亜急性連合性脊髄変性症
WordNet
- join for a common purpose or in a common action; "These forces combined with others"
- harvester that heads and threshes and cleans grain while moving across the field
- add together from different sources; "combine resources"
- the process of declining from a higher to a lower level of effective power or vitality or essential quality (同)devolution
- passing from a more complex to a simpler biological form (同)retrogression
- less than acute; relating to a disease present in a person with no symptoms of it
- made or joined or united into one
PrepTutorEJDIC
- …'を'『結合する』,合同する / …'を'兼ね備える;(…と)…'を'兼ね合わさせる《+『名』+『with』+『名』》 / …'を'化合させる;(…と)…'を'化合させる《+『名』+『with』+『名』》 / 『結合する』,連合する,合同する / (…と)化合する《+『with』+『名』》 / 連合,合同;(特に)企業合同,政治連合 / (また『combine harvester』)(刈り取りと脱穀が同時にできる)複式収穫機,コンバイン
- 退歩,退化,墜落
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/11/26 06:43:01」(JST)
[Wiki en表示]
| Subacute combined degeneration of spinal cord |
| Classification and external resources |
Diagram of the principal fasciculi of the spinal cord. (In subacute combined degeneration of spinal cord, the "combined" refers to the fact that the dorsal columns and lateral corticospinal tracts are both affected, in contrast to tabes dorsalis which is selective for the dorsal columns.) |
| DiseasesDB |
12591 |
| MeSH |
D052879 |
Subacute combined degeneration of spinal cord, also known as Lichtheim's disease,[1][2] refers to degeneration of the posterior and lateral columns of the spinal cord as a result of vitamin B12 deficiency (most common), vitamin E deficiency. It is usually associated with pernicious anemia.
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Contents
- 1 Etiologies
- 2 Presentation
- 3 Tests
- 4 Treatment
- 5 References
- 6 External links
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Etiologies
This condition can be due to a dietary deficiency of B12, malabsorption of B12 in the terminal ileum, lack of intrinsic factor secreted from gastric parietal cells, low gastric pH inhibiting attachment of intrinsic factor to ileal receptors. [3]
Presentation
The onset is gradual and uniform. The pathological findings of subacute combined degeneration consist of patchy losses of myelin in the dorsal and lateral columns. Patients present with weakness of legs, arms, trunk, tingling and numbness that progressively worsens. Vision changes and change of mental state may also be present. Bilateral spastic paresis may develop and pressure, vibration and touch sense are diminished. A positive Babinski sign may be seen.[citation needed] Prolonged deficiency of vitamin B12 leads to irreversible nervous system damage.
If someone is deficient in vitamin B12 and folic acid, the vitamin B12 deficiency must be treated first to avoid precipitating subacute combined degeneration of the cord (giving folic acid first will turn the remaining B12 into methylcobalamin which will not be able to participate in fatty acid metabolism).
Tests
Serum B12 Malonic Acid Methylmalonic Acid Schilling Test Complete Blood Count- looking for megaloblastic anemia if there is also folic acid deficiency or macrocytic anemia
MRI- T2 images may reveal increased signal within the white matter of the spinal cord predominately in the posterior columns but also may be in the spinothalamic tracts.
Treatment
Therapy with vitamin B12 results in partial to full recovery, depending on the duration and extent of neurodegeneration.
References
- ^ synd/492 at Who Named It?
- ^ L. Lichtheim. Zur Kenntnis der perniciösen Anämie. Verhandlungen des Deutschen Kongress für innere Medizin, 1889, 6: 84-96. 42: 1887.
- ^ Greenburg, Mark (2010). Handbook of Neurosurgery 7th Edition. New York: Thieme Publishers. pp. 1187-1188. ISBN 978-1-60406-326-4.
External links
- http://www.neuropathologyweb.org/chapter8/chapter8Nutritional.html
- http://www.macrorad.com/case-reports/subacute-combined-degeneration.html
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Nutrition disorders (E40–E68, 260–269)
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Hypoalimentation/
malnutrition |
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Protein-energy
malnutrition
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Kwashiorkor · Marasmus · Catabolysis
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Avitaminosis
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B vitamins
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B1: Beriberi/Wernicke's encephalopathy (Thiamine deficiency) · B2: Ariboflavinosis · B3: Pellagra (Niacin deficiency) · B6: Pyridoxine deficiency · B7: Biotin deficiency · B9: Folate deficiency · B12: Vitamin B12 deficiency
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Other
vitamins
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A: Vitamin A deficiency/Bitot's spots · C: Scurvy · D: Hypovitaminosis D/Rickets/Osteomalacia · E: Vitamin E deficiency · K: Vitamin K deficiency
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Mineral
deficiency
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Sodium · Potassium · Magnesium · Calcium · Iron · Zinc · Manganese · Copper · Iodine · Chromium · Molybdenum · Selenium (Keshan disease)
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| Hyperalimentation |
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Overweight · Obesity
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Childhood obesity · Obesity hypoventilation syndrome · Abdominal obesity
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Vitamin poisoning
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Hypervitaminosis A · Hypervitaminosis D · Hypervitaminosis E
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Mineral overload
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see inborn errors of metal metabolism, toxicity
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noco, nuvi, sysi/epon, met
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Pathology of the nervous system, primarily CNS (G04–G47, 323–349)
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| Inflammation |
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Brain
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- Encephalitis
- Viral encephalitis
- Herpesviral encephalitis
- Cavernous sinus thrombosis
- Brain abscess
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Spinal cord
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- Myelitis: Poliomyelitis
- Demyelinating disease
- Tropical spastic paraparesis
- Epidural abscess
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Both/either
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- Encephalomyelitis
- Meningoencephalitis
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Brain/
encephalopathy |
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Degenerative
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Extrapyramidal and
movement disorders
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- Basal ganglia disease
- Parkinsonism
- PKAN
- Tauopathy
- Striatonigral degeneration
- Hemiballismus
- HD
- OA
- Dyskinesia
- Dystonia
- Status dystonicus
- Spasmodic torticollis
- Meige's
- Blepharospasm
- Athetosis
- Chorea
- Myoclonus
- Akathesia
- Tremor
- Essential tremor
- Intention tremor
- Restless legs
- Stiff person
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Dementia
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- Tauopathy
- Alzheimer's
- Primary progressive aphasia
- Frontotemporal dementia/Frontotemporal lobar degeneration
- Pick's
- Dementia with Lewy bodies
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Mitochondrial disease
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Demyelinating
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- autoimmune
- Multiple sclerosis
- Neuromyelitis optica
- Schilder's disease
- hereditary
- Adrenoleukodystrophy
- Alexander
- Canavan
- Krabbe
- ML
- PMD
- VWM
- MFC
- CAMFAK syndrome
- Central pontine myelinolysis
- Marchiafava-Bignami disease
- Alpers' disease
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Episodic/
paroxysmal
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Seizure/epilepsy
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- Focal
- Generalised
- Status epilepticus
- Myoclonic epilepsy
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Headache
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Cerebrovascular
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- TIA
- Amaurosis fugax
- Transient global amnesia
- Acute aphasia
- Stroke
- MCA
- ACA
- PCA
- Foville's
- Millard-Gubler
- Lateral medullary
- Weber's
- Lacunar stroke
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Sleep disorders
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- Insomnia
- Hypersomnia
- Sleep apnea
- Obstructive
- Ondine's curse
- Narcolepsy
- Cataplexy
- Kleine-Levin
- Circadian rhythm sleep disorder
- Advanced sleep phase disorder
- Delayed sleep phase disorder
- Non-24-hour sleep-wake disorder
- Jet lag
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CSF
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- Intracranial hypertension
- Hydrocephalus/NPH
- Idiopathic intracranial hypertension
- Cerebral edema
- Intracranial hypotension
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Other
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- Brain herniation
- Reye's
- Hepatic encephalopathy
- Toxic encephalopathy
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Spinal cord/
myelopathy |
- Syringomyelia
- Syringobulbia
- Morvan's syndrome
- Vascular myelopathy
- Foix-Alajouanine syndrome
- Spinal cord compression
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| Both/either |
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Degenerative
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SA
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- Friedreich's ataxia
- Ataxia telangiectasia
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MND
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- LMN only:
- Distal hereditary motor neuropathies
- Spinal muscular atrophies
- SMA
- SMAX1
- SMAX2
- DSMA1
- SMA-PCH
- SMA-LED
- PMA
- PBP
- Fazio-Londe
- Infantile progressive bulbar palsy
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anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp
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noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr
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proc, drug(N1A/2AB/C/3/4/7A/B/C/D)
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Lesions of spinal cord and brain
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Spinal cord/
vascular myelopathy |
- sensory: Sensory ataxia
- Tabes dorsalis
- motor: Motor neurone disease
- mixed: Brown-Séquard syndrome
- cord syndrome (Posterior
- Anterior
- Central/Syringomyelia)
- Subacute combined degeneration of spinal cord (B12)
- Cauda equina syndrome
- Anterior spinal artery syndrome
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| Brainstem |
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Medulla (CN 8, 9, 10, 12)
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- Lateral medullary syndrome/Wallenberg
- Medial medullary syndrome/Dejerine
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Pons (CN 5, 6, 7, 8)
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- Lateral pontine syndrome (AICA) (lateral)
- Medial pontine syndrome/Millard-Gubler syndrome
- basilar/Foville's syndrome
- Locked-in syndrome (ventral)
- Internuclear ophthalmoplegia
- One and a half syndrome
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Midbrain (CN 3, 4)
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- Weber's syndrome
- Benedikt syndrome
- Parinaud's syndrome
- Nothnagel's syndrome
- Claude's syndrome
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Other
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| Cerebellum |
- lateral (Dysmetria
- Dysdiadochokinesia
- Intention tremor)
- medial (Cerebellar ataxia)
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| Basal ganglia |
- Chorea
- Dystonia
- Parkinson's disease
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| Cortex |
- ACA syndrome
- MCA syndrome
- PCA syndrome
- frontal lobe: Expressive aphasia
- Abulia
- parietal lobe: Receptive aphasia
- Hemispatial neglect
- Gerstmann syndrome
- Astereognosis
- occipital lobe: Balint's syndrome
- Cortical blindness
- Pure alexia
- temporal lobe: Cortical deafness
- Prosopagnosia
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| Other |
- Subclavian steal syndrome
- Upper motor neurone lesion (Clasp-knife response)
- Lower motor neurone lesion
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anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp
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noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr
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proc, drug(N1A/2AB/C/3/4/7A/B/C/D)
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UpToDate Contents
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English Journal
- Subacute combined degeneration of the spinal cord despite prophylactic vitamin B12 treatment.
- Jaiser SR, Winston GP.SourceDepartment of Neurology, Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne NE1 4LP, United Kingdom Tel.: +44 (0)191 222 3961; fax +44 (0)191 222 5227.. Electronic address: stephan.jaiser@ncl.ac.uk.
- Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia.J Clin Neurosci.2012 Nov;19(11):1607. doi: 10.1016/j.jocn.2012.03.014. Epub 2012 Aug 25.
- PMID 22925415
- Subacute combined degeneration without nutritional anemia.
- Miscusi M, Testaverde L, Rago A, Raco A, Colonnese C.SourceSection of Neurosurgery, Department of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Corso della Repubblica 79, 04100 Latina, Italy. Electronic address: massimo.miscusi@uniroma1.it.
- Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia.J Clin Neurosci.2012 Sep 27. pii: S0967-5868(12)00255-X. doi: 10.1016/j.jocn.2012.01.039. [Epub ahead of print]
- Subacute combined degeneration (SCD) is a rare neurological complication of cobalamin deficiency, characterized by demyelination of the dorsal and lateral spinal cord. The diagnosis and treatment of SCD can be delayed if a patient does not present with clear clinical and laboratory signs of nutritio
- PMID 23022212
Japanese Journal
- 症例報告 悪性貧血や亜急性脊髄連合変性症がなく,認知症,失調性歩行を呈したビタミンB12欠乏の1例
- 悪性貧血と亜急性連合性脊髄変性症に合併した栄養欠乏性視神経症の1例 (特集 第63回日本臨床眼科学会講演集(2))
Related Links
- Subacute combined degeneration is caused by a vitamin B12 deficiency. It mainly affects the spinal cord. But its effects on the brain and the peripheral (body) nerves are the reason for use of the term “combined.” At first ...
- subacute combined degeneration Subacute combined degeneration of the spinal cord Neurology A disorder caused by vitamin B 12 deficiency, characterized by weakness, sensory defects, mental changes, vision defects, often ...
Related Pictures
★リンクテーブル★
[★]
- 英
- subacute combined degeneration of spinal cord, subacute combined degeneration of the spinal cord, SCDC
- 同
- 後索側索硬化症 posterolateral sclerosis、subacute combined degeneration of the dorsal (posterior) and lateral spinal columns (uptodate)
- 関
- ビタミンB12、subacute combined degeneration？
[show details]
病因
- メカニズムは不明であるがミエリンの形成に異常が生じる (参考1)
病理
- 脊髄後索と側索の脱髄・変性
- 大脳白質・末梢神経にも髄鞘の脱髄と軸索の変性が起こる。軸索の変性は軽度。
病態
- 錐体路徴候、深部感覚障害・運動失調(BET.156)
- 初発症状は感覚異常と深部感覚の異常であり、重度の脱力、痙性(spasticity)、痙縮(clonus)、麻痺、さらに便失禁・便失禁をきたすことがある。(参考1)
- 下肢遠位部が優位となる。
- 1) 後索症状：感覚障害(特に深部感覚)、後索性失調、ロンベルク徴候
- 2) 錐体路徴候：痙性対麻痺、膝蓋腱反射亢進、バビンスキー徴候
- 3) 末梢神経の脱髄(参考1)：四肢末梢の痺れ、痛み、深部腱反射消失
- 4) 中枢神経症状(記憶喪失、易刺激性、および認知症)(参考1)
病因
検査
- 画像は参考3より引用
診断
- 症候と検査(血中ビタミンB12、シリングテスト)により診断
治療
参考
- http://www.nurs.or.jp/~academy/igaku/s7/s75182.htm
- . [charged] Etiology and clinical manifestations of vitamin B12 and folic acid deficiency - uptodate [1]
- 3. Anitha Sen et al;Ann Indian Acad Neurol 2013 Apr;16(2):255-8. Spinal MR Imaging in Vitamin B12 Deficiency: Case Series; Differential Diagnosis of Symmetrical Posterior Spinal Cord Lesions
- https://pubmed.ncbi.nlm.nih.gov/23956577/
[★]
- 英
- subacute combined degeneration
[★]
亜急性連合性脊髄変性症
[★]
亜急性連合性脊髄変性症
[★]
- 関
- ally、associate、bind、binding、bond、bonding、combination、combined application、complicate、concomitant、concurrent、conjoin、conjugate、conjugation、conjunction、connect、connection、couple、dock、engage、engagement、join、ligate、linkage、merge、merger、symphysial、union
[★]
- 関
- degeneracy、degenerate、degenerative、denaturation、denature、involution、retract、retrograde degeneration、retrograde degradation、unfolding
[★]
- 関
- mixed