Answer for BIR CoW 25 Sep 2022
AMYOTROPHIC LATERAL SCLEROSIS
Findings
Cortical atrophy of bilateral frontoparietal region with symmetrical T2 /FLAIR hyperintensities noted along bilateral corticospinal tracts from precentral gyrus to the level of midbrain with patchy diffusion restriction and corresponding low ADC values. IMPRESSION: Symmetrical T2 /FLAIR hyperintensities along bilateral corticospinal tracts with patchy diffusion restriction-Features suggestive of amyotrophic lateral sclerosis.
Discussion
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a fatal neurodegenerative disorder affecting upper motor neurons (UMN) in the motor cortex as well as lower motor neurons (LMN) in the brain stem and spinal cord. Patients with ALS suffer from a progressive paralysis of the skeletal muscles, resulting in loss of voluntary movement, ability to speak and eat, and eventual loss of respiratory function The cause of ALS is generally unknown, although approximately 2% of cases are due to mutations in the superoxide dismutase gene The diagnosis of ALS is currently based on clinical features, electromyography, and the exclusion of other diseases with similar symptoms Consistent features in MRI that are indicative but not specific for the diagnosis of ALS include hyperintensity in the CST, hypointensity in the motor cortex, and brain atrophy T1: hyperintensity of the tongue may be seen in patients with bulbar involvement, known as the "bright tongue sign" T2: hyperintensity in the corticospinal tracts GRE/SWI: hypointensity in the precentral gyrus bilaterally, known as the "motor band sign" MR spectroscopy 2 -decreased NAA -decreased glutamate -increased choline -increased myo-inositol
REFERENCES:
1.Rowland LP. Diagnosis of amyotrophic lateral sclerosis. J Neurol Sci. 1998;160(Suppl 1):S6–24. doi: 10.1016/S0022-510X(98)00193-2
2..Neuroimaging in Amyotrophic Lateral Sclerosis,Sumei Wang, Elias R. Melhem, Harish Poptani, and John H. Woo. Neurotherapeutics. 2011 Jan; 8(1): 63–71. doi: 10.1007/s13311-010-0011-3
3.Fox M & Cohen A. "Bright Tongue Sign" in ALS. Neurology. 2012;79(14):1520. doi:10.1212/wnl.0b013e31826d5ffc
Findings
Cortical atrophy of bilateral frontoparietal region with symmetrical T2 /FLAIR hyperintensities noted along bilateral corticospinal tracts from precentral gyrus to the level of midbrain with patchy diffusion restriction and corresponding low ADC values. IMPRESSION: Symmetrical T2 /FLAIR hyperintensities along bilateral corticospinal tracts with patchy diffusion restriction-Features suggestive of amyotrophic lateral sclerosis.
Discussion
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a fatal neurodegenerative disorder affecting upper motor neurons (UMN) in the motor cortex as well as lower motor neurons (LMN) in the brain stem and spinal cord. Patients with ALS suffer from a progressive paralysis of the skeletal muscles, resulting in loss of voluntary movement, ability to speak and eat, and eventual loss of respiratory function The cause of ALS is generally unknown, although approximately 2% of cases are due to mutations in the superoxide dismutase gene The diagnosis of ALS is currently based on clinical features, electromyography, and the exclusion of other diseases with similar symptoms Consistent features in MRI that are indicative but not specific for the diagnosis of ALS include hyperintensity in the CST, hypointensity in the motor cortex, and brain atrophy T1: hyperintensity of the tongue may be seen in patients with bulbar involvement, known as the "bright tongue sign" T2: hyperintensity in the corticospinal tracts GRE/SWI: hypointensity in the precentral gyrus bilaterally, known as the "motor band sign" MR spectroscopy 2 -decreased NAA -decreased glutamate -increased choline -increased myo-inositol
REFERENCES:
1.Rowland LP. Diagnosis of amyotrophic lateral sclerosis. J Neurol Sci. 1998;160(Suppl 1):S6–24. doi: 10.1016/S0022-510X(98)00193-2
2..Neuroimaging in Amyotrophic Lateral Sclerosis,Sumei Wang, Elias R. Melhem, Harish Poptani, and John H. Woo. Neurotherapeutics. 2011 Jan; 8(1): 63–71. doi: 10.1007/s13311-010-0011-3
3.Fox M & Cohen A. "Bright Tongue Sign" in ALS. Neurology. 2012;79(14):1520. doi:10.1212/wnl.0b013e31826d5ffc
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!