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20-September-2008 10:08:30 - Fazio-Londe disease July 2008 Fazio-Londe disease Classification and external resources ICD-10 G12.1 ICD-9 335.2 OMIM 211500 DiseasesDB 29491 MeSH D010244 Fazio-Londe disease, also called progressive bulbar palsy of childhood,1 is an inherited motor neuron disease found in children and young adults. Progressive bulbar palsy is characterized by progressive paralysis of muscles innervated by cranial nerves. Contents 1 Presentation 2 Prognosis 3 History 4 Eponym 5 References 6 External links Presentation It produces rapidly progressive weakness of tongue, face and pharyngeal muscles in a clinical pattern similar to myasthenia bulbar palsy. Neuromuscular transmission may be abnormal in these muscles because of rapid denervation and immature reinervation, and strength may improve with administration of cholinesterase inhibitors. Paralysis occurs secondary to degeneration of the motor neurons of the brain stem. It causes progressive bulbar paralysis due to involvement of motor neurons of the cranial nerve nuclei. The most frequent symptoms at onset of progressive bulbar paralysis of childhood has been a unilateral facial paralysis. It is followed in frequency by dysarthria due to facial weakness or by dysphagia. Palatal weakness and palpebral ptosis also have been reported in few patients. Both sexes can be affected. It has been proposed that Fazio-Londe disease and Brown-Vialetto-Van-Laere syndrome are a phenotypically associated condition.2 Prognosis Onset of first symptom has been reported between 1-12 years, with a mean age of onset at 8 years. Genetic expression is either an autosomal dominant or an autosomal recessive type. Clinical course can be divided into early 6 yrs age, predominance of respiratory symptoms and late course 6-20 years of age, predominance of motor symptoms on superior limbs. Progression to involve other cranial nerve muscles occurs over a period of months or years. In the Gomez review facial nerve was affected in all cases while hypoglossal nerve was involved in all except one case. Other cranial nerves involved were vagus, trigeminal, spinal accessory nerve, abducent, occulomotor and glossopharyngeal in this order. Corticospinal tract signs were found in 2 of the 14 patients. The disease may progress to patient's death in a period as short as 9 months or may have a slow evolution or may show plateaus. Post mortem examination of cases have found depletion of nerve cells in the nuclei of cranial nerves. The histologic alterations found in patient with Fazio-Londe disease were identical to those seen in Werdnig-Hoffman syndrome. Fazio-Londe disease, infantile progressive spinal muscular atrophy Werdnig-Hoffman syndrome, Juvenile progressive spinal muscular atrophy Kugelberg-Welander disease and the juvenile type of slowly progressive bulbar palsy, all have been considered variants of chronic progressive disease of lower motor neurons. History Berger, in 1876, first reported a case of 12-year-old child with progressive bulbar paralysis. From India, Reddy and Murthy first reported a Fazio-Londe disease case in 1982. Until now only 31 cases have been published in the literature.citation needed Eponym It is named for E. Fazio and Paul Londe.34 References ^ Online 'Mendelian Inheritance in Man' OMIM 211500 ^ Dipti S, Childs AM, Livingston JH, et al September 2005. Brown-Vialetto-Van Laere syndrome; variability in age at onset and disease progression highlighting the phenotypic overlap with Fazio-Londe disease. Brain Dev. 27 6: 443-446. doi:10.1016/j.braindev.2004.10.003. PMID 16122634. ^ synd/1909 at Who Named It ^ Londe, P. Paralysie bulbaire progressive, infantile et familiale. Rev. Med. 14: 212-254, 1894. External links 470155312 at GPnotebook v d e Pathology of the nervous system, primarily CNS G00-G47, 320-349 Inflammatory Meningitis Arachnoiditis - Encephalitis - Myelitis - Encephalomyelitis Acute disseminated - Tropical spastic paraparesis - Cavernous sinus thrombosis Systemic atrophies Huntington's disease - Spinocerebellar ataxia Friedreich's ataxia, Ataxia telangiectasia, Herary spastic paraplegia - Spinal muscular atrophy: Werdnig-Hoffman - Kugelberg-Welander - Fazio-Londe - MND ALS, PMA, PBP, PP, PLS Extrapyramidal and movement disorders Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration - Dystonia/Dyskinesia Spasmodic torticollis, Meige's, Blepharospasm - Essential tremor - Myoclonus - Lafora - Chorea Choreoathetosis - Restless legs - Stiff person Other degenerative/ demyelinating diseases dementia: Alzheimer's - Pick's - Dementia with Lewy bodies - Frontotemporal lobar degeneration mitochondrial disease: Leigh's demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis - Marchiafava-Bignami disease - CAMFAK syndrome - Alpers' Seizure/epilepsy Focal - Generalised - Status epilepticus - Myoclonic epilepsy Headache Migraine Familial hemiplegic - Cluster - Vascular - Tension Vascular Transient ischemic attack Amaurosis fugax, Transient global amnesia Cerebrovascular disease MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke Sleep disorders Insomnia - Hypersomnia - Sleep apnea Obstructive, Ondine's curse - Narcolepsy - Cataplexy - Kleine-Levin - Circadian rhythm sleep - Delayed sleep phase - Advanced sleep phase Intracranial hypertension Hydrocephalus Normal pressure - Idiopathic intracranial hypertension Other encephalopathy Brain herniation - Cerebral edema - Reye's Other spinal cord disease Syringomyelia - Syringobulbia - Morvan's syndrome - Spinal cord compression This disease article is a stub. Retrieved from http://en..org/wiki/Fazio-Londe_disease Categories: Disease stubs | Diseases | Genetic disorders | Neurological disordersHidden categories: Articles needing additional references from July 2008 | All articles with statements | Articles with statements since January 2008 Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Polski This page was last modified on 6 July 2008, at 08:35
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