Cervical Spondylotic Radiculopathy Producing Motor Manifestations Mimicking Primary Muscular Atrophy

Neurosurgery ◽  
1979 ◽  
Vol 5 (4) ◽  
pp. 427-431 ◽  
Author(s):  
Michael Dorsen ◽  
George Ehni
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Muscular Atrophy ◽  
Case Reports ◽  
Cervical Radiculopathy ◽  
Additional Case ◽  
Curative Surgery ◽  
Diagnostic Difficulty ◽  
Cervical Spondylotic Radiculopathy ◽  
Primary Motor Neuron

Abstract Cervical radiculopathy unaccompanied by pain or sensory disorder but manifested only by paresis, atrophy, fasciculation, and reflex loss is unusual. Three such cases are presented. Considerable diagnostic difficulty may arise in distinguishing patients presenting with these features from those who have primary motor neuron disease. Two additional case reports demonstrate that this distinction is not always possible. Diagnosis, management, and prognosis are discussed. Differentiation between motor neuron disease and spondylosis will avert needless surgery in the former group and will result in beneficial, sometimes curative surgery in the latter group.

scholarly journals Muscle Expression of Mutant Androgen Receptor Accounts for Systemic and Motor Neuron Disease Phenotypes in Spinal and Bulbar Muscular Atrophy

Neuron ◽  
2014 ◽  
Vol 82 (2) ◽  
pp. 295-307 ◽  
Author(s):  
Constanza J. Cortes ◽  
Shuo-Chien Ling ◽  
Ling T. Guo ◽  
Gene Hung ◽  
Taiji Tsunemi ◽  
...  
Keyword(s):  
Androgen Receptor ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Muscular Atrophy ◽  
Disease Phenotypes

scholarly journals Dysgraphic features in motor neuron disease: a review

2021 ◽  
Author(s):  
Edoardo Nicolò Aiello ◽  
Sarah Feroldi ◽  
Alice Naomi Preti ◽  
Stefano Zago ◽  
Ildebrando Marco Appollonio
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Language Impairment ◽  
Oral Language ◽  
Case Reports ◽  
Angular Gyrus ◽  
Meta Analyses ◽  
Graphemic Buffer ◽  
Left Angular Gyrus ◽  
Writing Errors

AbstractBackgroundMotor neuron disease (MND) patients can show oral language deficits mimicking those of frontotemporal degenerations (FTD). Although dysgraphic features have been also reported within the MND-FTD continuum, their characteristics and clinical relevance are still largely unexplored.AimsTo profile writing disorders in MND patients can help further define their cognitive semiology and thus conveys relevant clinical entailments. Therefore, this study aimed at reviewing evidence of writing impairment in MND patients. This review was implemented and reported by consulting Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Qualitative/quantitative measures of writing abilities in MND patients was the primary outcome. Both group studies and case reports/series were taken into consideration. Twenty-four contributions were included out of an initial N=83. Potential biases in generalizing results were qualitatively controlled for by extracting background, disease-related, neuropsychological and neuroanatomofunctional secondary outcomes.Main ContributionFifteen studies assessed writing abilities in Japaneses patients, whereas the remaining eight in western patients. Central dysgraphic features were reported in both neuropsychologically-impaired and –unimpaired MND patients. Phonetic/phonological paragraphias and morpho-syntactic errors were frequently reported. Although FTD was frequently co-occurent, neither cognitive nor language impairment fully accounted for writing impairment in some patients. By contrast, evidence of peripheral dysgraphia was scarce. Patients displaying writing deficits often presented with bulbar signs and perisylvian cortices involvement (including Exner’s area and the left angular gyrus). Writing deficits proved to be associated with abnormalities in executive functioning and its neural substrates. Writing-to-dictation tasks as well as writing samples assessment proved to be useful to detect writing errors.ConclusionsDysgraphic features in MND patients might be due to dysfunctions of the graphemic buffer – and possibly the phonological route. The lexico-semantic route appeared to be less involved. However, a mixed peripheral/central involvement cannot be ruled out. In this population, executive/attentive deficits are likely to contribute to writing errors as well. Writing deficits might thus be specific of MND patients’ cognitive/language impairment profile. The evaluation of writing abilities via writing-to-dictation/narrative writing tasks may be useful when assessing cognition/language in both neuropsychological-impaired and -unimpaired MND patients - especially when severe dysarthria/anarthria is present and prevents clinicians from assessing oral language.

scholarly journals Activity-Driven Synaptic and Axonal Degeneration in Canine Motor Neuron Disease

2004 ◽  
Vol 92 (2) ◽  
pp. 1175-1181 ◽  
Author(s):  
Dario I. Carrasco ◽  
Mark M. Rich ◽  
Qingbo Wang ◽  
Timothy C. Cope ◽  
Martin J. Pinter
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Unit ◽  
Neuromuscular Junctions ◽  
Muscular Atrophy ◽  
Motor Axon ◽  
Medial Gastrocnemius ◽  
Neuron Activity ◽  
Functional Changes ◽  
Unit Function

The role of neuronal activity in the pathogenesis of neurodegenerative disease is largely unknown. In this study, we examined the effects of increasing motor neuron activity on the pathogenesis of a canine version of inherited motor neuron disease (hereditary canine spinal muscular atrophy). Activity of motor neurons innervating the ankle extensor muscle medial gastrocnemius (MG) was increased by denervating close synergist muscles. In affected animals, 4 wk of synergist denervation accelerated loss of motor-unit function relative to control muscles and decreased motor axon conduction velocities. Slowing of axon conduction was greatest in the most distal portions of motor axons. Morphological analysis of neuromuscular junctions (NMJs) showed that these functional changes were associated with increased loss of intact innervation and with the appearance of significant motor axon and motor terminal sprouting. These effects were not observed in the MG muscles of age-matched, normal animals with synergist denervation for 5 wk. The results indicate that motor neuron action potential activity is a major contributing factor to the loss of motor-unit function and degeneration in inherited canine motor neuron disease.

scholarly journals Motor neuron disease presenting with fetal akinesia

2018 ◽  
Vol 45 (S1) ◽  
pp. S4-S4
Author(s):  
P. Shannon ◽  
D. Chitayat ◽  
K. Chong ◽  
C. Dunham ◽  
C. Fallet-Bianco
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Globus Pallidus ◽  
Motor Neurons ◽  
Muscular Atrophy ◽  
Neuronal Loss ◽  
Ischemic Injury ◽  
Neuroaxonal Dystrophy ◽  
Lysosomal Storage ◽  
Whole Exome

By contrast to infantile spinal muscular atrophy, which usually links to deletions in the SMN genes, fetal onset motor neuron disease is poorly reported. We collected a series of twelve cases of fetal arthrogryposis (16-31 weeks gestational age) with fetal motor neuron disease and excluded infectious diseases, lysosomal storage disease and neuroaxonal dystrophy. Of these twelve, 3 were thought to be ischemic in nature with microvascular alterations and systemic or central nervous system ischemic injury. The remaining 9 all displayed marked reduction in anterior horn motor neurons. Of these 9, four demonstrated mineralised neurons, four demonstrated either neuronal loss or cavitation in the globus pallidus, and in two, degenerating neurons were detectable in the brainstem or globus pallidus. Specific sequencing of SMN1 was performed in 6 of 9 and was reported as normal. Whole exome sequencing was performed in 4 without definitive diagnosis. We conclude that fetal motor neuron disease can be distinguished from ischemic injury, is morphologically heterogeneous, may affect the globus pallidus and is rarely linked to SMN1 mutations.

Motor neuron disease

2018 ◽  
Author(s):  
Martin R. Turner
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Neurons ◽  
Corticospinal Tract ◽  
Muscular Atrophy ◽  
Muscle Denervation ◽  
Genetic Overlap ◽  
Upper Motor Neurons ◽  
Primary Lateral ◽  
Lateral Sclerosis

Motor neuron disease (MND) is characterized by progressive muscular weakness due to simultaneous degeneration of lower and upper motor neurons (L/UMNs). Involvement of LMNs, arising from the anterior horns of the spinal cord and brainstem, leads to secondary wasting as a result of muscle denervation. Involvement of the UMNs of the motor cortex and corticospinal tract results in spasticity. In ~85% of cases, there is clear clinical involvement of both, and the condition is termed ‘amyotrophic lateral sclerosis’ (ALS; a term often used synonymously with MND). In ~13% of cases, there may be only LMN signs apparent, in which case the condition is termed ‘progressive muscular atrophy’, although such cases have a natural history that is to largely identical to that of ALS. In a very small group of patients (~2%), there are only UMN signs for at least the first 4 years, in which case the condition is termed ‘primary lateral sclerosis’; such cases have a uniformly slower progression. There is clinical, neuropathological, and genetic overlap between MND and some forms of frontotemporal dementia.

scholarly journals Inherited Motor Neuron Disease in Domestic Cats: A Model of Spinal Muscular Atrophy

2005 ◽  
Vol 57 (3) ◽  
pp. 324-330 ◽  
Author(s):  
Qianchuan He ◽  
Charles Lowrie ◽  
G Diane Shelton ◽  
Rudy J Castellani ◽  
Marilyn Menotti-Raymond ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Muscular Atrophy ◽  
Domestic Cats

Motor Neuron Disease (DRAFT)

2018 ◽  
Author(s):  
Tamara Kaplan ◽  
Tracey Milligan
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Muscular Atrophy ◽  
Lower Motor Neuron ◽  
Lateral Sclerosis

The video in this chapter explores motor neuron disease, including amytrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). It discusses the signs of upper motor neuron (UMN) and lower motor neuron (LMN) pathology, as well as Kennedy disease.

scholarly journals 103 Atypical Progressive Bulbar Palsy presenting with Dropped Head

CNS Spectrums ◽  
2020 ◽  
Vol 25 (2) ◽  
pp. 267-267
Author(s):  
Alexander Carvajal-González ◽  
Antonio H Iglesias
Keyword(s):  
Motor Neuron ◽  
Neurological Examination ◽  
Muscular Atrophy ◽  
Case Reports ◽  
Nemaline Myopathy ◽  
Carnitine Deficiency ◽  
Neck Muscle ◽  
Thyroid Function Tests ◽  
Dropped Head Syndrome ◽  
Bulbar Palsy

Abstract:Introduction:Typical amyotrophic lateral sclerosis (ALS) presents on neurological examination with specific signs of upper and lower motor neuron degeneration (Brooks et al, 1995), which can account for 85% of patients with ALS (Turner and Talbot, 2013). There are different types of clinical presentations, including progressive bulbar palsy (PBP), Limb-onset ALS, progressive muscular atrophy (PMA) and upper motor neuron (UMN) predominant ALS. PBP has mainly brainstem signs. There are a few case reports of dropped head syndrome in ALS, mainly in patients with the limb involvement variant.Methods:Case reportResults:A 56 year old right-handed male, presented to the clinic with four months of dysphagia to liquids and solids, neck pain and progressive neck weakness causing constant drop head. No dysarthria or other neurological symptoms, no dyspnea. Neurological examination: Cranial Nerve (CN) CN XII: Nasal voice, bilateral atrophy of the tongue with tremor and fasciculations. Motor: Diffuse atrophy and decreased tone of the sternocleidomastoid and trapezii bilaterally, strength: 2/5 in neck flexors and extensors. Sensory: Hypoesthesia of the tongue. The rest of his neurological examination was normal. Labs: Routine blood work, thyroid function tests, collagen vascular work-up, and protein electrophoresis were normal. Creatine Phosphokinase (CPK) and Acetylcholine Receptor Antibodies (AChR Ab) were negative. Brain and Spinal Cord MRI: Showed mild brainstem, cerebellar and cervical spinal atrophy.Conclusions:Patients with ALS initially present with symptoms localized to the limbs or bulbar muscles. A very small percentage 1-2% of ALS patients had neck muscle weakness with head drop (Jokelainen et al, 1977; Gourie-Devi et al, 2003). However, in all the previously reported cases, the patients had limb involvement at the time of presentation which was absent in this case, and the head drop occurred after the onset of symptoms (Lange et al, 1986; Katz et al; 1996). Dropped head syndrome can be seen in inflammatory myopathies, myasthenia gravis, facioscapulohumeral muscular dystrophy, spinal muscular atrophy, nemaline myopathy and carnitine deficiency (Umapathy et al, 2003) but ALS should also be considered in patients with atypical presentations.

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