ALS is a fatal motor neuron disease characterized by muscle weakness and disability in both the upper and lower motor neurons. It is usually sporadic but can be inherited. The disease pathology involves the degeneration of motor neurons in the brain stem and spinal cord. Initial symptoms often include asymmetric weakness in the extremities that progresses to involve trunk and bulbar muscles. Management focuses on palliative care through respiratory support, physical therapy, and medications like riluzole to delay disease progression and improve survival.
ALS is a fatal motor neuron disease characterized by muscle weakness and disability in both the upper and lower motor neurons. It is usually sporadic but can be inherited. The disease pathology involves the degeneration of motor neurons in the brain stem and spinal cord. Initial symptoms often include asymmetric weakness in the extremities that progresses to involve trunk and bulbar muscles. Management focuses on palliative care through respiratory support, physical therapy, and medications like riluzole to delay disease progression and improve survival.
ALS is a fatal motor neuron disease characterized by muscle weakness and disability in both the upper and lower motor neurons. It is usually sporadic but can be inherited. The disease pathology involves the degeneration of motor neurons in the brain stem and spinal cord. Initial symptoms often include asymmetric weakness in the extremities that progresses to involve trunk and bulbar muscles. Management focuses on palliative care through respiratory support, physical therapy, and medications like riluzole to delay disease progression and improve survival.
ALS is a fatal motor neuron disease characterized by muscle weakness and disability in both the upper and lower motor neurons. It is usually sporadic but can be inherited. The disease pathology involves the degeneration of motor neurons in the brain stem and spinal cord. Initial symptoms often include asymmetric weakness in the extremities that progresses to involve trunk and bulbar muscles. Management focuses on palliative care through respiratory support, physical therapy, and medications like riluzole to delay disease progression and improve survival.
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Amyotrophic Lateral Sclerosis (ALS)
Definition
ALS is a fatal degenerative disorder that is characterized by muscle
weakness and disability due to both upper and lower motor neuron lesions.
Epidemiology
The disease is usually sporadic; genetic inheritance is less common. It is
the most common disorder of the motor neuron system. It is a fatal disease with a median survival rate of three years, but this can be extended with the right treatment.
Pathology
The upper motor lesions are due to the involvement of neurons in the corticospinal tract of the spinal cord, medulla, pons, internal capsule and Brodman area 4 in the cortex. The lower motor lesions are due to the involvement of motor nuclei in the brain stem and spinal cord.
Clinical presentation of ALS
The initial presentation of ALS is asymmetric extremity weakness affecting
the upper or lower limbs. Foot drop or lateral hand weakness and atrophy are common initial presentations.
Weakness progresses to the axial trunk muscles, with a head drop, or bulbar muscles with dysarthria and dysphagia; respiratory muscles are affected in rare cases. Upper motor neuron manifestations include spasticity, hyperreflexia, and ankle clonus. Bulbar upper motor neuron symptoms include dysphagia and dysarthria. Sometimes, pseudobulbar effect, which is inappropriate laughing or crying spontaneously triggered by stimuli, occurs. Lower motor neuron manifestations include weakness, muscular atrophy, and fasciculation.
Image: “Amyotrophic lateral sclerosis. MRI (axial FLAIR) demonstrates increased T2 signal within the posterior part of the internal capsule, consistent with the clinical diagnosis of ALS” by Frank Gaillard. License: CC BY-SA 3.0
Autonomic manifestations present with urinary urgency, constipation, and
sweating. Cognitive impairment with dementia can also be found in up to 50% of patients.
Diagnosis is clinical, requiring a careful history and clinical
examination. Electromyography can be applied, with muscle biopsy and neuroimaging, to exclude other causes of upper or motor neuron disease. Electromyography results will show combined acute and chronic denervation.
Management of Neuromuscular Diseases
Managing acute occurrences of neuromuscular diseases includes respiratory support, pain medications, and muscle relaxants. Long-term therapy involves pain control, muscle relaxation, and physiotherapy. Riluzole is proven to delay disease progression and, hence, the need for ventilation support. It improves survival for a few months. Enteral nutrition, via cutaneous gastrostomy tube, is encouraged to the boost patient’s weight. Creatine and high dose vitamin E should be avoided.
Management with respiratory support, physical therapy, pain
medications, and muscle relaxants can help as palliative therapy for patients with ALS. Riluzole is proven to delay the need for ventilation support and improve survival for a few months.
