The following medical news items are relevant to diagnosis of Amyotrophic lateral sclerosis:
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Motor weakness should be distinguished from functional weakness due to systemic disease. Patients with true motor weakness will complain of difficulty performing specific tasks or a feeling of heaviness or stiffness in their limbs. Myopathy can be distinguished by proximal weakness (climbing stairs or combing hair), symmetrical distribution, absence of paresthesias and pain, or disturbance of bowel or bladder function. Proximal muscle weakness is more prominent in myopathy, distal weakness in peripheral nerve or anterior horn cell disease. Bulbar weakness, manifest as difficulty speaking and swallowing, is consistent with anterior horn cell disease or neuromuscular junction disorders. Ocular weakness occurs with myasthenia gravis and myotonic or oculopharyngeal dystrophy.
Acute generalized weakness with an onset over hours can be caused by low levels of potassium, calcium, sodium, magnesium, or phosphate; by botulism; or by viral inflammatory myopathy. Spasticity, manifest as increased tone and sudden release (“clasp-knife”), occurs with CNS lesions. Rigidity occurs with extrapyramidal disorders. Functional overlay should be suspected in the presence of ratcheting “give-away” weakness.
Peek sign: Orbicularis oculi weakness is observed on gentle eye closure. After initial complete apposition of the lid margins, they separate within seconds and the white of the sclera peeks through. Ice test: A glove finger filled with crushed ice is placed over the more ptotic eyelid for 2 minutes. An increase in opening of 2 mm or more is a positive result. Quiver eye movements: very fast, small twitch, jerk-like movements of the eyes upon changing the direction of gaze. Unintelligible speech: after prolonged speaking.
Source: Field Guide to Bedside Diagnosis, 2007
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