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Substantia nigra gliosis

MedGen UID:
Concept ID:
Synonym: Gliosis in the substantia nigra
HPO: HP:0011960


Focal proliferation of glial cells in the substantia nigra. [from HPO]

Term Hierarchy

Conditions with this feature

Autosomal dominant Parkinson disease 8
MedGen UID:
Concept ID:
Disease or Syndrome
LRRK2 Parkinson disease (PD) is characterized by features consistent with idiopathic PD: initial motor features of slowly progressive asymmetric tremor at rest and/or bradykinesia, cogwheel muscle rigidity, postural instability, and gait abnormalities that may include festination and freezing. Certain nonmotor symptoms in LRRK2-PD, especially REM sleep behavior disorder and cognitive decline, may occur at similar or slightly reduced frequency compared to typical idiopathic* PD. Onset is generally after age 50, although early-onset (in the 20s) and late-onset (in the 90s) disease has been described. * Idiopathic PD refers to the presence of signs and symptoms of PD for which the etiology is currently unknown and in which there is no known family history of PD.
Dementia/parkinsonism with non-Alzheimer amyloid plaques
MedGen UID:
Concept ID:
Disease or Syndrome
Autosomal recessive juvenile Parkinson disease 2
MedGen UID:
Concept ID:
Disease or Syndrome
Parkin type of early-onset Parkinson disease (PARK-Parkin) is characterized by the cardinal signs of Parkinson disease (PD): bradykinesia, resting tremor, and rigidity. The median age at onset is 31 years (range: 3-81 years). The disease is slowly progressive: disease duration of more than 50 years has been reported. Clinical findings vary; hyperreflexia is common. Lower-limb dystonia may be a presenting sign and cognitive decline appears to be no more frequent than in the general population. Dyskinesia as a result of treatment with levodopa frequently occurs.
Parkinson disease, late-onset
MedGen UID:
Concept ID:
Disease or Syndrome
Generally, Parkinson's disease that begins after age 50 is called late-onset disease. The condition is described as early-onset disease if signs and symptoms begin before age 50. Early-onset cases that begin before age 20 are sometimes referred to as juvenile-onset Parkinson's disease.\n\nParkinson's disease can also affect emotions and thinking ability (cognition). Some affected individuals develop psychiatric conditions such as depression and visual hallucinations. People with Parkinson's disease also have an increased risk of developing dementia, which is a decline in intellectual functions including judgment and memory.\n\nOften the first symptom of Parkinson's disease is trembling or shaking (tremor) of a limb, especially when the body is at rest. Typically, the tremor begins on one side of the body, usually in one hand. Tremors can also affect the arms, legs, feet, and face. Other characteristic symptoms of Parkinson's disease include rigidity or stiffness of the limbs and torso, slow movement (bradykinesia) or an inability to move (akinesia), and impaired balance and coordination (postural instability). These symptoms worsen slowly over time.\n\nParkinson's disease is a progressive disorder of the nervous system. The disorder affects several regions of the brain, especially an area called the substantia nigra that controls balance and movement.

Professional guidelines


Davis AA, Inman CE, Wargel ZM, Dube U, Freeberg BM, Galluppi A, Haines JN, Dhavale DD, Miller R, Choudhury FA, Sullivan PM, Cruchaga C, Perlmutter JS, Ulrich JD, Benitez BA, Kotzbauer PT, Holtzman DM
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Zijlmans JC, Daniel SE, Hughes AJ, Révész T, Lees AJ
Mov Disord 2004 Jun;19(6):630-40. doi: 10.1002/mds.20083. PMID: 15197700

Recent clinical studies


Russo T, Kolisnyk B, B S A, Plessis-Belair J, Kim TW, Martin J, Ni J, Pearson JA, Park EJ, Sher RB, Studer L, Riessland M
Aging Cell 2024 Apr;23(4):e14077. Epub 2024 Feb 1 doi: 10.1111/acel.14077. PMID: 38303548Free PMC Article
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Mov Disord 2023 Mar;38(3):464-473. Epub 2023 Jan 4 doi: 10.1002/mds.29311. PMID: 36598274Free PMC Article
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Parkinsonism Relat Disord 2018 Jan;46 Suppl 1:S1-S5. Epub 2017 Aug 1 doi: 10.1016/j.parkreldis.2017.07.020. PMID: 28784297
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Clinical prediction guides

Boonstra JT, McGurran H, Temel Y, Jahanshahi A
Brain Struct Funct 2022 Sep;227(7):2231-2242. Epub 2022 Jul 19 doi: 10.1007/s00429-022-02531-9. PMID: 35854141Free PMC Article
Huin V, Coarelli G, Guemy C, Boluda S, Debs R, Mochel F, Stojkovic T, Grabli D, Maisonobe T, Gaymard B, Lenglet T, Tard C, Davion JB, Sablonnière B, Monin ML, Ewenczyk C, Viala K, Charles P, Le Ber I, Reilly MM, Houlden H, Cortese A, Seilhean D, Brice A, Durr A
Brain 2022 Jun 30;145(6):2121-2132. doi: 10.1093/brain/awab449. PMID: 34927205
Fischer NM, Hinkle JT, Perepezko K, Bakker CC, Morris M, Broen MPG, Butala A, Dawson TM, Leentjens AFG, Mari Z, Marvel CL, Mills KA, Rosenthal LS, Shepard MD, Pantelyat A, Bakker A, Pletnikova O, Troncoso JC, Wang J, Pontone GM
Am J Geriatr Psychiatry 2021 Sep;29(9):958-968. Epub 2021 Jan 15 doi: 10.1016/j.jagp.2020.12.009. PMID: 33455856Free PMC Article
O'Callaghan JP, Sriram K, Miller DB
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Armstrong DD
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Recent systematic reviews

Wong JC, Armstrong MJ, Lang AE, Hazrati LN
Mov Disord 2013 Mar;28(3):274-81. Epub 2012 Oct 31 doi: 10.1002/mds.25232. PMID: 23114877

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