Moreover, recent studies have shown that MUS has higher sensitivity in detecting fasciculation compared to EMG and qualitative physical assessment. The advantages of non-invasiveness and easy operation provide muscle ultrasonography (MUS) with unique advantages in the observation of fasciculations. Recently, the clinical application of neuromuscular ultrasound technology has reduced the time from onset to diagnosis for ALS patients. However, EMG has certain limitations due to the small recording area, its time-consuming nature, and invasiveness. Electromyography (EMG) is currently the primary method for detecting fasciculation. Since the Awaji criteria in 2008 emphasized the significance of fasciculation potential, fasciculation has been widely considered to be a critical biomarker for the early diagnosis of ALS. The fasciculation grade and distribution pattern detected with MUS could help distinguish ALS from non-ALS patients.Īmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that involves motor neurons in the cerebral cortex, brainstem, and spinal cord. The distribution pattern of fasciculation was different between ALS and non-ALS patients. The fasciculation grade was higher in ALS than non-ALS patients. Fasciculations in non-ALS patients were primarily low-grade and mostly distributed in distal limbs. Fasciculations were observed in four regions for ALS patients and primarily distributed in proximal limbs. The fasciculation grades (median ) were 2 (0–3) in ALS and 0 (0–0) in non-ALS patients ( P < 0.001). The overall detection rates were 72.8% in ALS and 18% in non-ALS patients. The intensity of fasciculation was divided into five grades based on firing frequency and number in the involved muscle groups. MUS was performed on 19 muscle groups in cervical, lumbosacral, bulbar, and thoracic regions for each patient. MethodsĮighty-eight ALS patients and fifty-four non-ALS (eight multifocal motor neuropathy, 32 chronic inflammatory demyelinating polyneuropathy/Charcot-Marie-Tooth, and 14 cervical spondylopathy or lumbar spondylopathy) patients were recruited. Our study aimed to analyze the difference in fasciculation detected with muscle ultrasonography (MUS) between ALS patients and non-ALS patients with symptoms resembling ALS. It is important to keep ALS in the differential diagnosis of patients with progressive weakness, even with non-definitive EMG studies, especially in patients with a history of cancer.Fasciculation is an important sign for the diagnosis of amyotrophic lateral sclerosis (ALS). Some studies have suggested that ALS is a paraneoplastic syndrome and its incidence is increased in certain malignancies.Ĭonclusion: ALS is a rare disease, of which there is still much to be discovered. The atypical presentation in this case may be related to her underlying CML. A workup of ruling out all other possible causes eventually led to the diagnosis of ALS. Diffuse leptomeningeal disease from recurrence of CML and Imatinib toxicity, although typically sensorimotor, were both not excluded by the EMG. However, sensory findings are not typically found in ALS. After recovering she refused to undergo the procedure again.ĭiscussion: The progression of her motor symptoms are typical of ALS although her EMG studies were not definitive. Sural nerve biopsy was scheduled, however the patient suffered cardiac arrest while undergoing anesthesia and the procedure was aborted.

She was treated with plasmapheresis and IVIG with no improvement in symptoms. EMG showed diffuse motor neuron pathology but did not have giant MUAPs with reduced recruitment seen in ALS.

Differential diagnosis included neural invasion of CML, tyrosine kinase inhibitor toxicity, and ALS. Results: MRI revealed nonspecific foci of T2/FLAIR signal hyperintensity in the bilateral subcortical white matter. Patient: 35 year old female with history of chronic myeloid leukemia (CML) Assessment/ An anterior truncal sensory level involvement at Right T2 and Left C3 segment was found. She initially developed muscle spasms and stiffness, which progressed to weakness of left lower, then right lower, followed by left upper extremity. Disclosures: Clarisse San Juan, MD: No financial relationships or conflicts of interestĬase Description: 35 year-old female with a 7-year history of Chronic Myeloid Leukemia in remission on Imatinib, who presented with frequent falls.

Als emg findings