Answer for BIR CoW 02 July 2023
Amyotrophic lateral sclerosis
Findings
Curvilinear area of gradient blooming noted in along both motor cortices (precentral gyrus regions). Relatively prominent central sulcus and post central sulcus are noted in both sides. No significant abnormal FLAIR hyperintensities noted in the corticospinal tracts in both sides. Curvilinear area of gradient blooming noted in along both motor cortices (precentral gyrus regions) - Reflecting Motor Band sign - Suggesting Amyotrophic lateral sclerosis
Discussion
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig disease and Charcot disease, is the most common form of motor neuron disease resulting in progressive weakness and eventual death due to respiratory insufficiency. There is both upper motor neuron and lower motor neuron damage. 'Lateral sclerosis' indicates degeneration of the pyramidal tracts. Both upper and lower motor neurons are affected, with decreased motor strength and wasting of the limb muscles, bulbar muscles, and diaphragm. There is a progressive loss of motor strength, with preservation of intellectual and sensory function. In the hands, the split hand sign or split hand plus sign may be characteristically seen. El Escorial criteria for the diagnosis of amyotrophic lateral sclerosis: it requires the presence of signs of lower motor neuron (LMN) degeneration by clinical, electrophysiological or neuropathologic examination signs of upper motor neuron (UMN) degeneration by clinical examination progressive spread of signs within a region or to other regions together with the absence of electrophysiological evidence of other disease processes that might explain the signs of LMN and/or UMN degeneration neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs
Imaging features:
Iron deposition in the cortex, most notably in the precentral gyrus, is demonstrated as loss of signal on GRE and SWI , but may also be seen on T2-weighted imaging in ~50%.
T2: hyperintensity in the corticospinal tracts
MR spectroscopy decreased NAA, decreased glutamate, increased choline, increased myo-inositol
Findings
Curvilinear area of gradient blooming noted in along both motor cortices (precentral gyrus regions). Relatively prominent central sulcus and post central sulcus are noted in both sides. No significant abnormal FLAIR hyperintensities noted in the corticospinal tracts in both sides. Curvilinear area of gradient blooming noted in along both motor cortices (precentral gyrus regions) - Reflecting Motor Band sign - Suggesting Amyotrophic lateral sclerosis
Discussion
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig disease and Charcot disease, is the most common form of motor neuron disease resulting in progressive weakness and eventual death due to respiratory insufficiency. There is both upper motor neuron and lower motor neuron damage. 'Lateral sclerosis' indicates degeneration of the pyramidal tracts. Both upper and lower motor neurons are affected, with decreased motor strength and wasting of the limb muscles, bulbar muscles, and diaphragm. There is a progressive loss of motor strength, with preservation of intellectual and sensory function. In the hands, the split hand sign or split hand plus sign may be characteristically seen. El Escorial criteria for the diagnosis of amyotrophic lateral sclerosis: it requires the presence of signs of lower motor neuron (LMN) degeneration by clinical, electrophysiological or neuropathologic examination signs of upper motor neuron (UMN) degeneration by clinical examination progressive spread of signs within a region or to other regions together with the absence of electrophysiological evidence of other disease processes that might explain the signs of LMN and/or UMN degeneration neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs
Imaging features:
Iron deposition in the cortex, most notably in the precentral gyrus, is demonstrated as loss of signal on GRE and SWI , but may also be seen on T2-weighted imaging in ~50%.
T2: hyperintensity in the corticospinal tracts
MR spectroscopy decreased NAA, decreased glutamate, increased choline, increased myo-inositol
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!