Clinical Manifestations of Amyotrophic Lateral Sclerosis

        Amyotrophic Lateral Sclerosis (ALS) symptoms are varies from one person to another according to the muscle groups that are affected by this disease. Tripping, dropping things, abnormal fatigue of the arms and / or legs, slurred speech, difficulty in speaking loud, uncontrollable laughing or crying, and muscle cramps and twitches all the symptoms of ALS. ALS usually starts first in the hand and will cause problems in dressing, bathing, or other simple tasks. It could develop into more on one side of the body and generally goes to the hands or feet. If  start on foot, walking would be difficult. ALS can also start in the throat, causing difficulty swallowing. People with ALS do not lose their ability to see, hear, touch, smell, or taste. bladder and the muscles of the eye are not affected, nor desire and sexual function. The disease does not affect the mind of the patient.

        Weakness may begin from feet, hands, proximal arms, or oropharinx ( by speaking slurred or difficulty swallowing ) dysarthria. Often the hand is affected first, usually asymmetrical. Disturbed gait characteristics because the muscles are weak and footdrop, although the proximal muscles are affected first sometimes. Alternatively, spastic gait disturbances may occur. Slowly becoming more severe weakness and various parts of the body affected.

        Spasm of the muscles (the muscles associated with hypersensitivity) and weight loss (resulting from the combination of the smaller muscles and dysphagia) are characteristic symptoms. Respiration is usually affected late but, sometimes it may be an early manifestation or even the first. Breathing is interrupted by the intercostal muscles and diaphragm paresis, or dysphagia can lead to aspiration and pneumonitis, which may happen eventually clinically unaffected sensation, pain and paresthesias are allowed with this diagnosis, unless there are complications of the disease, (eg, diabetic neuropathy) and bladder function spared. Pain is not an early symptom but may occur later when the limb moves .

        LMN sign must be clear for a valid diagnosis. Fasciculations may be seen on the tongue though without dysarthria. If there is a weakness and torso muscles are shrinking , fasciculations usually have started to appear. tendon reflexes may be increased or decreased. The combination of excessive reflex degan Hoffman sign on with a weak hand and muscle fasciculations, actually a pathognomonic sign of ALS ( except for the motor neuropathy syndrome ). Unequivocal sign of UMN abnormality is Babinsky and clonus . Spastic disorders which can be seen walking without LMN signs in the legs, weakness in the legs may not be found , but as evidenced by the clumsiness and incoordination of irregularities in appearance as it moves .

       Cranial nerve motor nucleus involved in dysarthria, tongue fasciculations and uvula impaired movement. Facial weakness, especially on the mental muscle but usually not prominent. Dysarthria and dysphagia caused by UMN lesion ( pseudobulbay palsy ) is made clear by the movement of the uvula stronger on innervation of on a whim, so uvula can not move as well. But the strong response seen in pharyngeal or impaired reflexes.

         Common manifestation of pseudobulbar palsy is emotionally unstable with a laugh reasonable or more often, crying can be regarded as a reaction to depression because of erroneous diagnosis, better regarded as a reflex release of complex phenomena involved in emotional expression. Death is caused by respiratory failure, aspiration pneumonitis, or pulmonary embolism after a long immobility.

          Progression of the disease Amyotrophic Lateral Sclerosis (ALS) with increased weakness, and more muscles are affected. When the weakness has spread to the trunk of the body, speech, swallowing and breathing become impaired. Finally the required ventilator support. Death is usually the result of complications from inactivity or paralysis of the respiratory muscles.

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