scholarly journals Upper Motor Neuron Signs in the Cervical Region of Patients With Flail Arm Syndrome

2021 ◽  
Vol 12 ◽  
Author(s):  
Yingsheng Xu ◽  
Junyi Chen ◽  
Shuo Zhang ◽  
Dongsheng Fan
Keyword(s):  
Motor Neuron ◽  
Amplitude Ratio ◽  
Lower Motor Neuron ◽  
Cervical Region ◽  
Quantitative Detection ◽  
Neurophysiological Tests ◽  
Tendon Reflex ◽  
Peking University ◽  
Reflex Testing

Objective: We investigated upper motor neuron (UMN) signs in the cervical region in a Chinese clinic-based cohort of patients with flail arm syndrome (FAS) by clinical examination and neurophysiological tests such as triple stimulation technique (TST) and pectoralis tendon reflex testing.Methods: A total of 130 consecutive FAS patients from Peking University Third Hospital underwent physical examination and neurophysiological tests at baseline and 3 months, 6 months, 9 months, and 12 months later. Pyramidal signs, pectoralis tendon reflex and TST results were evaluated to estimate the function of cervical spinal UMNs.Results: At the first visit, weakness of the bilateral proximal upper limbs was found in 99 patients, while weakness of a single proximal upper limb was found in 31 patients. There were 49 patients with tendon hyperreflexia, 42 patients with tendon hyporeflexia and 39 patients with tendon areflexia. All except 4 of the patients had brisk pectoralis tendon reflex. The UMN score of the cervical region was 1.7 ± 0.4, and the lower motor neuron score of that region was 3.5 ± 0.3. The TSTtest/TSTcontrol amplitude ratio was 65.7 ± 7.5%. The latency of quantitative detection of the pectoralis tendon reflex was 7.7 ± 1.2 ms. In the follow-up study, the UMN score and the TSTtest/TSTcontrol amplitude ratio decreased, while the lower motor neuron score increased, and the latency of quantitative detection of the pectoralis tendon reflex remained steady.Conclusion: Although the signs of cervical spinal UMN dysfunction in patients with FAS were often concealed by muscle atrophy in the progression of the disease, TST and pectoralis tendon reflex could reveal it.

Downbeat nystagmus and lower motor neuron disease: 14 years follow-up

2018 ◽  
Vol 265 (3) ◽  
pp. 714-716
Author(s):  
E. Anagnostou ◽  
G. Papadimas ◽  
M. Rentzos ◽  
T. Zambelis
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Lower Motor Neuron ◽  
Downbeat Nystagmus ◽  
Lower Motor Neuron Disease

scholarly journals Hypermetabolism in ALS is associated with greater functional decline and shorter survival

2018 ◽  
Vol 89 (10) ◽  
pp. 1016-1023 ◽  
Author(s):  
Frederik J Steyn ◽  
Zara A Ioannides ◽  
Ruben P A van Eijk ◽  
Susan Heggie ◽  
Kathryn A Thorpe ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Primary Outcome ◽  
Rating Scale ◽  
Matched Control ◽  
Functional Decline ◽  
Lower Motor Neuron ◽  
Risk Of Death ◽  
Lateral Sclerosis ◽  
Age And Sex

ObjectiveTo determine the prevalence of hypermetabolism, relative to body composition, in amyotrophic lateral sclerosis (ALS) and its relationship with clinical features of disease and survival.MethodsFifty-eight patients with clinically definite or probable ALS as defined by El Escorial criteria, and 58 age and sex-matched control participants underwent assessment of energy expenditure. Our primary outcome was the prevalence of hypermetabolism in cases and controls. Longitudinal changes in clinical parameters between hypermetabolic and normometabolic patients with ALS were determined for up to 12 months following metabolic assessment. Survival was monitored over a 30-month period following metabolic assessment.ResultsHypermetabolism was more prevalent in patients with ALS than controls (41% vs 12%, adjusted OR=5.4; p<0.01). Change in body weight, body mass index and fat mass (%) was similar between normometabolic and hypermetabolic patients with ALS. Mean lower motor neuron score (SD) was greater in hypermetabolic patients when compared with normometabolic patients (4 (0.3) vs 3 (0.7); p=0.04). In the 12 months following metabolic assessment, there was a greater change in Revised ALS Functional Rating Scale score in hypermetabolic patients when compared with normometabolic patients (−0.68 points/month vs −0.39 points/month; p=0.01). Hypermetabolism was inversely associated with survival. Overall, hypermetabolism increased the risk of death during follow-up to 220% (HR 3.2, 95% CI 1.1 to 9.4, p=0.03).Conclusions and relevanceHypermetabolic patients with ALS have a greater level of lower motor neuron involvement, faster rate of functional decline and shorter survival. The metabolic index could be important for informing prognosis in ALS.

Diagnostic accuracy of electrically elicited multiplet discharges in patients with motor neuron disease

2014 ◽  
Vol 86 (11) ◽  
pp. 1234-1239 ◽  
Author(s):  
Boudewijn T H M Sleutjes ◽  
Inger Montfoort ◽  
Pieter A van Doorn ◽  
Gerhard H Visser ◽  
Joleen H Blok
Keyword(s):  
Diagnostic Accuracy ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Muscular Atrophy ◽  
High Density ◽  
Lower Motor Neuron ◽  
Cervical Region ◽  
Lower Sensitivity ◽  
Diagnostic Study ◽  
Motor Neuropathy

ObjectiveTo determine and compare the diagnostic accuracy of electrically elicited multiplet discharges (MDs) and fasciculation potentials (FPs) in motor neuron disease (MND).MethodsPatients were eligible when they had MND in their differential diagnosis and were referred for electromyogram (EMG). Stimulated high-density surface EMG of the thenar muscles was performed on the same day as standard EMG examination. High-density recordings were analysed for presence of MDs and needle EMG of any muscle investigated in the cervical region for presence of FPs.ResultsOf the 61 patients enrolled in this diagnostic study, 24 patients were clinically diagnosed with amyotrophic lateral sclerosis (ALS) and 11 patients with progressive muscular atrophy (PMA). Another diagnosis was made in 26 patients. Sixteen patients in whom MDs were detected were diagnosed with either ALS (n=11) or PMA (n=5; sensitivity=47.1%, PPV=94.1%). MDs were detected in only one patient initially diagnosed with PMA, but in whom later on, multifocal motor neuropathy could not be excluded (specificity=96.2%). Electrically elicited MDs had a higher specificity than FPs (96.2% vs 53.9%, p<0.001, n=26) and lower sensitivity (47.1% vs 85.3%, p=0.002, n=34). When considering presence of MDs in MND as neurogenic EMG abnormality, lower motor neuron involvement of ≥1 EMG region increased from 50% to 73.5% (p=0.008, n=34).ConclusionsElectrically evoked MDs are highly specific for ALS and PMA and are an early sign of lower motor neuron dysfunction.

scholarly journals The rostral to caudal gradient of clinical and electrophysiological features in sporadic amyotrophic lateral sclerosis with bulbar-onset

2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095650
Author(s):  
Song-Jie Liao ◽  
Zi Huang ◽  
Chong-Yuan Lai ◽  
Jing-Yan Chen ◽  
Pei-Yao Xiao ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Lower Motor Neuron ◽  
Sporadic Amyotrophic Lateral Sclerosis ◽  
Lumbosacral Spinal Cord ◽  
Leg Muscles ◽  
Bulbar Onset ◽  
Spinal Segments ◽  
Lateral Sclerosis

Objective Amyotrophic lateral sclerosis (ALS) with bulbar-onset (BO-ALS) tends to propagate to the adjacent anatomical regions symptomatically. However, the spreading pattern of clinical and electrophysiological features is not well documented. Methods This retrospective study enrolled consecutive patients with sporadic BO-ALS. The clinical progression and electrophysiological data by electromyography examination were retrospectively analysed based on information from the medical records. Results The study enrolled 57 patients: 43 presented with contiguous (37 of 57) or non-contiguous (6 of 57) progression clinically; and 14 patients did not present with symptomatic propagation to other spinal segments. Lower motor neuron dysfunction was more frequently involved in the bulbar and cervical segments and less in the thoracic and lumbosacral segments. As a result, a small proportion of patients had intact thoracic paraspinal or leg muscles or both by electromyography examination. Furthermore, the patients with diagnostic latency ≤6 months showed a significantly lower incidence of neurogenic changes in the lumbosacral spinal cord compared with those with diagnostic latency > 6 months. Conclusion This current study demonstrated a relative rostral–caudal descending gradient of lower motor neuron dysfunction in patients with BO-ALS. These results suggest that follow-up EMG might be necessary for a proportion of patients.

scholarly journals Unilateral lower motor neuron-type of facial palsy following snake bite presumably due to Krait (Bungarus Caeruleus)

2012 ◽  
Vol 32 (2) ◽  
pp. 184-186
Author(s):  
S Verma ◽  
K Kumar ◽  
S Arvind ◽  
A Khadwal
Keyword(s):  
Facial Nerve ◽  
Motor Neuron ◽  
Complete Recovery ◽  
Good Prognosis ◽  
Snake Bite ◽  
Lower Motor Neuron ◽  
Uncommon Presentation ◽  
Paediatric Age ◽  
History Of

We describe an unusual case of snake bite presumably due to Krait (Bungarus Caeruleus) in a 12-year-old child from Mohali, Punjab. He presented with a history of bite behind his left ear, while he was sleeping at night on floor. He had bilateral ptosis, dysphonia initially, which progressed gradually to cause respiratory paralysis. Child was managed with antisnake venom, ventilation and other supportive measures. He recovered gradually, but persisted to have lower motor neuron paralysis of left facial nerve, which was noted post extubation. This uncommon presentation could be because of exposure of the facial muscles to the venom, spreading directly from the injection site and destroying the nerve terminals of facial nerve in the muscle tissue. At three months follow-up, child showed complete recovery. Facial nerve involvement following snake bite, which usually has a good prognosis, remain an uncommon presentation in paediatric age group. J Nepal Paediatr Soc 2012;32(2):184-186 doi: http://dx.doi.org/10.3126/jnps.v32i2.5824

Late onset Sandhoff disease presenting with lower motor neuron disease and stuttering

2021 ◽  
Author(s):  
Jorge Alonso-Pérez ◽  
Ana Casasús ◽  
Álvaro Gimenez-Muñoz ◽  
Jennifer Duff ◽  
Ricard Rojas-Garcia ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Late Onset ◽  
Sandhoff Disease ◽  
Lower Motor Neuron ◽  
Lower Motor Neuron Disease

scholarly journals Inheritance and reflection: “re-study” of three anthropology fieldwork sites in China’s Yunnan Province

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Mingming Wang
Keyword(s):  
Rural Society ◽  
Research Report ◽  
Ancestor Worship ◽  
Land System ◽  
Peking University ◽  
The Difference ◽  
And Shifts ◽  
West Town ◽  
The Relationship

AbstractThis article is a research report involving three anthropological studies conducted during the period of “Kuige” and their “re-studies.” By narrating the project, I set forth my views on the connections and differences between Chinese anthropological explorations from two historical periods. These anthropological explorations refer to the study of Lu Village conducted by Fei Xiaotong, that of “West Town” (Xizhou) by Francis L. K. Hsu, and that of “Pai-IPai” (Dai) villages by Tien Ju-Kang. They were all completed in the late 1930s and early 1940s. Each writer extracted a framework to analyze the land system, ancestor worship, and the relationship between humans and gods from the writer’s own field experience. Despite the difference in research methods, all three studies noticed the cultural differences between rural society and modernity. Since 2000, Peking University and Yunnan Minzu University have launched a “Province-university Cooperation Project.” During the project, a research team formed of several young scholars revisited Lu Village, “West Town” (Xizhou), and Namu Village. These writers’ works were based on the data acquired in their fieldwork and drew upon the opinions raised by global anthropologists on “re-study” in recent decades. Considering the dual effects of social change and shifts in academic concepts around “follow-up research,” the scholars put forward several points of view with their ethnographies, which all featured the characteristics of inheritance and reflection. Based on the results of the three “re-studies,” this article emphasizes the importance of the study of public rituals for the research of rural society. This article also attempts to re-examine the methodology of “human ecology,” which profoundly impacts Chinese anthropology and sociology.

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