Amyotrophic lateral sclerosis (ALS) - a case report
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by rapidly progressive muscular paralysis caused by degeneration of motor neurons leads to muscle wasting (atrophy), muscle spasticity, muscle weakness. Respiratory failure limits survival to 2-5 years after disease onset.

Both upper motor neuron and lower motor neuron gets affected. It is otherwise known as Lou Gehrig’s disease or motor neuron disease. The clinical presentation of amyotrophic lateral sclerosis is variable. Typically, there is both upper motor neuron and lower motor neuron sings along with electrodiagnostic studies which is indicative of ALS.

Published in the Indian Journal of Pharmacy Practice, the authors present a case of a 54-year-old male patient with Amyotrophic Lateral Sclerosis. The patient reported with features of quadriparesis of 1 year duration. Limb weakness started in each limb and slowly progressed to involve all four limbs. The patient also has difficulty in walking due to severe stiffness in both lower limb (scissoring gait).

Now the patient is bedridden. He also has dysarthria with stiff tongue (features of pseudobulbar palsy). Jaw jerk position with bilateral upper motor neuron type facial palsy. Limb examination showed signs of bilateral pyramidal tract disease (extensor plantar bilaterally, spasticity, clonus and exaggerated refluxes) and signs of anterior horn cell damage (severe muscle atrophy).

Fasciculation absent (burnt out disease) sensory spincture whereas sensorium is normal . It was finally diagnosed as MND-ALS was made because of limbs having both UMN and LMN signs which was confirmed by nerve conduction study.

He was treated with neuroprotective agent Gabapentin 300 mg BD, Tizanidine 1 mg thrice a day and Baclofen 10 mg thrice a day for muscle relaxation and whereas Riluzole which is anti-glutaminergic drug 50 mg OD was given for all the days. Following the given therapy patient reported symptom relief and was discharged.

Clinical pearls:-
• Amyotrophic lateral sclerosis should be considered in patients with a rapidly progressive, unexplained neuropathic process

• The early diagnosis is essential for the prevention of disease progression

• The incidence of ALS is increasing every year so efforts must be taken to promote awareness of the disease and encourage the research for ALS management

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A P●●●l General Medicine
Recent research in Australia ( stage 1 trial ) suggested use of CuATSM has reversible effects on disease .
Feb 8, 2019Like