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Oddities – unusual non-motor symptoms in 3 ALS patients Authors: Muhammad Khan, MD, Rajan Patel, MD, Katalin Scherer, MD(Associate Professor of Neurology,

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Presentation on theme: "Oddities – unusual non-motor symptoms in 3 ALS patients Authors: Muhammad Khan, MD, Rajan Patel, MD, Katalin Scherer, MD(Associate Professor of Neurology,"— Presentation transcript:

1 Oddities – unusual non-motor symptoms in 3 ALS patients Authors: Muhammad Khan, MD, Rajan Patel, MD, Katalin Scherer, MD(Associate Professor of Neurology, University of Arizona

2 Introduction We present cases of unusual non-motor symptoms associated with ALS. Through our literature review, there are no reports of dysguesia occurring as a result of ALS. It is interesting to note that after rx with riluzole the alopecia improved.

3 Case 1- “woman in the barrel with dysgeusia” A 52-year-old woman had 2 years of progressive arm paralysis, atrophy and fasciculations. She developed malnutrition due to altered taste and required tube feeding despite normal swallow function and felt miserable. She died 5 years after onset from malnutrition and respiratory failure.

4 Case 2- “man in the barrel with dysgeusia” A 36-year-old man has had 3 years of progressive weakness, atrophy and fasciculation of his arms followed by respiratory dysfunction. He reported that spanish rice tasted like lemon. Zinc level was normal.

5 Case 3- “I have hair!” A 50-year-old d man had 1.5 years of progressive bilateral upper and lower limb paralysis, fasciculations, hyperreflexia, bulbar dysfunction. He had male pattern baldness with complete alopecia of the top and the crown. 2 months after starting riluzole new hair grew. 7 months after starting riluzole, the top of the head hairs measured 7cm. He passed away 3 years from onset.

6 BeforeAfter

7 Discussion Well recognized non-motor symptoms include cognitive and sensory disturbances. Zinc deficiency is a known cause of dysgeusia, however this was not the case in our patients. The effect on quality of life in case 1 was profound as she lost the enjoyment of food years before bulbar dysfunction set in. Anosmia has been described in ALS as a potential biomarker and this along with dysgeusia may develop long before the development of motor symptoms. Riluzole may have effects far and beyond its disease modifying effect, the pathophysiology of these effect is unknown.

8 Reference Ward A, et al. B-62Anosmia as a Biomarker for Personality Change in Amyotrophic Lateral Sclerosis. Arch Clin Neuropsychol Sep;29(6):560.doi: /arclin/acu Hammad M, Silva A, Glass J, Sladky JT, Benatar M. Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS. Neurology. 2007;69(24):2236. Isaacs JD, Dean AF, Shaw CE, Al-Chalabi A, Mills KR, Leigh PN. Amyotrophic lateral sclerosis with sensory neuropathy: part of a multisystem disorder? J Neurol Neurosurg Psychiatry. 2007;78(7):750. Santos-Bento M, de Carvalho M, Evangelista T, Sales Luís ML. Sympathetic sudomotor function and amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001;2(2):105. Pugdahl K, Fuglsang-Frederiksen A, de Carvalho M, et al. Generalised sensory system abnormalities in amyotrophic lateral sclerosis: a European multicentre study. J Neurol Neurosurg Psychiatry 2007; 78:746. Feller TG, Jones RE, Netsky MG. Amyotrophic lateral sclerosis and sensory changes. Va Med Mon (1918). 1966;93(6):328


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