As there is currently no cure for ALS and the mean survival time from symptom onset is 34 -years, it is important to consider all potentially treatable diseases that might produce a similar syndrome.
The role of neuroimaging in the investigation of suspected ALS is most frequently to exclude cervical spine pathology, which can present clinically with upper and lower motor neuron signs in the limbs. In this case MRI conclusively excluded compressive pathology. Cerebral MRI can also be useful in patients with bulbar dysfunction to exclude an alternative cause such as stroke, demyelination or a brain stem tumor. Although hyperintensity of the corticospinal tracts on MRI particularly with fluid-attenuated inversion recovery sequences-is a well established phenomenon in ALS casesthis sign has low diagnostic sensitivity and specificity. The hyperintensity seen in the present case, which was particularly extensive in the region of the precentral gyrus, showed an unusual degree of confluence.
The lesions in the present case were revealed by MRI to have some features in common with those seen in progressive multifocal leukoencephalopathy. The effects of progressive multifocal leukoencephalopathy are usually widespread, but a case has been described in which the lesions were confined to the pyramidal tract,For this reason, the present patient was tested for HIV and JC virus, with both tests being negative.
EMG can provide supportive evidence in the investigation of a case of suspected ALS, but it is only diagnostic in the context of a compatible clinical history and examination. The slow NCV will mean the patient may have CIDP and conduction block will show that the condition is MMN multifocal motor neuropathy. Both of latter conditions respond to IVIg.
In published reports of neurological complications of celiac disease, improvement with dietary modification hasThe patient discussed in this Case Study presented with a syndrome of progressive hemiparesis associated with gluten-sensitive enteropathy. The clear clinical and neuroradiological improvements that followed treatment with a gluten-free diet are evidence that the syndrome of progressive hemiparesis represents another addition to the growing list of neurological manifestations of celiac disease. The symptoms displayed by the patient in this case were initially suspected to result from ALS. This condition remains predominantly a clinical diagnosis, and the aim of diagnostic investigations is the exclusion of alternative ;mimic; syndromes that might be responsive to treatment. ALS is a condition with relentless progression; for this reason, the simple observation of an improvement in symptoms is most pertinent in rendering the diagnosis of ALS untenable. It has been reported. Lack of improvement in ALS might be the result of unintentional poor dietary compliance, caused by variable food labeling practices and the fact that even minute traces of gluten, enough to perpetuate the immune response, can persist on cooking implements. The patient discussed here is currently following a wheat-free diet
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