The Stripe of Primary Lateral Sclerosis

Claassen, Daniel O.; Josephs, Keith A.; Peller, Patrick J.

doi:10.1001/archneurol.2009.298

Cited by 33 publications

(6 citation statements)

References 16 publications

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“…We were also able to demonstrate dominant right temporal lobe hypometabolism on FDG-PET scan, which also revealed evidence of motor cortex hypometabolism. Motor cortex hypometabolism has been demonstrated in cases of PLS where it is referred to as the stripe of PLS[29]. In addition to motor cortex dysfunction, we were also able to demonstrate corticospinal tract abnormalities with diffusion tensor imaging showing reduced fiber density on the right compared to the left, in the descending corticospinal tract.…”

Section: (1) Discussionsupporting

confidence: 53%

Right temporal variant frontotemporal dementia with motor neuron disease

Coon

Whitwell

Parisi

et al. 2012

Journal of Clinical Neuroscience

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Patterns of atrophy in frontotemporal dementia (FTD) correlate with the clinical subtypes of behavioral variant FTD (bvFTD), semantic dementia, progressive non-fluent aphasia (PNFA) and FTD with motor neuron disease (FTD-MND). Right temporal variant FTD is associated with behavioral dyscontrol and semantic impairment, with tau abnormalities more common in right temporal bvFTD and TDP-43 accumulation in right temporal semantic dementia. However, no clinical and anatomical correlation has been described for patients with predominant right temporal atrophy and FTD-MND. Therefore, we performed a database screen for all patients diagnosed with FTD-MND at Mayo Clinic and reviewed their MRI scans to identify those with striking, dominant, right temporal lobe atrophy. For cases with volumetric MRI we performed voxel based morphometry and for those with brain tissue we performed pathological examination. Of three such patients identified, each patient had different presenting behavioral and/or aphasic characteristics. MRI, including DTI sequence in one patient, and FDG PET scan, revealed striking and dominant right temporal lobe atrophy, right corticospinal tract degeneration, and right temporal hypometabolism. Archived brain tissue was available in 2 patients; both demonstrating TDP-43 type 3 pathology (Mackenzie scheme) with predominant neuronal cytoplasmic inclusions. In one case, neurofibrillary tangles (Braak V) and neuritic plaques were also present in keeping with a diagnosis of Alzheimer's disease. There appears to be an association between FTD-MND and severe right temporal lobe atrophy. Until further characterization of such cases are determined, they may be best classified as right temporal variant FTD-MND.

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Section: (1) Discussionsupporting

confidence: 53%

Right temporal variant frontotemporal dementia with motor neuron disease

Coon

Whitwell

Parisi

et al. 2012

Journal of Clinical Neuroscience

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“…With FDG-PET, a stripe of hypometabolism may be seen in the precentral gyrus. 20 N-acetyl aspartate, a neuronal marker measured in magnetic resonance spectroscopy studies, was reduced in the precentral cortex. 10,21,22 Flumazenil-PET, which binds to receptors for GABA on cortical neurons, was decreased in PLS patients, particularly in motor regions, whereas ALS patients also had decreased binding in frontal regions.…”

Section: Diagnosismentioning

confidence: 99%

Primary Lateral Sclerosis

et al. 2015

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Synopsis Primary lateral sclerosis (PLS) is characterized by insidious onset of progressive upper motor neuron dysfunction in the absence of clinical signs of lower motor neuron involvement. Patients experience stiffness, decreased balance and coordination, and mild weakness, and if the bulbar region is affected, difficulty speaking and swallowing, and emotional lability. The diagnosis is made based on clinical history, typical exam findings, and diagnostic testing negative for other causes of upper motor neuron dysfunction. EMG is normal, or only shows mild neurogenic findings in a few muscles, not meeting El Escorial criteria. Although no test is specific for PLS, some neurodiagnostic tests are supportive: including absent or delayed central motor conduction times; and changes in the precentral gyrus or corticospinal tracts on MRI, DTI or MR Spectroscopy. Treatment is largely supportive, and includes medications for spasticity, baclofen pump, and treatment for pseudobulbar affect. The prognosis in PLS is more benign than ALS, making this a useful diagnostic category.

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“…No clear diagnostic neuroimaging markers have been identified in PLS. MRI changes reported in PLS have included (i) atrophy specifically of the precentral gyrus [67], (ii) reduced N-acetyl aspartate/choline ratio over the primary motor cortex on MR spectroscopy, suggesting neuronal loss [69], (iii) reduced fluorodeoxyglucose (FDG) uptake over the precentral gyrus on positron emission tomography (PET) studies [70], suggesting reduced metabolism, (iv) increased quantitative FLAIR MRI intensity in both corticospinal tracts greater than the change noted in classical ALS [71] and (v) reduced fractional anisotropy and increased mean diffusivity in the corticospinal tracts and motor fibres of the corpus callosum [72], suggesting that motor fibres of the descending corticospinal tract and also more proximal segments are involved.…”

Section: Neuroimaging In Plsmentioning

confidence: 99%

The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders

Menon

Vucic

2021

Brain Sciences

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Upper motor neuron (UMN) is a term traditionally used for the corticospinal or pyramidal tract neuron synapsing with the lower motor neuron (LMN) in the anterior horns of the spinal cord. The upper motor neuron controls resting muscle tone and helps initiate voluntary movement of the musculoskeletal system by pathways which are not completely understood. Dysfunction of the upper motor neuron causes the classical clinical signs of spasticity, weakness, brisk tendon reflexes and extensor plantar response, which are associated with clinically well-recognised, inherited and acquired disorders of the nervous system. Understanding the pathophysiology of motor system dysfunction in neurological disease has helped promote a greater understanding of the motor system and its complex cortical connections. This review will focus on the pathophysiology underlying progressive dysfunction of the UMN in amyotrophic lateral sclerosis and three other related adult-onset, progressive neurological disorders with prominent UMN signs, namely, primary lateral sclerosis, hereditary spastic paraplegia and primary progressive multiple sclerosis, to help promote better understanding of the human motor system and, by extension, related cortical systems.

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The Stripe of Primary Lateral Sclerosis

Cited by 33 publications

References 16 publications

Right temporal variant frontotemporal dementia with motor neuron disease

Right temporal variant frontotemporal dementia with motor neuron disease

Primary Lateral Sclerosis

The Upper Motor Neuron—Improved Knowledge from ALS and Related Clinical Disorders

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