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Stroke-like episode

MedGen UID:
346558
Concept ID:
C1857287
Finding
Synonym: Stroke-like episodes
 
HPO: HP:0002401

Definition

No consensus exists on what a stroke-like episode is, but these episodes can be functionally defined as a new neurological deficit, occurring with or without the context of seizures, which last longer than 24 hours. [from HPO]

Term Hierarchy

Conditions with this feature

Juvenile myopathy, encephalopathy, lactic acidosis AND stroke
MedGen UID:
56485
Concept ID:
C0162671
Disease or Syndrome
MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) is a multisystem disorder with protean manifestations. The vast majority of affected individuals develop signs and symptoms of MELAS between ages two and 40 years. Common clinical manifestations include stroke-like episodes, encephalopathy with seizures and/or dementia, muscle weakness and exercise intolerance, normal early psychomotor development, recurrent headaches, recurrent vomiting, hearing impairment, peripheral neuropathy, learning disability, and short stature. During the stroke-like episodes neuroimaging shows increased T2-weighted signal areas that do not correspond to the classic vascular distribution (hence the term "stroke-like"). Lactic acidemia is very common and muscle biopsies typically show ragged red fibers.
PMM2-congenital disorder of glycosylation
MedGen UID:
138111
Concept ID:
C0349653
Disease or Syndrome
PMM2-CDG, the most common of a group of disorders of abnormal glycosylation of N-linked oligosaccharides, is divided into three clinical stages: infantile multisystem, late-infantile and childhood ataxia–intellectual disability, and adult stable disability. The clinical manifestations and course are highly variable, ranging from infants who die in the first year of life to mildly affected adults. Clinical findings tend to be similar in sibs. In the infantile multisystem presentation, infants show axial hypotonia, hyporeflexia, esotropia, and developmental delay. Feeding problems, vomiting, faltering growth, and developmental delay are frequently seen. Subcutaneous fat may be excessive over the buttocks and suprapubic region. Two distinct clinical courses are observed: (1) a nonfatal neurologic course with faltering growth, strabismus, developmental delay, cerebellar hypoplasia, and hepatopathy in infancy followed by neuropathy and retinitis pigmentosa in the first or second decade; and (2) a more severe neurologic-multivisceral course with approximately 20% mortality in the first year of life. The late-infantile and childhood ataxia–intellectual disability stage, which begins between ages three and ten years, is characterized by hypotonia, ataxia, severely delayed language and motor development, inability to walk, and IQ of 40 to 70; other findings include seizures, stroke-like episodes or transient unilateral loss of function, coagulopathy, retinitis pigmentosa, joint contractures, and skeletal deformities. In the adult stable disability stage, intellectual ability is stable; peripheral neuropathy is variable, progressive retinitis pigmentosa and myopia are seen, thoracic and spinal deformities with osteoporosis worsen, and premature aging is observed; females may lack secondary sexual development and males may exhibit decreased testicular volume. Hypogonadotropic hypogonadism and coagulopathy may occur. The risk for deep venous thrombosis is increased.
Stormorken syndrome
MedGen UID:
350028
Concept ID:
C1861451
Disease or Syndrome
Stormorken syndrome is an autosomal dominant disorder characterized by mild bleeding tendency due to platelet dysfunction, thrombocytopenia, anemia, asplenia, tubular aggregate myopathy, congenital miosis, and ichthyosis. Additional features may include headache or recurrent stroke-like episodes (summary by Misceo et al., 2014).
Amyloidogenic transthyretin amyloidosis
MedGen UID:
414031
Concept ID:
C2751492
Disease or Syndrome
Hereditary transthyretin (ATTR) amyloidosis is characterized by a slowly progressive peripheral sensorimotor and/or autonomic neuropathy as well as non-neuropathic changes of cardiomyopathy, nephropathy, vitreous opacities, and CNS amyloidosis. The disease usually begins in the third to fifth decade in persons from endemic foci in Portugal and Japan; onset is later in persons from other areas. Typically, sensory neuropathy starts in the lower extremities with paresthesias and hypesthesias of the feet, followed within a few years by motor neuropathy. In some persons, particularly those with early-onset disease, autonomic neuropathy is the first manifestation of the condition; findings can include: orthostatic hypotension, constipation alternating with diarrhea, attacks of nausea and vomiting, delayed gastric emptying, sexual impotence, anhidrosis, and urinary retention or incontinence. Cardiac amyloidosis is mainly characterized by progressive cardiomyopathy. Individuals with leptomeningeal amyloidosis may have the following CNS findings: dementia, psychosis, visual impairment, headache, seizures, motor paresis, ataxia, myelopathy, hydrocephalus, or intracranial hemorrhage.
Moyamoya angiopathy-short stature-facial dysmorphism-hypergonadotropic hypogonadism syndrome
MedGen UID:
463207
Concept ID:
C3151857
Disease or Syndrome
This multisystem disorder is characterized by moyamoya disease, short stature, hypergonadotropic hypogonadism, and facial dysmorphism. Other variable features include dilated cardiomyopathy, premature graying of the hair, and early-onset cataracts. Moyamoya disease is a progressive cerebrovascular disorder characterized by stenosis or occlusion of the internal carotid arteries and the main branches, leading to the development of small collateral vessels (moyamoya vessels) at the base of the brain. Affected individuals can develop acute neurologic events due to stroke-like episodes (summary by Miskinyte et al., 2011). For a general phenotypic description and a discussion of genetic heterogeneity of moyamoya disease, see MYMY1 (252350).
Wolfram syndrome 1
MedGen UID:
1641635
Concept ID:
C4551693
Disease or Syndrome
WFS1 Wolfram syndrome spectrum disorder (WFS1-WSSD) is a progressive neurodegenerative disorder characterized by onset of diabetes mellitus (DM) and optic atrophy (OA) before age 16 years, and typically associated with other endocrine abnormalities, sensorineural hearing loss, and progressive neurologic abnormalities (cerebellar ataxia, peripheral neuropathy, dementia, psychiatric illness, and urinary tract atony). Although DM is mostly insulin-dependent, overall the course is milder (with lower prevalence of microvascular disease) than that seen in isolated DM. OA typically results in significantly reduced visual acuity in the first decade. Sensorineural hearing impairment ranges from congenital deafness to milder, sometimes progressive, hearing impairment.
Mitochondrial complex 1 deficiency, nuclear type 37
MedGen UID:
1783339
Concept ID:
C5543281
Disease or Syndrome
Mitochondrial DNA depletion syndrome 20 (mngie type)
MedGen UID:
1804209
Concept ID:
C5676934
Disease or Syndrome
Mitochondrial DNA depletion syndrome-20 (MTDPS20) is an autosomal recessive multisystem disorder with variable manifestations and severity. Most patients develop symptoms in childhood, although the onset can range from infancy to the teenage years. Prominent features include severe gastrointestinal dysmotility often requiring parenteral nutrition, neurogenic bladder, and muscle weakness and atrophy. Neurologic involvement manifests as headaches, stroke-like episodes, seizures, pyramidal signs, and learning difficulties or cognitive decline. Brain imaging usually shows diffuse leukoencephalopathy and may show cerebellar atrophy. The disorder results from a defect in the maintenance and repair of mitochondrial DNA, resulting in mtDNA depletion and impaired mitochondrial function (summary by Bonora et al., 2021). For a discussion of genetic heterogeneity of mtDNA depletion syndromes, see MTDPS1 (603041).

Professional guidelines

PubMed

Dumrikarnlert C, Thakolwiboon S, Senanarong V
BMC Neurol 2023 Sep 22;23(1):334. doi: 10.1186/s12883-023-03305-4. PMID: 37737161Free PMC Article
Finsterer J, Zarrouk-Mahjoub S
Seizure 2017 Aug;50:166-170. Epub 2017 Jun 24 doi: 10.1016/j.seizure.2017.06.010. PMID: 28686997
El-Hattab AW, Adesina AM, Jones J, Scaglia F
Mol Genet Metab 2015 Sep-Oct;116(1-2):4-12. Epub 2015 Jun 15 doi: 10.1016/j.ymgme.2015.06.004. PMID: 26095523

Curated

UK NICE Guideline NG128, Stroke and transient ischaemic attack in over 16s: diagnosis and initial management, 2019

Recent clinical studies

Etiology

Martinelli D, Catesini G, Greco B, Guarnera A, Parrillo C, Maines E, Longo D, Napolitano A, De Nictolis F, Cairoli S, Liccardo D, Caviglia S, Sidorina A, Olivieri G, Siri B, Bianchi R, Spagnoletti G, Dello Strologo L, Spada M, Dionisi-Vici C
J Inherit Metab Dis 2023 May;46(3):450-465. Epub 2023 Mar 15 doi: 10.1002/jimd.12599. PMID: 36861405
Ng YS, Gorman GS
Handb Clin Neurol 2023;194:65-78. doi: 10.1016/B978-0-12-821751-1.00005-1. PMID: 36813321
Zhao Y, Yu X, Ji K, Lin Y, Xu X, Wang W, Yan C
J Neurol Sci 2022 Oct 15;441:120345. Epub 2022 Jul 19 doi: 10.1016/j.jns.2022.120345. PMID: 35940028
Ng YS, Lax NZ, Blain AP, Erskine D, Baker MR, Polvikoski T, Thomas RH, Morris CM, Lai M, Whittaker RG, Gebbels A, Winder A, Hall J, Feeney C, Farrugia ME, Hirst C, Roberts M, Lawthom C, Chrysostomou A, Murphy K, Baird T, Maddison P, Duncan C, Poulton J, Nesbitt V, Hanna MG, Pitceathly RDS, Taylor RW, Blakely EL, Schaefer AM, Turnbull DM, McFarland R, Gorman GS
Brain 2022 Apr 18;145(2):542-554. doi: 10.1093/brain/awab353. PMID: 34927673Free PMC Article
Finsterer J, Stollberger C
Cerebrovasc Dis 2010;29(1):6-13. Epub 2009 Nov 5 doi: 10.1159/000255968. PMID: 19893306

Diagnosis

Zhao Y, Yu X, Ji K, Lin Y, Xu X, Wang W, Yan C
J Neurol Sci 2022 Oct 15;441:120345. Epub 2022 Jul 19 doi: 10.1016/j.jns.2022.120345. PMID: 35940028
Finsterer J
Medicina (Kaunas) 2022 May 13;58(5) doi: 10.3390/medicina58050660. PMID: 35630076Free PMC Article
Finsterer J, Aliyev R
J Neurol Sci 2020 May 15;412:116726. Epub 2020 Feb 7 doi: 10.1016/j.jns.2020.116726. PMID: 32088469
Friedman SD, Shaw DW, Ishak G, Gropman AL, Saneto RP
Dev Disabil Res Rev 2010;16(2):129-35. doi: 10.1002/ddrr.103. PMID: 20818727
Finsterer J, Stollberger C
Cerebrovasc Dis 2010;29(1):6-13. Epub 2009 Nov 5 doi: 10.1159/000255968. PMID: 19893306

Therapy

Servais A, Saitovitch A, Hummel A, Boisgontier J, Scemla A, Sberro-Soussan R, Snanoudj R, Lemaitre H, Legendre C, Pontoizeau C, Antignac C, Anglicheau D, Funalot B, Boddaert N
J Inherit Metab Dis 2020 Mar;43(2):348-356. Epub 2019 Sep 18 doi: 10.1002/jimd.12164. PMID: 31444911
Finsterer J
J Neurol Sci 2019 May 15;400:135-141. Epub 2019 Mar 23 doi: 10.1016/j.jns.2019.03.021. PMID: 30946993
Finsterer J, Zarrouk-Mahjoub S
Clin Neurol Neurosurg 2018 Mar;166:44-49. doi: 10.1016/j.clineuro.2018.01.020. PMID: 29408771
Finsterer J, Zarrouk-Mahjoub S
Seizure 2017 Aug;50:166-170. Epub 2017 Jun 24 doi: 10.1016/j.seizure.2017.06.010. PMID: 28686997
El-Hattab AW, Adesina AM, Jones J, Scaglia F
Mol Genet Metab 2015 Sep-Oct;116(1-2):4-12. Epub 2015 Jun 15 doi: 10.1016/j.ymgme.2015.06.004. PMID: 26095523

Prognosis

Ng YS, Gorman GS
Handb Clin Neurol 2023;194:65-78. doi: 10.1016/B978-0-12-821751-1.00005-1. PMID: 36813321
Lopriore P, Gomes F, Montano V, Siciliano G, Mancuso M
Int J Mol Sci 2022 Oct 30;23(21) doi: 10.3390/ijms232113216. PMID: 36362003Free PMC Article
Ng YS, Lax NZ, Blain AP, Erskine D, Baker MR, Polvikoski T, Thomas RH, Morris CM, Lai M, Whittaker RG, Gebbels A, Winder A, Hall J, Feeney C, Farrugia ME, Hirst C, Roberts M, Lawthom C, Chrysostomou A, Murphy K, Baird T, Maddison P, Duncan C, Poulton J, Nesbitt V, Hanna MG, Pitceathly RDS, Taylor RW, Blakely EL, Schaefer AM, Turnbull DM, McFarland R, Gorman GS
Brain 2022 Apr 18;145(2):542-554. doi: 10.1093/brain/awab353. PMID: 34927673Free PMC Article
Finsterer J, Aliyev R
J Neurol Sci 2020 May 15;412:116726. Epub 2020 Feb 7 doi: 10.1016/j.jns.2020.116726. PMID: 32088469
Finsterer J, Stollberger C
Cerebrovasc Dis 2010;29(1):6-13. Epub 2009 Nov 5 doi: 10.1159/000255968. PMID: 19893306

Clinical prediction guides

Martinelli D, Catesini G, Greco B, Guarnera A, Parrillo C, Maines E, Longo D, Napolitano A, De Nictolis F, Cairoli S, Liccardo D, Caviglia S, Sidorina A, Olivieri G, Siri B, Bianchi R, Spagnoletti G, Dello Strologo L, Spada M, Dionisi-Vici C
J Inherit Metab Dis 2023 May;46(3):450-465. Epub 2023 Mar 15 doi: 10.1002/jimd.12599. PMID: 36861405
Ng YS, Gorman GS
Handb Clin Neurol 2023;194:65-78. doi: 10.1016/B978-0-12-821751-1.00005-1. PMID: 36813321
Zhao Y, Yu X, Ji K, Lin Y, Xu X, Wang W, Yan C
J Neurol Sci 2022 Oct 15;441:120345. Epub 2022 Jul 19 doi: 10.1016/j.jns.2022.120345. PMID: 35940028
Ng YS, Lax NZ, Blain AP, Erskine D, Baker MR, Polvikoski T, Thomas RH, Morris CM, Lai M, Whittaker RG, Gebbels A, Winder A, Hall J, Feeney C, Farrugia ME, Hirst C, Roberts M, Lawthom C, Chrysostomou A, Murphy K, Baird T, Maddison P, Duncan C, Poulton J, Nesbitt V, Hanna MG, Pitceathly RDS, Taylor RW, Blakely EL, Schaefer AM, Turnbull DM, McFarland R, Gorman GS
Brain 2022 Apr 18;145(2):542-554. doi: 10.1093/brain/awab353. PMID: 34927673Free PMC Article
El-Hattab AW, Adesina AM, Jones J, Scaglia F
Mol Genet Metab 2015 Sep-Oct;116(1-2):4-12. Epub 2015 Jun 15 doi: 10.1016/j.ymgme.2015.06.004. PMID: 26095523

Recent systematic reviews

Gramegna LL, Cortesi I, Mitolo M, Evangelisti S, Talozzi L, Cirillo L, Tonon C, Lodi R
J Neuroradiol 2021 Sep;48(5):359-366. Epub 2021 Feb 15 doi: 10.1016/j.neurad.2021.02.002. PMID: 33596430
Finsterer J, Aliyev R
J Neurol Sci 2020 May 15;412:116726. Epub 2020 Feb 7 doi: 10.1016/j.jns.2020.116726. PMID: 32088469

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2019
      UK NICE Guideline NG128, Stroke and transient ischaemic attack in over 16s: diagnosis and initial management, 2019

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