according to disease progression using the ALSFRS-R score which can be a useful tool in determining disease prognosis at an early stage.

Adult onset Sandhoff disease: a rare mimicker of amyotrophic lateral sclerosis.

Sandhoff disease is an under-recognized disease that may present as a lower motor neuron disorder in adulthood. We report the case of siblings presenting in their late 40s with a motor neuron disease phenotype and were misdiagnosed as amyotrophic lateral sclerosis and later found to have Sandhoff disease. Sandhoff disease should be considered in patients presenting with a slowly progressive predominately lower motor neuron disorder. A simple low-cost blood test can confirm the diagnosis.

TOAST classification and risk factors of ischemic stroke in Lebanon.

OBJECTIVES: Ischemic stroke is a common cause of morbidity and mortality especially in the increasingly older population. The variability in ischemic stroke subtypes and its risk factors across different populations suggest that more effort is needed to describe the stroke characteristics in order to develop a more tailored management for each population. We aim to describe the demographic characteristics, risk factors, and subtype distribution of subjects with ischemic stroke in the Lebanese population. MATERIALS AND METHODS: We conducted a cross-sectional study based on chart review on patients with ischemic stroke/transient ischemic attack at the American University of Beirut Medical center between 2015 and 2017. RESULTS: A total of 284 cases were included with a mean age of 72 years, and 58% male gender. The most commonly identified risk factors were hypertension (77%), dyslipidemia (62%), and diabetes mellitus (42%), while atrial fibrillation was only found in 27% of cases. The cohort distribution according to TOAST classification was as follows: 15% large artery stroke, 31% cardioembolic stroke, 17% small artery stroke, 10% stroke of other determined causes, and 27% stroke of unknown cause. CONCLUSION: This is the first study to address ischemic stroke characteristics in Lebanon.

Successful Thrombolysis in the Setting of Marfan Syndrome and Chronic Aortic Dissection.

INTRODUCTION: Thrombolysis is the standard of care in acute ischemic stroke. It is usually contraindicated in patients with aortic dissection. CASE REPORT: We report a case of Marfan syndrome and chronic aortic dissection presenting with acute ischemic stroke treated with thrombolysis. CONCLUSION: To our knowledge, this is the first case of Marfan syndrome with chronic aortic dissection undergoing thrombolysis for acute ischemic stroke with a near-complete recovery of neurological deficit and favorable long-term outcome.

Common Entrapment Neuropathies.

Entrapment neuropathies are defined as compression of peripheral nerves due to known or unknown causes. The high incidence and variety of presentations require a comprehensive knowledge of these conditions, especially in neurology and orthopedic surgery clinical practices. Detailed knowledge of topographic anatomy, clinical manifestations, and appropriate use of electrophysiological studies with selective addition of neuromuscular ultrasonography are needed to establish an early and accurate diagnosis to advice patients and provide them with a comprehensive treatment plan. In this article, we discuss the most common forms of entrapment neuropathies in the upper and lower extremities.

Guillain-Barre Syndrome.

Guillain-Barre syndrome (GBS) is the leading cause of acute paralysis that can potentially affect all of the human population. GBS is believed to be an immune-mediated disease, possibly triggered by a recent infection, and driven by an immune attack targeting the peripheral nervous system. GBS can be divided into several subtypes depending on the phenotype, pathophysiology, and neurophysiological features. Unfortunately, morbidity and mortality rates are still high despite the current understanding of the pathophysiology and available treatment options. Additional research is still needed to shed more light into the pathogenesis for a better understanding and treatment of this condition.