Split Hand Syndrome in a Family with <i>GARS</i>-Associated Axonal Neuropathy

Kang, You-Ri; Kang, Kyung Wook; Nam, Tai‐Seung; Im, Jae Hwan; Kim, Sang Hoon; Lee, Seung Jin

doi:10.3988/jcn.2019.15.4.566

Cited by 2 publications

(2 citation statements)

References 7 publications

(11 reference statements)

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“…Although split-hand syndrome is primarily associated with ALS, split-hand has been observed in other conditions, such as spinal muscular atrophy (SMA), spinocerebellar ataxia type-3, Kennedy's disease (SBMA) or postpolio syndrome. [11][12][13][14] A recent study suggested that the split-hand syndrome may be a feature of SBMA, although this finding was discordant when compared with a previous study from this group. 4 14 'Reverse split-hand syndrome', which refers to the predominant wasting of ADM and the relative sparing of APB, may suggest the diagnosis of Hirayama disease (HD) in the appropriate clinical context (box 1).…”

Section: Other Split Phenotypes In the Upper Limbsmentioning

confidence: 61%

Split-hand and split-limb phenomena in amyotrophic lateral sclerosis: pathophysiology, electrophysiology and clinical manifestations

Corcia

Bede

Couratier

et al. 2021

J Neurol Neurosurg Psychiatry

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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting the upper and lower motor neurons. A key clinical feature of ALS is the absence of accurate, early-stage diagnostic indicators. ‘Split-hand syndrome’ was first described in ALS at the end of the last century and a considerable body of literature suggests that the split-hand phenomenon may be an important clinical feature of ALS. Considering the published investigations, it is conceivable that the ‘split-hand syndrome’ results from the associated upper and lower motor neuron degeneration, whose interaction remains to be fully clarified. Additionally, other split syndromes have been described in ALS involving upper or lower limbs, with a nuanced description of clinical and neurophysiological manifestations that may further aid ALS diagnosis. In this review, we endeavour to systematically present the spectrum of the ‘split syndromes’ in ALS from a clinical and neurophysiology perspective and discuss their diagnostic and pathogenic utility.

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Section: Other Split Phenotypes In the Upper Limbsmentioning

confidence: 61%

Split-hand and split-limb phenomena in amyotrophic lateral sclerosis: pathophysiology, electrophysiology and clinical manifestations

Corcia

Bede

Couratier

et al. 2021

J Neurol Neurosurg Psychiatry

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“…Split hand syndrome has been widely described in amyotrophic lateral sclerosis, affecting around 70% of patients at the time of diagnosis [3]. However, it has also been observed in other motor neuron disorders and neuropathies, comprising spinal muscular atrophy, Kennedy's disease and post-polio syndrome, Charcot-Marie-Tooth disease, and spinocerebellar ataxia type-3 [4,5]. Three main theories have been put forward to explain the underlying mechanisms that give rise to this pattern of muscle wasting.…”

Section: Split Handmentioning

confidence: 99%

When the diagnosis is in the patient’s hand and in the neurologist’s eye

Bertini,

Lenti,

Libelli

et al. 2024

Neurol Sci

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The objective of this study was to encompass current knowledge about pathophysiological mechanisms of those specific hand postures or deformities caused by central nervous system disorders. In the era of high-resolution neuroimaging and molecular biology, clinicians are progressively losing confidence with neurological examination. Careful hand observation is of key importance in order to differentiate neurological from non-neurological conditions, central from peripheral aetiologies, and organic from functional disorders. Localizing the potential anatomical site is essential to properly conduct subsequent exams. We provided a practical guide for clinicians to recognize hand patterns caused by central nervous system disorders, avoiding mimicking conditions, thus optimizing and prompting the diagnostic pathway.

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Split Hand Syndrome in a Family with GARS-Associated Axonal Neuropathy

Cited by 2 publications

References 7 publications

Split-hand and split-limb phenomena in amyotrophic lateral sclerosis: pathophysiology, electrophysiology and clinical manifestations

Split-hand and split-limb phenomena in amyotrophic lateral sclerosis: pathophysiology, electrophysiology and clinical manifestations

When the diagnosis is in the patient’s hand and in the neurologist’s eye

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