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Kugelberg-Welander disease / Spinal Muscular Atrophy type 3

ICD-10	G121
ICD-9	335.11
OMIM	158600
DiseasesDB	12315
MedlinePlus	[1]
eMedicine	pmr/62
MeSH	{{{MeshNumber}}}

Kugelberg-Welander disease (also known as "juvenile spinal muscular atrophy, "spinal muscular atrophy type 3, or "spinal muscular atrophy type III") is an autosomal recessive muscular disease. It is a form of spinal muscular atrophy, or SMA. It appears between 2 and 17 years of age, and include abnormal manner of walking; difficulty running, climbing steps or rising from a chair and slight tremor of the fingers.

[edit] Treatment

Treatment is symptomatic and supportive and includes treating pneumonia, curvature of the spine and respiratory infections if present. Also, physical therapy, orthotic supports, and rehabilitation are useful. Genetic counseling is imperative.

[edit] Prognosis

Children with Kugelberg-Welander disease / spinal muscular atrophy type 3 may appear "normal" until they are five or 10 or even older. These children achieve independent walking and their weakness may be so mild that medical attention is not sought for years. Many children have a very benign course and may remain ambulatory for decades after the onset of symptoms. Other patients exhibit slowly progressive symptoms resulting in a loss of independent walking. Respiratory complications are uncommon and swallowing problems are rarely encountered.

[edit] External links

• sma at NINDS

• Fight SMA / Andrew's Buddies

Nervous system

Brain - Spinal cord - Central nervous system - Peripheral nervous system - Somatic nervous system - Autonomic nervous system - Sympathetic nervous system - Parasympathetic nervous system

This box: view • talk • edit Nervous system pathology, primarily CNS (G00-G47, 320-349)
Inflammatory diseases of the CNS	Meningitis (Arachnoiditis) - Encephalitis - Myelitis - Encephalomyelitis (Acute disseminated) - Tropical spastic paraparesis
Systemic atrophies primarily affecting the CNS	Huntington's disease - Spinocerebellar ataxia (Friedreich's ataxia, Ataxia telangiectasia, Hereditary spastic paraplegia) Spinal muscular atrophy: Werdnig-Hoffman disease - Kugelberg-Welander disease - Fazio Londe syndrome - MND (Amyotrophic lateral sclerosis (ALS), Progressive muscular atrophy (PMA), Progressive bulbar, Pseudobulbar, PLS)
Extrapyramidal and movement disorders	Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration - Dystonia (Spasmodic torticollis, Meige's syndrome, Blepharospasm) - Essential tremor - Myoclonus - Chorea - Restless legs syndrome - Stiff person syndrome
Other degenerative / demyelinating diseases	Alzheimer's disease - Pick's disease - Alpers' disease - Dementia with Lewy bodies - Leigh's disease - Multiple sclerosis - Devic's disease - Central pontine myelinolysis - Transverse myelitis
Seizure/epilepsy	Focal (Simple partial, Complex partial) - Generalised (Tonic-clonic, Absence, Atonic, Benign familial neonatal) - Lennox-Gastaut - West - Epilepsia partialis continua - Status epilepticus (Complex partial status epilepticus)
Headache	Migraine (Familial hemiplegic) - Cluster - Vascular - Tension
Vascular	Transient ischemic attack (Amaurosis fugax, Transient global amnesia) - Cerebrovascular disease (MCA, ACA, PCA, Foville's syndrome, Millard-Gubler syndrome, Lateral medullary syndrome, Weber's syndrome, Lacunar stroke)
Sleep disorders	Insomnia - Hypersomnia - Sleep apnea (Ondine's curse) - Narcolepsy - Cataplexy - Kleine-Levin syndrome
Other	Hydrocephalus (Normal pressure) - Idiopathic intracranial hypertension - Encephalopathy - Brain herniation - Cerebral edema - Reye's syndrome - Syringomyelia - Syringobulbia - Spinal cord compression