Neuralgic Amyotrophy Patients Research Articles

Needle electromyography abnormalities in the upper trapezius muscle in neuromuscular disorders.

: Needle electromyography (EMG) abnormalities in the trapezius muscle (TM) can be seen in neuromuscular disorders. The aim was to determine the characteristics of needle EMG abnormalities observed in the TM in neuromuscular disorders. The data of patients who applied to the Clinical Neurophysiology Laboratory of University of Health Sciences Adana City Training and Research Hospital between December 2018 and October 2021 were reviewed. Polio survivors, amyotrophic lateral sclerosis (ALS) patients, patients with sensorimotor polyneuropathy, patients with spinal cord lesions involving C2/C3/C4 segments, patients with spinal accessory nerve (SAN) lesions, neuralgic amyotrophy (NA) patients, and patients with myopathy were included. Needle EMG findings of the upper TM of the patients were analyzed. Positive sharp waves, fibrillation potentials, fasciculation potentials, myotonic discharges, and motor unit action potential (MUAP) changes were considered needle EMG abnormalities. Eighty-one polio survivors, 23 ALS patients, 39 patients with sensorimotor polyneuropathy, 10 patients with cervical spinal lesions, eight NA patients, seven patients with SAN lesions, and three patients with myopathy were included in the study. Fifteen (65.2%) ALS patients, 18 (22.2%) polio survivors, three (30%) patients with cervical spinal lesions, two (5.1%) patients with sensorimotor neuropathy, one (12.5%) NA patient, seven (100%) patients with SAN lesions, and two (66.7%) patients with myopathies had at least one needle EMG abnormality in the TM. Fasciculation potentials in the TM were seen in 10 (43.5%) ALS patients. In four patients with SAN lesions and one polio survivor, MUAP could not be obtained from the TM. There may be more frequent needle EMG abnormalities, particularly in ALS patients and patients with SAN lesions. Since the number of patients with myopathy included in this study was low, it is difficult to comment on the needle EMG features of the TM for these patients. In addition, this study indicated that fasciculation potentials in the TM are typical in ALS patients and that MUAP may not be obtained from the TM in patients with SAN lesions.

HEV-Associated Neuralgic Amyotrophy: A Multicentric Case Series.

Background: Neuralgic amyotrophy (NA) has been described as a possible extrahepatic manifestation of hepatitis E virus (HEV) infection. Usually, HEV-associated NA occurs bilaterally. The clinical characteristics determining the course of HEV-associated NA have still not been defined. Methods: In this retrospective multicentric case series, 16 patients with HEV-associated NA were studied and compared to 176 HEV patients without NA in terms of their age, sex, and ALT levels. Results: Neither gender distribution (75% vs. 67% male) nor age (47 vs. 48 years median) differed significantly between the NA patients and controls. Eight NA patients (50%) presented with bilateral involvement—seven of these had right-side dominance and one had left-side dominance. Thirteen cases (81%) were hospitalized. Eight of these patients stayed in hospital for five to seven days, and five patients stayed for up to two weeks. The time from the onset of NA to the HEV diagnosis, as well as the diagnostic and therapeutic proceedings, showed a large variability. In total, 13 (81%) patients received treatment: 1/13 (8%) received intravenous immunoglobulins, 8/13 (62%) received glucocorticoids, 3/13 (23%) received ribavirin, and 6/13 (46%) received pregabalin/gabapentin. Patients with ages above the median (47 years) were more likely to be treated (p = 0.001). Conclusion: HEV-associated NA causes a relevant morbidity. In our case series neither the type of treatment nor the time of initiation of therapy had a significant effect on the duration of hospitalization or the course of the disease. The clinical presentation, the common diagnostic and therapeutic procedures, and the patients’ characteristics showed large variability, demonstrating the necessity of standardized protocols for this rare but relevant disease.

Altered sensorimotor representations after recovery from peripheral nerve damage in neuralgic amyotrophy

Neuralgic amyotrophy is a common peripheral nerve disorder caused by acute autoimmune inflammation of the brachial plexus. Subsequent weakness of the stabilizing shoulder muscles leads to compensatory strategies and abnormal motor control of the shoulder. Despite recovery of peripheral nerves and muscle strength over time, motor dysfunction often persists. Suboptimal motor recovery has been linked to maladaptive changes in the central motor system in several nervous system disorders. We therefore hypothesized that neuralgic amyotrophy patients with persistent motor dysfunction may have altered cerebral sensorimotor representations of the affected upper limb. To test this hypothesis, 21 neuralgic amyotrophy patients (mean age 45 ± 12 years, 5 female) with persistent lateralized symptoms in the right upper limb and 20 age- and sex-matched healthy controls, all right-handed, performed a hand laterality judgement task in a cross-sectional comparison. Previous evidence has shown that to solve this task, subjects rely on sensorimotor representations of their own upper limb, using a first-person imagery perspective without actual motor execution. This enabled us to investigate altered central sensorimotor representations while controlling for altered motor output and altered somatosensory afference. We found that neuralgic amyotrophy patients were specifically less accurate for laterality judgments of their affected right limb, as compared to healthy controls. There were no significant group differences in reaction times. Both groups used a first-person imagery perspective, as evidenced by changes in reaction times as a function of participants’ own arm posture. We conclude that cerebral sensorimotor representations of the affected upper limb are altered in neuralgic amyotrophy patients. This suggests that maladaptive central neuroplasticity may occur in response to peripheral nerve damage, thereby contributing to motor dysfunction. Therapies focused on altering cerebral sensorimotor representations may help to treat peripheral nerve disorders such as neuralgic amyotrophy.

Pathological Cerebrospinal Fluid Findings in Patients With Neuralgic Amyotrophy and Acute Hepatitis E Virus Infection.

There is growing evidence that hepatitis E virus (HEV) infection can present with extrahepatic manifestations including neurological disorders. Among these, neuralgic amyotrophy (NA) has been reported to occur in some industrialized countries. We investigated 35 patients with NA and a control group for markers of HEV infection. Acute HEV infection was found in NA patients only and was associated with an inflammatory response in the central nervous system. Shedding of HEV RNA into the cerebrospinal fluid and intrathecal production of anti-HEV immunoglobulin M occurred in 1 patient, suggesting that HEV is neurotropic.

55. Lesion distributions among neuralgic amyotrophy patients

55. Lesion distributions among neuralgic amyotrophy patients

Blood lymphocytes are sensitized to brachial plexus nerves in patients with neuralgic amyotrophy

The percentage of lymphocytic subsets in the blood of cases with neuralgic amyotrophy (NA), and the proliferative response of blood lymphocytes cultured with different nerve extracts, obtained from normal subjects at postmortem, were examined in 6 patients with NA and in 18 age-matched controls with shoulder pain not related to NA. Most (5/6) NA patients had decreased CD3 values and increased CD4/CD8 ratios due to a decreased of the CD8 subset. Lymphocytes of NA patients increased their blastogenic activity in cultures with nerve extracts from different brachial plexus nerves and its branches, but not in cultures with extracts of sacral plexus nerves. Cultures did not respond to nerve extracts in any of the control cases, although mitogenic activity was similarly elicited in cultured lymphocytes stimulated with phytohemagglutinin in both control cases and NA patients. These results suggest that NA is probably an immune mediated disease.