primary lateral sclerosis

WordNet

1. of first rank or importance or value; direct and immediate rather than secondary; "primary goals"; "a primary effect"; "primary sources"; "a primary interest"
2. a preliminary election where delegates or nominees are chosen (同)primary_election
3. one of the main flight feathers projecting along the outer edge of a birds wing (同)primary feather, primary quill
4. (astronomy) a celestial body (especially a star) relative to other objects in orbit around it
5. not derived from or reducible to something else; basic; "a primary instinct"
6. lying away from the median and sagittal plane of a body; "lateral lemniscus"
7. situated at or extending to the side; "the lateral branches of a tree"; "shot out sidelong boughs"- Tennyson (同)sidelong
8. comparative of the adverb `late; "he stayed later than you did"
9. coming at a subsequent time or stage; "without ulterior argument"; "the mood posterior to" (同)ulterior, posterior
10. any pathological hardening or thickening of tissue (同)induration

PrepTutorEJDIC

1. 『第一の』,『主要な』 / 『初期の』,『初等の』,初級の / 『根本的な』,基本的な,本来の,直接的な / 《名詞の前にのみ用いて》(回路・コイル・巻き・電流などが)一次の / 原色(三原色の一つ) / 《米》=primary election
2. 横の,側面の;横からの / (音声が)側音の / 側面,(道路の)側溝(そっこう) / 側音(英語の[1]など)
3. 《補語にのみ用いて》(人などが)(…するのが)『もっと遅い』《+『in』+『名』(do『ing』)》 / (時刻・時期が)『もっと遅い』,もっと後の / 後で,のちほど / (lateの比較級)(定刻・予定時間・他の人(物))より遅れて,遅く
4. (組織・血管などの)硬化,硬化症

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/03/24 12:41:26」(JST)

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Primary lateral sclerosis
Classification and external resources
Specialty	neurology
ICD-10	G12.2
ICD-9-CM	335.24
OMIM	606353
DiseasesDB	29150
eMedicine	neuro/324
MeSH	D016472

Primary lateral sclerosis (PLS) is a rare neuromuscular disease characterized by progressive muscle weakness in the voluntary muscles. PLS belongs to a group of disorders known as motor neuron diseases. Motor neuron diseases develop when the nerve cells that control voluntary muscle movement degenerate and die, causing weakness in the muscles they control.

PLS only affects upper motor neurons. There is no evidence of the degeneration of spinal motor neurons or muscle wasting (amyotrophy) that occurs in amyotrophic lateral sclerosis (ALS).

Symptoms and progression

Onset of PLS usually occurs spontaneously after age 50 and progresses gradually over a number of years, or even decades. The disorder usually begins in the legs, but it may start in the tongue or the hands. Symptoms may include difficulty with balance, weakness and stiffness in the legs, and clumsiness. Other common symptoms are spasticity (involuntary muscle contraction due to the stretching of muscle. Muscle contraction is dependent on the velocity of the stretch) in the hands, feet, or legs, foot dragging, and speech and swallowing problems due to involvement of the facial muscles. Breathing may also become compromised in the later stages of the disease, causing those patients who develop ventilatory failure to require noninvasive ventilatory support. Hyperreflexia is another key feature of PLS as seen in patients presenting with the Babinski's sign. Some people present with emotional lability and bladder urgency, and occasionally people with PLS experience mild cognitive changes detectable on neuropsychological testing, particularly on measures of executive function.

PLS is not considered hereditary when onset is in adulthood; however, juvenile primary lateral sclerosis (JPLS) has been linked to a mutation in the ALS2 gene which encodes the cell-signalling protein alsin.

The issue of whether PLS exists as a different entity from ALS is not clear, as some patients initially diagnosed as having PLS ultimately develop lower motor neuron signs.

There are no specific tests for the diagnosis of PLS. Therefore, the diagnosis occurs as the result of eliminating other possible causes of the symptoms and by an extended observation period.

Spasticity

Primary lateral sclerosis (PLS) usually presents with gradual-onset, progressive, lower-extremity stiffness and pain due to muscle spasticity. Onset is often asymmetrical. Although the muscles do not appear to atrophy as in ALS (at least initially), the disabling aspect of PLS is muscle spasticity and cramping, and intense pain when those muscles are stretched, resulting in joint immobility. A normal walking stride may become a tiny step shuffle with related instability and falling.

Cause

Diagnosis

Treatment

Treatment for individuals with PLS is symptomatic. Baclofen and tizanidine may reduce spasticity. Quinine or phenytoin may decrease cramps. Some patients who do not receive adequate relief from oral treatment may consider intrathecal baclofen (i.e., infusion of medication directly into the cerebrospinal fluid via a surgically placed continuous infusion pump). However, patients are carefully selected for this type of procedure to ensure that they will likely benefit from this invasive procedure.

Physical therapy often helps prevent joint immobility. Speech therapy may be useful for those with involvement of the facial muscles. Physiotherapy treatment focuses on reducing muscle tone, maintaining or improving range of motion, increasing strength and coordination, and improving functional mobility. In PLS, stretching is thought to improve flexibility and can also reduce muscle spasticity and cramps.

Patients with PLS may find it beneficial to have an evaluation, as well as follow-up visits at multidisciplinary clinics, similar to those available for people with ALS. These multidisciplinary clinics may provide patients with the necessary treatment that they require by having an occupational therapist, physical therapist, speech language pathologist, dietician and nutritionist, all in one site.

Prognosis

Patients can often live with PLS for many years and very often outlive their neurological disease and succumb to some unrelated condition. There is currently no effective cure, and the progression of symptoms varies. Some people may retain the ability to walk without assistance, but others eventually require wheelchairs, canes, or other assistive devices.

References

External links

• eNotes
• mayoclinic.com
• primary_lateral_sclerosis at NINDS
• PLS movie

UpToDate Contents

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• 1. 筋萎縮性側索硬化症およびその他の運動ニューロン疾患の臨床的特徴 clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease
• 2. 筋萎縮性側索硬化症およびその他の運動ニューロン疾患の診断 diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease
• 3. 筋萎縮性側索硬化症の症状に基づくマネージメント symptom based management of amyotrophic lateral sclerosis
• 4. 遺伝性痙性対麻痺 hereditary spastic paraplegia
• 5. 脊髄に影響を与える疾患 disorders affecting the spinal cord

English Journal

• Mathematical models of α-synuclein transport in axons.

• Kuznetsov IA1, Kuznetsov AV2.
• Computer methods in biomechanics and biomedical engineering.Comput Methods Biomech Biomed Engin.2016 Apr;19(5):515-26. doi: 10.1080/10255842.2015.1043628. Epub 2015 Jul 24.
• To investigate possible effects of diffusion on α-synuclein (α-syn) transport in axons, we developed two models of α-syn transport, one that assumes that α-syn is transported only by active transport, as part of multiprotein complexes, and a second that assumes an interplay between motor-driven
• PMID 26207359

• C9orf72 expansion presenting as an eating disorder.

• Sanders P1, Ewing I1, Ahmad K2.
• Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia.J Clin Neurosci.2016 Mar;25:157-9. doi: 10.1016/j.jocn.2015.06.019. Epub 2015 Nov 4.
• This report describes a 64-year-old woman with a strong family history of motor neuron disease, whose diagnosis of behavioural variant frontotemporal dementia was delayed due to her initial presentation with atypical manifestations, including restriction of oral intake resulting in low weight, disor
• PMID 26547294

Japanese Journal

• 在宅筋萎縮性側索硬化症患者の主介護者の介護肯定感とその関連要因

• 日本看護研究学会雑誌 Journal of Japan Society of Nursing Research 38(2), 43-55, 2015-06
• NAID 40020592389

• 在宅筋萎縮性側索硬化症患者の主介護者の介護肯定感とその関連要因

• 日本看護研究学会雑誌 38(2), 2_43-2_55, 2015
• NAID 130005119238

Related Pictures

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★リンクテーブル★

リンク元	「原発性側索硬化症」「secondary motor neuron disease」「lateral sclerosis」「motor system disease」「anterior horn cell disease」
関連記事	「primary」「lateral」「lateral sclerosis」

「原発性側索硬化症」

　　[★]

英

primary lateral sclerosis

関

側索硬化症、運動ニューロン疾患、下位運動ニューロン疾患、前角細胞疾患、上位運動ニューロン疾患、運動系疾患、二次運動ニューロン病

「secondary motor neuron disease」

　　[★]

二次運動ニューロン病

関

anterior horn cell disease、lateral sclerosis、lower motor neuron disease、motor neuron disease、motor system disease、primary lateral sclerosis、upper motor neuron disease

「lateral sclerosis」

　　[★] 側索硬化症

関

anterior horn cell disease、lower motor neuron disease、motor neuron disease、motor system disease、primary lateral sclerosis、secondary motor neuron disease、upper motor neuron disease

「motor system disease」

　　[★]

• 運動系疾患

関

anterior horn cell disease、lateral sclerosis、lower motor neuron disease、motor neuron disease、primary lateral sclerosis、secondary motor neuron disease、upper motor neuron disease

「anterior horn cell disease」

　　[★]

• 前角細胞疾患

関

lateral sclerosis、lower motor neuron disease、motor neuron disease、motor system disease、primary lateral sclerosis、secondary motor neuron disease、upper motor neuron disease

「primary」

　　[★]

• adj.

• 一次の、第一次の、主要な、(化学)第一級の、一級の、初生の、(病名)原発性の、原発の、プライマリーの

関

cardinal、chief、first-order、foremost、main、mainly、major、master、primarily、principal、principally

　 　 　 　 　 　

「lateral」

　　[★]

• adj.

• 側面の、外側の、側の、外側性の、ラテラル

関

aspect、external、externally、flanking、laterale、lateralis、laterally、outer、outside、profile、side、sided、sidedness

　 　 　

「lateral sclerosis」

　　[★] 側索硬化症

関

anterior horn cell disease、lower motor neuron disease、motor neuron disease、motor system disease、primary lateral sclerosis、secondary motor neuron disease、upper motor neuron disease