| 词条 | Diffuse myelinoclastic sclerosis |
| 释义 |
| name = Diffuse myelinoclastic sclerosis | image = | caption = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}Diffuse myelinoclastic sclerosis (DMS), sometimes referred to as Schilder's disease, is a very infrequent neurodegenerative disease that presents clinically as pseudotumoural demyelinating lesions, that make its diagnosis difficult. It usually begins in childhood, affecting children between 5 and 14 years old,[1][2] but cases in adults are possible.[3] This disease is considered one of the borderline forms of multiple sclerosis because some authors consider them different diseases and others MS variants. Other diseases in this group are neuromyelitis optica (NMO), Balo concentric sclerosis and Marburg multiple sclerosis.[4] SymptomsSymptoms are similar to those in multiple sclerosis and may include dementia, aphasia, seizures, personality changes, poor attention, tremors, balance instability, incontinence, muscle weakness, headache, vomiting, and vision and speech impairment.[5] DiagnosticThe Poser criteria for diagnosis are:[6]
Neuropathological examinationThe typical demyelinating plaques in Schilder's sclerosis are usually found bilaterally in the semioval center; both hemispheres are almost completely occupied by large, well defined lesions. Although plaques of this kind are largely prevalent in Schilder's sclerosis, smaller lesions can also be observed. ImmunologyIt has been reported that DMS cases show no oligoclonal bands, being therefore distinct from standard MS.[7] TreatmentsManagement Corticosteroids may be effective in some patients. Additional treatment options are beta-interferon or immunosuppressive therapy. Otherwise management is supportive and includes physiotherapy, occupational therapy and nutritional support in the later stages as patients lose their ability to eat. Prognosis and clinical courseThe prognosis of this disease is very variable and can take three different courses: a monophasic, not remitting; [2],[8] remitting;[9] [10][11]and finally, progressive, with increase in deficits.[12] HistoryIt was first described by Paul Ferdinand Schilder in 1912,[13][14] and for nearly one hundred years the term "Schilder disease" was used to describe it, but the same name was also used for some other white matter pathologies described by him.[15] In 1986 Poser tried to restrict the use of Schilder's disease name to the disease described here, but this name has remained still ambiguous. The name comes from a traditional classification of demyelinating diseases in two groups: demyelinating myelinoclastic diseases and demyelinating leukodystrophic diseases. In the first group, a normal and healthy myelin is destroyed by a toxic, chemical, or autoimmune substance. In the second group, myelin is abnormal and degenerates.[16] The second group was denominated dysmyelinating diseases by Poser.[17] References1. ^{{cite journal |vauthors=Garrido C, Levy-Gomes A, Teixeira J, Temudo T |title=[Schilder's disease: two new cases and a review of the literature] |language=Spanish|journal=Revista de Neurologia |volume=39 |issue=8 |pages=734–8 |year=2004 |pmid=15514902}} 2. ^1 {{cite journal |vauthors=Afifi AK, Bell WE, Menezes AH, Moore SA |title=Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature |journal=J. Child Neurol. |volume=9 |issue=4 |pages=398–403 |year=1994 |pmid=7822732 |doi=10.1177/088307389400900412|citeseerx=10.1.1.1007.559 }} 3. ^{{cite journal | last1 = Bacigaluppi | first1 = S | last2 = Polonara | first2 = G | last3 = Zavanone | first3 = ML | last4 = Campanella | first4 = R | last5 = Branca | first5 = V | last6 = Gaini | first6 = SM | last7 = Tredici | first7 = G | last8 = Costa | first8 = A | title = Schilder's disease: non-invasive diagnosis? :A case report and review. | journal = Neurological Sciences | volume = 30 | issue = 5 | pages = 421–30 | year = 2009 | pmid = 19609739 | doi = 10.1007/s10072-009-0113-z }} 4. ^{{cite journal |author=Fontaine B |title=[Borderline forms of multiple sclerosis] |language=French |journal=Rev. Neurol. (Paris) |volume=157 |issue=8–9 Pt 2 |pages=929–34 |year=2001 |pmid=11787357}} 5. ^NINDS Schilder's Disease Information Page 6. ^{{cite journal |vauthors=Poser CM, Goutières F, Carpentier MA, Aicardi J |title=Schilder's myelinoclastic diffuse sclerosis |journal=Pediatrics |volume=77 |issue=1 |pages=107–12 |year=1986 |pmid=3940347}} 7. ^S. Jarius et al. Myelinoclastic diffuse sclerosis (Schilder’s disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures, Journal of Neuroinflammation, 28 Feb. 2019, https://doi.org/10.1186/s12974-019-1425-4 8. ^{{cite journal |vauthors=Pretorius ML, Loock DB, Ravenscroft A, Schoeman JF |title=Demyelinating disease of Schilder type in three young South African children: dramatic response to corticosteroids |journal=J. Child Neurol. |volume=13 |issue=5 |pages=197–201 |year=1998 |pmid=9620009 |doi=10.1177/088307389801300501}} 9. ^{{cite journal |vauthors=de Lacour A, Guisado F, Zambrano A, Argente J, Acosta J, Ramos C |title=[Pseudotumor forms of demyelinating diseases. Report of three cases and review of the literature] |language=Spanish|journal=Revista de Neurologia |volume=27 |issue=160 |pages=966–70 |year=1998 |pmid=9951014}} 10. ^{{cite journal |vauthors=Leuzzi V, Lyon G, Cilio MR, Pedespan JM, Fontan D, Chateil JF, Vital A |title=Childhood demyelinating diseases with a prolonged remitting course and their relation to Schilder's disease: report of two cases |journal=J. Neurol. Neurosurg. Psychiatry |volume=66 |issue=3 |pages=407–8 |year=1999 |pmid=10084548 |doi=10.1136/jnnp.66.3.407 |pmc=1736247}} 11. ^{{cite journal |vauthors=Brunot E, Marcus JC |title=Multiple sclerosis presenting as a single mass lesion |journal=Pediatr. Neurol. |volume=20 |issue=5 |pages=383–6 |year=1999 |pmid=10371386 |doi=10.1016/S0887-8994(98)00164-7}} 12. ^{{cite journal |vauthors=Garell PC, Menezes AH, Baumbach G, Moore SA, Nelson G, Mathews K, Afifi AK |title=Presentation, management and follow-up of Schilder's disease |journal=Pediatric Neurosurgery |volume=29 |issue=2 |pages=86–91 |year=1998 |pmid=9792962 |doi=10.1159/000028695}} 13. ^{{WhoNamedIt|synd|1554}} 14. ^P. F. Schilder, Zur Kenntnis der sogenannten diffusen Sklerose (über Encephalitis periaxialis diffusa). Zeitschrift für die gesamte Neurologie und Psychiatrie, 1912, 10 Orig.: 1-60. 15. ^{{cite journal |vauthors=Martin JJ, Guazzi GC |title=Schilder's diffuse sclerosis |journal=Dev. Neurosci. |volume=13 |issue=4–5 |pages=267–73 |year=1991 |pmid=1817032 |doi=10.1159/000112172}} 16. ^{{cite journal |author1=Fernández O. |author2=Fernández V.E. |author3=Guerrero M. | year = 2015 | title = Demyelinating diseases of the central nervous system | url = | journal = Medicine | volume = 11 | issue = 77| pages = 4601–4609 | doi = 10.1016/j.med.2015.04.001 }} 17. ^{{cite journal | author = Poser C. M. | year = 1961 | title = Leukodystrophy and the Concept of Dysmyelination | url = | journal = Arch Neurol | volume = 4 | issue = 3| pages = 323–332 | doi = 10.1001/archneur.1961.00450090089013 }} External links{{Medical resources| DiseasesDB = 11849 | ICD10 = {{ICD10|G|37|0|g|35}} | ICD9 = {{ICD9|341.1}} | ICDO = | OMIM = 272100 | MedlinePlus = | eMedicineSubj = neuro | eMedicineTopic = 92 | MeshID = D002549 }}
2 : Autoimmune diseases|Demyelinating diseases of CNS |
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