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Abstract Details

Progressive neurochemical abnormalities in cognitive and motor subgroups of ALS: a multicenter study
Neuromuscular and Clinical Neurophysiology (EMG)
Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)
031

To evaluate progressive cerebral degeneration in vivo in amyotrophic lateral sclerosis (ALS) and to compare abnormalities between clinical subgroups using magnetic resonance spectroscopy (MRS).

ALS is associated with degeneration of motor and extra-motor regions of the brain, and with variable patterns of onset and progression. MRS permits the assessment of cerebral degeneration in vivo, quantified by the neurometabolite N-acetylaspartate (NAA), a marker of neuronal integrity.
Seventy-six ALS patients and 59 healthy controls were recruited from the Canadian ALS Neuroimaging Consortium. Subjects underwent clinical evaluations and 3T MRI scans with follow-up assessments at 4 and 8 months. NAA ratios were quantified in the motor cortex and mesial prefrontal cortex (mPFC). Disease progression rate, upper motor neuron (UMN) signs, and cognitive status were used to stratify patients into subgroups. Linear mixed models were used for baseline and longitudinal comparisons of NAA metabolite ratios.
Compared to healthy controls, ALS patients had reduced NAA in the motor regions (P < 0.001). Faster progressing ALS patients had significantly reduced motor cortex NAA compared to slower progressing patients (P < 0.05). Patients with greater UMN burden had reduced motor cortex NAA compared to patients with lower burden (P < 0.05). NAA was reduced in the mPFC only in cognitively impaired patients (P < 0.05). A significant decline in NAA in the motor cortex was observed only in the fast progressing (P < 0.01) and high UMN burden (P < 0.01) cohorts.
Progressive degeneration of the motor cortex in ALS as measured by MRS is associated with more aggressive clinical presentations, such as faster progression rate or greater degree of UMN signs. These findings provide biological evidence of variable spatial and temporal cerebral degeneration linked to the disease heterogeneity in ALS.
Authors/Disclosures
Daniel Ta, MD (University of Alberta)
PRESENTER 		Daniel Ta has nothing to disclose.
Abdullah Ishaque Abdullah Ishaque has nothing to disclose.
Ojas Srivastava (University of Alberta Hospital) Mr. Srivastava has nothing to disclose.
No disclosure on file
No disclosure on file
No disclosure on file
Collin C. Luk, MD (University of Calgary) Dr. Luk has nothing to disclose.
Hannah R. Briemberg, MD, FRCPC (Diamond Health Care Centre) Dr. Briemberg has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. The institution of an immediate family member of Dr. Briemberg has received research support from CIHR. Dr. Briemberg has received publishing royalties from a publication relating to health care. An immediate family member of Dr. Briemberg has received publishing royalties from a publication relating to health care.
No disclosure on file
Angela L. Genge, MD (Mcgill University) Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for AL-S Pharma. Dr. Genge has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Amylyx. Dr. Genge has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Quralis. Dr. Genge has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MTPA. Dr. Genge has received personal compensation in the range of $0-$499 for serving as a Consultant for WAVE. Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for eikonizo. Dr. Genge has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for rapa.
No disclosure on file
Lawrence Korngut, MD Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi Tanabe. Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche.
Lorne H. Zinman, MD Dr. Zinman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen, MTP, AB Science, Cytokinetics, Amylyx. Dr. Zinman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amylyx.
Sanjay Kalra, MD (University of Alberta) The institution of Dr. Kalra has received research support from Brain Canada Foundation. The institution of Dr. Kalra has received research support from Biogen.