"T1: hyperintensity of the tongue may be seen in patients with bulbar involvement, known as the bright tongue sign 13"
"T2: hyperintensity in the corticospinal tracts"
"GRE/SWI: hypointensity in the precentral gyrus bilaterally, known as the motor band sign 8,9,14"
"EMG abnormalities that must include both:"
"decreased NAA"
"Most cases are sporadic, with no known genetic basis. In the familial form of amyotrophic lateral sclerosis, and in some sporadic cases, several gene mutations have been identified (e.g. the hexanucleotide repeat expansion in C9orf72 (most common familial cause), or mutations in SOD1, TDP-43, FUS, or UNC13A) 5,26."
"increased myo-inositol"
"MRI spine is often less helpful, and features of amyotrophic lateral sclerosis are less well defined. Often the role of spinal imaging is to exclude a cervical myelopathy which may mimic some of the clinical symptoms, rather than to find supportive features of amyotrophic lateral sclerosis itself. Nevertheless, there may be a decrease in cross-sectional area of cervical spinal cord, particularly at C4-C7 17. Additionally, in patients with SOD1 mutation, there may rarely be enhancement of the cauda equina roots, mimicking inflammatory pathologies 28."
"Wallerian degeneration"
Expected headings
"Associations"
"Many consider amyotrophic lateral sclerosis synonymous with motor neurone disease 18. In fact, amyotrophic lateral sclerosis is a type of motor neurone disease, accounting for 85% of all cases 18. This article considers only amyotrophic lateral sclerosis; see the article on motor neurone disease for a more general discussion."
"Amyotrophic lateral sclerosis typically is diagnosed in middle age. There is a recognised male predilection 1."
"Most cases are sporadic, with no known genetic basis. In the familial form of amyotrophic lateral sclerosis, and in some sporadic cases, several gene mutations have been identified (e.g. the hexanucleotide repeat expansion in C9orf72 (most common familial cause), or mutations in SOD1, TDP-43, FUS, or UNC13A) 5,26."
"Adjuvant symptomatic measures may include allied health involvement (e.g. speech therapy), symptomatic pharmacotherapy (e.g. for spasticity or cramps), use of noninvasive ventilation (i.e. bilevel positive airway pressure) for type 2 respiratory failure, and percutaneous endoscopic gastrostomy (PEG) or radiologically inserted gastrostomy (RIG) insertion for nutritional support 22."
"Amyotrophic lateral sclerosis was also historically known as Charcot disease in honour of French neurologist Jean-Martin Charcot (1825-1893) who diagnosed and described the first case in the nineteenth century (1865-1869) 12. It is also popularly known as Lou Gehrig disease, especially in North America, in honour of legendary New York Yankees baseball player Henry Louis (Lou) Gehrig (1903-1941), who was diagnosed with the disease in 1939."
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