motor neuron disease pdf

0000004878 00000 n 0000009781 00000 n Other motor neuron diseases include hereditary spastic paraparesis, spinobulbar muscular atrophy, and infectious motor neuron diseases including polio and West Nile virus. 0000003913 00000 n 0000107554 00000 n

0000030969 00000 n 0000108203 00000 n [9] TDP-43 is rapidly recruited to double-strand breaks where it acts as a scaffold for the recruitment of the XRCC4-DNA ligase protein complex that then acts to repair double-strand breaks.
Pure upper motor neuron diseases, or those with just UMN findings, include PLS. 0000004658 00000 n 116 0 obj<>stream 0000107743 00000 n %PDF-1.6 %âãÏÓ 0000009818 00000 n xÚb```f``{ÃÀÆÀ ú�Aˆ„€bì,?&00¬IlsN`Š²ÜoX¬“»€�0PÒ(ø¨èäϧԠy˜­Ñ£SVéi¢ŒUÎV‹‹W4CÖ¶Dd&¸1NäH¸ qI©ÕϤ¸İwš^×5®éêa}B'¶á“O—†ÇÅF¦v!•�‡2Vys*�vOõ[´hêªé'…dD‹“Öğt:RöèHå¼|l†Q Gá!G�¹®™A§gl úª=#æPFAAA%µ´€‰ Š„†††AÄP%„”ÜÒ€ &Ş. 0000046380 00000 n 0000007391 00000 n 0000010951 00000 n 0000009021 00000 n 0000004320 00000 n 0000047292 00000 n 0000107814 00000 n trailer 0000009739 00000 n While MND refers to a specific subset of similar diseases, there are numerous other diseases of motor neurons that are referred to collectively as "motor neuron disorders", for instance the diseases belonging to the spinal muscular atrophies group. 0000005220 00000 n ~bA|'1d(�*���1R����dr�&I@l=_�ѿ�ܗ��������|0�܂i��xC���)�Z�v�"0��|�� ���� Some disorders, like ALS, can occur sporadically (85%) or can have a genetic cause (15%) with the same clinical symptoms and progression of disease.[5]. 0000006823 00000 n 0000074832 00000 n [3] Studies into these inherited forms have led to discoveries of various genes (e.g. 0000006938 00000 n 0000107593 00000 n 0000007280 00000 n 0000017409 00000 n [3][5] There can be lower motor neuron findings (e.g. startxref 0000014726 00000 n 0000011620 00000 n 0000032401 00000 n %PDF-1.7 %���� 0000033120 00000 n

Most of these diseases seem to occur randomly without known causes, but some forms are inherited.

12, DecembeR 2011 physiotherapists, occupational therapists, speech pathologists, dieticians, social workers and palliative care teams. 0000028978 00000 n 0000006570 00000 n ALS, PBP, PLS), For patients with MND affecting the LMNs, the EMG will show evidence of: (1) acute denervation, which is ongoing as motor neurons degenerate, and (2) chronic denervation and, By contrast, the NCS in these patients is usually normal. In the United States, the term motor neuron disease is often used to denote amyotrophic lateral sclerosis (Lou Gehrig's disease), the most common disorder in the group. 0000109827 00000 n 0000005448 00000 n [5] Some are fatal (e.g. SOD1) that are thought to be important in understanding how the disease occurs.

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