Baseline demographics, time to hospital visit after symptom onset, the Medical Research Council (MRC) scale of the weakest muscle, the predominantly affected site between proximal and distal muscles, upper motor neuron (UMN) signs (jaw jerk, glabella reflex, snout reflex, brisk deep tendon reflexes, Hoffman sign, extensor plantar response) at initial visit, and EMG findings were specifically noted. Patients with distal upper limb weakness or wasting without proximal involvement at presentation were excluded from this category. Maps of FA reductions are depicted in Fig. NCI CPTC Antibody Characterization Program. The comparison of MRC grades of different muscles between the two groups has obvious limitations. The lack of information on fasciculation potentials in the EMG study prevented comparison to clinical fasciculation. Presse arrow_forward. Liying Cui, Affiliation: PubMed  The fractures cause your ribs and chest wall to malfunction in the ways they move. However, the natural history and sexual predominance of FAS are different from that of PMA as well[6]. Clinically, ALS patients may present with phenotypes that differ from the ‘classical’ involvement of the upper and the lower motor neuron (UMN, LMN) according to established diagnostic criteria: on the one hand, the diagnostic criteria for primary lateral sclerosis as a slowly progressive upper motor neuron syndrome have been recently updated [3], whereas, on the other hand, it is recognized that upper motor neuron signs are not always clinically evident [4], such as in progressive muscular atrophy or progressive bulbar palsy. Hecheng Yang, However, no significant difference of CMCT was found between any two of these three groups. During the follow-up visits, we established the progression to ALS when they had evidence of bulbar or respiratory muscle involvement or lower limb weakness. Patients with FAS showed a male predominance pattern as compared to patients with UL-ALS (5:1 vs. 1.8:1, p=0.129). Because of its . BDNF ALS Study Group (Phase III). The pairs of surface electrodes for documenting MEPs of ADMs were the same pairs used in motor nerve conduction studies. And even if you had a genetic form of ALS, your age of onset would be more like your mom's, not decades before it. Katz JS, Wolfe GI, Andersson PB, Saperstein DS, Elliott JL, Nations SP, Bryan WW, Barohn RJ. Accessibility 1 for the group comparisons. Flail arm syndrome (FAS), an atypical presentation of amyotrophic lateral sclerosis (ALS), is characterized by progressive, predominantly proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or respiratory muscles. Therefore, the weakness and atrophy of hands in FAS might not have a cortical basis. Flail arm syndrome is a restricted phenotype of motor neuron disease that is characterized by progressive, predominantly proximal weakness and atrophy of the upper limbs. We considered p-values < 0.05 as statistically significant. Flail arm syndrome (FAS) is a variant of motor neuron disease which is characterized by progressive, predominantly proximal weakness and atrophy of the upper limbs (UL). Received 2014 Jul 31; Revised 2014 Aug 25; Accepted 2014 Aug 26. National Library of Medicine The clinical management of this motor neuron disease is compromised by a delay in diagnosis and a high rate of initial misdiagnoses [9]. Most patients with FAS showed proximal muscle weakness. Advanced neuroimaging approaches in amyotrophic lateral sclerosis: refining the clinical diagnosis. The center of magnetic coil was placed over C7 cervical spine to stimulate cervical nerve roots of lower segment to acquire the peripheral part of latency of MEPs responses. Clinical features of flail arm syndrome. Both in ‘classical’ ALS and in flail arm syndrome patients, the volume of the precentral gyrus was atrophied compared to controls, with more pronounced atrophy in ALS patients (Fig. Keywords: and transmitted securely. Professional info; FAQ; Note: This document contains side effect information about pregabalin. These six FAS patients in this study progressed very slowly, and the symptoms and signs were still confined to bilateral upper extremities 18 months after disease onset. FAS has male predominance, with a ratio of male to female being 4~10:1, which is only 1.1~1.5:1 in ALS patients[6, 10]. Significant differences were observed between the 2 groups pertaining to the rate of fasciculation, patterns of predominantly affected muscles, and the Medical Research Council scale of the weakest muscle. Gross brain pathology, including vascular brain alterations, was excluded by conventional MRI. Unauthorized use of these marks is strictly prohibited. doi:10.1016/s1474-4422(05)70326-4, Article  We could not evaluate the whole information because of the lack or missing of some clinical data such as checking upper motor neuron signs. The prognosis of FAS is significantly better than that of ALS, in terms of median survival rates, as well as five-year survival rates[6]. HHS Vulnerability Disclosure, Help Agosta F, Al-Chalabi A, Filippi M, et al. Of the remaining 22 patients, 5 had brachial plexitis, 4 had cervical radiculopathy, 2 had Charcot-Marie-Tooth disease (CMT1A), and 11 were classified as having disease of unknown origin. In conclusion, in case of proximal muscle weakness, FAS patients are more severely affected than UL-ALS patients. A summary of all significant clusters at the group level is provided in Supplementary Table 1. 2Department of Neurology, College of Medicine, Jeju National University, Jeju 690-767, Korea. Amyotrophic lateral sclerosis (ALS) is a relatively rare disease with a significant phenotypic variation often leading to misdiagnosis [].Bilateral flail arm syndrome also known as 'man-in-barrel syndrome' (MIBS) is one of the rarest and atypical presentation of regional ALS. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). Bilateral upper limb weakness could be a frequent presenting symptom of ALS cases [7]. The inclusion criteria: those FAS or ALS patients who agreed to participate in this study; healthy volunteers with age of 30–65 years old. Wijesekera LC, Mathers S, Talman P, Galtrey C, Parkinson MH, Ganesalingam J, Willey E, Ampong MA, Ellis CM, Shaw CE, Al-Chalabi A, Leigh PN. Baseline demographic, clinical and electrophysiological characteristics. Gorges M, Del Tredici K, Dreyhaupt J, et al. Amyotroph Lateral Scler Other Motor Neuron Disord 1(5):293–299, Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. The relationship between FAS and ALS is still unclear. Mingsheng Liu, This may contribute to difficulties in differential diagnosis and to ineffective treatment regimes. 1 Einleitung 2 Zu den wichtigen Formen werden jedoch fast immer gezählt: die klassische Form der amyotrophen Lateralsklerose, auch ALS mit spinalem Beginn oder Charcot-ALS genannt. Recently, structural and functional neuroimaging findings have greatly modified longstanding notions regarding the pathophysiology of ALS [11, 12]. Paris: Octave Doin, pp 412–491. Kassubek J, Müller HP. This study was supported by the German Research Foundation (Deutsche Forschungsgemeinschaft, DFG Grant Number LU 336/15-1) and the German Network for Motor Neuron Diseases (BMBF 01GM1103A). However, these 3 phenotypic categories do not fully capture the spectrum of clinical heterogeneity in ALS, which may contribute to diagnostic error. government site. 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 . Citation: Yang H, Liu M, Li X, Cui B, Fang J, Cui L (2015) Neurophysiological Differences between Flail Arm Syndrome and Amyotrophic Lateral Sclerosis. In this group, 40 patients presented with bilateral paresis in the arms at date of MRI, while unilateral arm symptoms at date of MRI were observed in 3 patients. PLOS is a nonprofit 501(c)(3) corporation, #C2354500, based in San Francisco, California, US. Kornelia Günther is thankfully acknowledged for her help with the Ulm MND patient registry. Prior to correction for the covariate age, a Gaussian filter of 8 mm full width at half maximum was applied for smoothing of FA, AD, and RD maps. Neurology 53(5):1071–1076, Couratier P, Truong C, Khalil M, Deviere F, Vallat JM (2000) Clinical features of flail arm syndrome. Clinical features and differential diagnosis of flail arm syndrome. AR: data collection, critical revision of manuscript for intellectual content. Before The Seoul National University Hospital ALS center is located in downtown Seoul and serves a diverse community of patients from the metropolitan area. Moreover, the RMT in ALS patients was also significantly higher than that of in FAS patients (p<0.05). Bethesda, MD 20894, Web Policies Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany, Annemarie Hübers, Viviane Hildebrandt, Angela Rosenbohm, Albert C. Ludolph & Johannes Dorst, Department of Neurology, Hannover Medical School, Hannover, Germany, Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, Dresden, Germany, German Center for Neurodegenerative Diseases (DZNE), Dresden, Germany, Department of Neurology, University Medical Center Rostock, Rostock, Germany, Department of Neurology, University of Halle, Halle, Germany, You can also search for this author in The clinical findings, with symmetric, predominantly proximal wasting and weakness of both arms (especially of the infra-, supraspinatus and deltoideus) leading to severe functional disability and contrasting with preserved independent ambulation and sparing of bulbar muscles, were consistent with the . MRC, Medical Research Council; UMN, upper motor neuron; EMG, electromyography. Part of Springer Nature. Initial symptoms were most frequently present either in distal muscles only or in both proximal and distal muscle groups combined (76%) and showed an asymmetric distribution pattern in the majority of cases (76%). Although UMN signs tend to be more frequent in UL-ALS than in FAS, many FAS patients also had UMN signs. In summary, there is limited evidence showing that upper motor neurons are obviously damaged in FAS patients. Flail arm syndrome was diagnosed in patients with paresis of both upper limbs and without bulbar and lower limbs symptoms during a time period of 12 months after their visit, as previously described [9]. All human studies have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. Sato M, Nakamura T, Nagashima K, Fujita Y, Ikeda Y. Neurol Res. It was reported that about 70% FAS patients developed UMNS in the lower extremities[12], and 50% FAS patients showed UMNS in the upper extremities[13]. Google Scholar, Singer MA, Statland JM, Wolfe GI, Barohn RJ (2007) Primary lateral sclerosis. Since these muscles of thenar and hypothenar were innervated by the same myotomes, the difference of involvement severity of these two sites seems not be derived from spinal cord. Yoon BN, Choi SH, Rha JH, Kang SY, Lee KW, Sung JJ. PubMed Central  DTI-based white matter mapping was performed both by an unbiased voxel-wise statistical comparison and by a hypothesis-guided tract-wise analysis of fractional anisotropy (FA) maps according to the neuropathological ALS-propagation pattern for 43 flail arm syndrome patients vs 43 ‘classical’ ALS patients vs 40 matched controls. This means that at least 10 (50%) patients had an UMN sign. These results are consistent with those reported in previous studies [3, 8, 9]; however, the small sample size of the current study may have impacted our ability to observe a statistically significant difference. Despite a uniformly fatal outcome, patients with ALS display a wide range of survival times from a few months to several decades [1]. The male-to-female ratio was 5:1 in the FAS group and 1.8:1 in the UL-ALS group. Atrophie musculaire progressive myélopathique.) doi:10.1212/01.wnl.0000345041.83406.a2, Article  2006. Since the mean age of patients with FAS was 59 years, these findings may have been attributable to patient age, as the elderly are known to have higher prevalence rates of lumbar or thoracic radiculopathy. Diffusion tensor imaging: concepts and applications. Cosottini M, Giannelli M, Siciliano G, et al. Initial symptoms were most frequently present either in distal muscles only or in both proximal and distal muscle groups combined (76 %) and showed an asymmetric distribution pattern in the majority of cases (76 %). the contents by NLM or the National Institutes of Health. Amyotroph Lateral Scler Other Motor Neuron Disord. #2. An official website of the United States government. The neuropathological process underlying amyotrophic lateral sclerosis (ALS) entails abnormal changes of the endogenous and predominantly intranuclear protein TDP-43 (transactive response DNA-binding protein 43) which progress at different rates, but in a similar sequence of four stages, in ALS patients’ brains [1, 2]. 2004 Aug;11(8):567-8 Our result is consistent with previous research, showing that the split-hand phenomenon is not commonly seen in FAS[25]. Flail arm syndrome patients presented with a revised ALS functional rating scale (ALS-FRS-R) [21] of 41 ± 6 on average. The same cortico-efferent tract involvement in progressive bulbar palsy and in 'classical' ALS: a tract of interest-based MRI study. In fact, only 1 patient with FAS had EMG LMN involvement in the bulbar region. Inclusion in an NLM database does not imply endorsement of, or agreement with, In the UL-ALS group, 36 patients were classified as clinically probable-laboratory supported ALS, and 3 as possible ALS. Fasciculation potentials are relevant for the diagnosis of ALS. This conclusion is supported by the fact that the patient group in the present imaging study is representative of the flail arm syndrome phenotype, including male preponderance and a slower disease course (Table ​(Table1).1). Distribution of staging categorization in flail arm syndrome patients and ‘classical’ ALS patients. Of these, 96 patients initially presented with symmetrical upper limb weakness. doi:10.1007/s00415-015-7927-9, Vucic S, Kiernan MC (2007) Abnormalities in cortical and peripheral excitability in flail arm variant amyotrophic lateral sclerosis. Therefore, we could not predict the slow clinical course during the early stages. As a library, NLM provides access to scientific literature. Kalra S, Müller HP, Ishaque A, et al. -. Forty-three flail arm syndrome patients (32 males, 11 females) were included who met the diagnostic criteria for flail arm syndrome as proposed by Wijesekera and colleagues [].Flail arm syndrome was diagnosed in patients with paresis of both upper limbs and without bulbar and lower limbs symptoms during a time period of 12 months after their visit, as . The diagnosis of FAS was made when patients presented progressive muscle weakness and wasting in both upper extremities, especially the arms, without functionally involving other parts such as legs, or bulbar muscles for at least 18 months from the disease onset. The DTI study including the tract-of-interest-based analysis showed a microstructural involvement pattern in the brains of flail arm syndrome patients, supporting the hypothesis that flail arm syndrome is a phenotypical variant of ALS. Therefore, some upper limb onset ALS patients might be misdiagnosed as FAS, since there was no definite criteria of how long the functional involvement must be confined to the flail arms to make the diagnosis of FAS. 2022 May;269(5):2619-2626. doi: 10.1007/s00415-021-10854-6. Second, this study was performed by retrospective chart review. The lack of information regarding FAS may make differential diagnosis between FAS and UL-ALS difficult in clinical settings. Careers, Unable to load your collection due to an error. your institution. Prognostic factors in ALS: A critical review. Forty-three flail arm syndrome patients (32 males, 11 females) were included who met the diagnostic criteria for flail arm syndrome as proposed by Wijesekera and colleagues [8]. Sasaki S, Iwata M. Atypical form of amyotrophic lateral sclerosis. Among 7 FAS patients who had proximal dominant weakness, 6 were classified as having MRC grade 2 at shoulder abduction, and 1 as MRC grade 3. For full functionality of this site, please enable JavaScript. The progression rate in the ALS group was higher compared to the flail arm syndrome group, however, not significant. Flail arm syndrome (FAS) is a variant of motor neuron disease which is characterized by progressive, predominantly proximal weakness and atrophy of the upper limbs (UL). government site. Atlas-based volumetry of the precentral gyrus. The routine motor nerve conduction studies of bilateral median and ulnar nerves were performed in all participants. and transmitted securely. Learn more about Institutional subscriptions, Uenal H, Rosenbohm A, Kufeldt J, Weydt P, Goder K, Ludolph A, Rothenbacher D, Nagel G (2014) Incidence and geographical variation of amyotrophic lateral sclerosis (ALS) in Southern Germany–completeness of the ALS registry Swabia. In ALS group, the ratio of female to male was 1:1.56 (16:25). J Neurol Neurosurg Psychiatry 78(8):849–852. The FAS and UL-ALS groups were compared by data analysis using the SPSS (ver. PLOS ONE promises fair, rigorous peer review, In the tract-specific analysis according to the proposed sequential cerebral pathology pattern of ALS, the flail arm syndrome patients showed significant alterations of the specific tract systems that were identical to ‘classical’ ALS if compared to controls. The band pass filter was set in the same way as in motor nerve conduction studies. From what I have read they have slower profession, so survival of patients with flail arm syndrome might be better than those with other forms of ALS as the median survival in the flail arm group . Studies on electrophysiological differences between them are limited at present, and still inconclusive. Kassubek J, Müller HP, Del Tredici K, et al. Careers. Flail chest is a traumatic disorder that happens when three or more ribs located next to each other are fractured in two or more places. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. 4). The aim of this study was to investigate typical clinical features of FAS with special regard to initial symptoms and differences to classical Charcot type amyotrophic lateral sclerosis (ALS). Google Scholar, Meyer T, Munch C, van Landeghem FK, Borisow N, Dullinger J, Linke P (2007) Progressive muscle atrophy. H-JH: data analysis, interpretation of data, critical revision of the manuscript for intellectual content. Whole brain-based spatial statistics (WBSS) of AD and RD maps of flail arm syndrome patients and ALS patients vs controls. In the initial stages of the disease, a substantial proportion of patient with ALS could be noted only upper limb weakness without bulbar or lower limb weakness, aside from those with PBP who account for approximately 20% of ALS patients [7]. Median slope of ALS-FRS-R reduction over disease duration was − 0.43/month (90th percentile, range − 0.08; − 2.03). A similar distribution could be observed for the ALS patients of whom 90% could be categorized into ALS stages: 21% were in ALS stage 1, 12% in ALS stage 2, 16% in ALS stage 3, and 44% in ALS stage 4. A regionally similar but more pronounced AD and RD increase pattern was observed in the comparison of ‘classical’ ALS patients vs controls. The tract-of interest (TOI) approach [13, 14, 16–18] defined tracts for the four ALS stages [1, 30], i.e. Conceived and designed the experiments: HY LC. The latency of motor conduction of bilateral median nerves was markedly prolonged. While FA is sensitive to microstructural changes, it does not indicate a specific type of lesion; on the other hand, AD tends to be strongly affected by axonal injury whereas RD is sensitive to white matter damage due to demyelination and less to changes in the axonal density or size [25–27]. The STROBE checklist of this study. The band pass filter of EMG machine (Key point, Denmark) was set between 2Hz and 10kHz. Natural history and clinical features of the flail arm and flail leg ALS variants. The demonstration of the involvement of the cerebral tracts according to the neuropathological ALS-propagation pattern supports previous studies which showed corticospinal tract pathology in pure lower motor neuron variants of ALS [36, 37]. and transmitted securely. Comparison between Flail Arm Syndrome and Upper Limb Onset Amyotrophic Lateral Sclerosis: Clinical Features and Electromyographic Findings. sharing sensitive information, make sure you’re on a federal The same tract involvement patterns as those seen in ´classical´ ALS have been demonstrated in all of these restricted phenotypes [20]. Epub 2021 Oct 21. No significant alterations between groups were observed in the reference tract. After stereotaxic normalization to the Montreal Neurological Institute (MNI) space, fractional anisotropy (FA), axial diffusivity (AD), and radial diffusivity (RD) maps were calculated as DTI metrics to analyze white matter microstructure [24]. Between the 2 groups, there were no statistically significant differences in LMN signs on EMG. The study was approved by the ethics committees of all participating centers and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.