Primary lateral sclerosis: diagnosis and management

Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder at the upper motor neurone extreme of the spectrum of motor neurone disease. The diagnosis is clinical and based on the characteristic features of slowly progressive spasticity beginning in the lower limbs, or more rarely with spastic dysarthria, typically presenting around 50 years of age. The absence of lower motor neurone involvement is considered to be a defining feature, but confident distinction of PLS from upper motor neurone-predominant forms of amyotrophic lateral sclerosis may be difficult in the first few years. Corticobulbar involvement in PLS is frequently accompanied by emotionality. While there may be dysphagia, gastrostomy is rarely required to maintain nutrition. Cognitive dysfunction is recognised, though dementia is rarely a prominent management issue. PLS is not necessarily life shortening. Specialised multidisciplinary care is recommended. Increasing international research cooperation is required if the aspiration of dedicated therapeutic trials for PLS is to be achieved.

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A case of presumptive primary lateral sclerosis with upper and lower motor neurone pathology

Journal of Clinical Neuroscience ◽

10.1016/j.jocn.2004.08.032 ◽

2005 ◽

Vol 12 (6) ◽

pp. 706-709 ◽

Cited By ~ 5

Author(s):

Cathy L. Short ◽

Grace Scott ◽

Peter C. Blumbergs ◽

Simon A. Koblar

Keyword(s):

Motor Neurone ◽

Primary Lateral Sclerosis ◽

Primary Lateral ◽

Lateral Sclerosis

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Natural History of “Pure” Primary Lateral Sclerosis

Neurology ◽

10.1212/wnl.0000000000011771 ◽

2021 ◽

Vol 96 (17) ◽

pp. e2231-e2238 ◽

Cited By ~ 1

Author(s):

Anhar Hassan ◽

Shivam Om Mittal ◽

William T. Hu ◽

Keith A. Josephs ◽

Eric J. Sorenson ◽

...

Keyword(s):

Disease Duration ◽

Neurodegenerative Disorder ◽

Retrospective Chart Review ◽

Feeding Tube ◽

Primary Lateral Sclerosis ◽

Bulbar Onset ◽

History Of ◽

Primary Lateral ◽

Lateral Sclerosis

ObjectiveTo assess whether primary lateral sclerosis (PLS), classified as pure when the EMG is normal, converts to amyotrophic lateral sclerosis (ALS) after longitudinal follow-up.MethodsRetrospective chart review was performed of patients with pure PLS at Mayo Clinic in Rochester, MN (1990–2016). Inclusion criteria required a normal EMG during the first 4 years of symptoms.ResultsForty-three patients had pure PLS (25 female, 58%) with a median onset age of 50 years (range 38–78 years) and median follow-up at 9 years’ disease duration (range 4–36 years). The ascending paraparesis phenotype (n = 30, 70%) was most common, followed by hemiparetic onset (n = 9, 21%) and bulbar onset (n = 4, 9%). Among the 30 paraparetic-onset cases, bladder symptoms (n = 18, 60%) and dysarthria (n = 15, 50%) were more common than pseudobulbar affect (n = 9, 30%) and dysphagia (n = 8, 27%). By the last follow-up, 17 of 30 (56%) used a cane and 6 (20%) required a wheelchair. The paraparetic variant, compared with hemiparetic and bulbar onset, had the youngest onset (48 vs 56 vs 60 years, respectively; p = 0.02). Five patients died; 1 patient required a feeding tube; and none required permanent noninvasive ventilation. Two patients developed an idiopathic multisystem neurodegenerative disorder, which surfaced after 19 and 20 years. Two patients developed minor EMG abnormalities. The remainder 39 had persistently normal EMGs.ConclusionsPure PLS did not convert to ALS after a median of 9 years’ disease duration follow-up in our study population. The ascending paraparetic phenotype was most common, with earlier onset and frequent bladder involvement. After years of pure PLS, <5% develop a more pervasive neurodegenerative disorder.

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Primary lateral sclerosis: a case report with SPECT study

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x1998000300018 ◽

1998 ◽

Vol 56 (3A) ◽

pp. 465-471 ◽

Cited By ~ 4

Author(s):

WALTER OLESCHKO ARRUDA ◽

MAURÍCIO COELHO NETO

Keyword(s):

Neurological Disorders ◽

Lower Limbs ◽

Upper Limbs ◽

Primary Lateral Sclerosis ◽

Cranial Nerve Involvement ◽

Cognitive Changes ◽

Sensory Modalities ◽

Primary Lateral ◽

Lateral Sclerosis

Primary lateral sclerosis (PLS) is a neurodegenerative disease with progressive corticospinal involvement and characterized by lower limbs spasticity followed by upper limbs involvement, rare cranial nerve involvement, typical sparing of all sensory modalities, sphincteric function and eventually mild cognitive changes. The authors report a case of PLS in a 43-year-old woman with 3 years of clinical follow-up and extensive laboratory investigation, including a SPECT study which disclosed bilateral frontal motor area hypometabolism. Several aspects about this unique disease were reviewed, including differential diagnosis with other more common neurological disorders.

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Primary lateral sclerosis: consensus diagnostic criteria

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2019-322541 ◽

2020 ◽

Vol 91 (4) ◽

pp. 373-377 ◽

Cited By ~ 15

Author(s):

Martin R Turner ◽

Richard J Barohn ◽

Philippe Corcia ◽

John K Fink ◽

Matthew B Harms ◽

...

Keyword(s):

Motor Neuron ◽

Diagnostic Criteria ◽

New Technologies ◽

Neurodegenerative Disorder ◽

Diagnostic Delay ◽

Therapeutic Trial ◽

Primary Lateral Sclerosis ◽

Therapeutic Development ◽

Primary Lateral ◽

Lateral Sclerosis

Primary lateral sclerosis (PLS) is a neurodegenerative disorder of the adult motor system. Characterised by a slowly progressive upper motor neuron syndrome, the diagnosis is clinical, after exclusion of structural, neurodegenerative and metabolic mimics. Differentiation of PLS from upper motor neuron-predominant forms of amyotrophic lateral sclerosis remains a significant challenge in the early symptomatic phase of both disorders, with ongoing debate as to whether they form a clinical and histopathological continuum. Current diagnostic criteria for PLS may be a barrier to therapeutic development, requiring long delays between symptom onset and formal diagnosis. While new technologies sensitive to both upper and lower motor neuron involvement may ultimately resolve controversies in the diagnosis of PLS, we present updated consensus diagnostic criteria with the aim of reducing diagnostic delay, optimising therapeutic trial design and catalysing the development of disease-modifying therapy.

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Slowly progressive dysarthria (pseudobulbar palsy) in primary lateral sclerosis

Klinische Neurophysiologie ◽

10.1055/s-0030-1250885 ◽

2010 ◽

Vol 41 (01) ◽

Author(s):

P Urban ◽

I Wellach ◽

C Pohlmann

Keyword(s):

Pseudobulbar Palsy ◽

Primary Lateral Sclerosis ◽

Primary Lateral ◽

Lateral Sclerosis

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Mills' syndrome. A clinical case of a rare degenerative pathology of the central nervous system

Spravočnik vrača obŝej praktiki (Journal of Family Medicine) ◽

10.33920/med-10-2004-07 ◽

2020 ◽

pp. 57-60

Author(s):

Konstantin Gulyabin

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Neurological Diseases ◽

Cortical Atrophy ◽

Primary Lateral Sclerosis ◽

System A ◽

The Central Nervous System ◽

Primary Lateral ◽

Lateral Sclerosis ◽

Mills Syndrome

Mills' syndrome is a rare neurological disorder. Its nosological nature is currently not completely determined. Nevertheless, Mills' syndrome is considered to be a rare variant of the degenerative pathology of the central nervous system – a variant of focal cortical atrophy. The true prevalence of this pathology is unknown, since this condition is more often of a syndrome type, observed in the clinical picture of a number of neurological diseases (primary lateral sclerosis, frontotemporal dementia, etc.) and is less common in isolated form.

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