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EEG with generalized slow activity

MedGen UID:
866862
Concept ID:
C4021217
Finding
Synonyms: EEG with generalised slow activity; EEG: generalised slow activity; EEG: generalized slow activity
 
HPO: HP:0010845

Definition

Diffuse slowing of cerebral electrical activity recorded along the scalp by electroencephalography (EEG). [from HPO]

Conditions with this feature

Cholestanol storage disease
MedGen UID:
116041
Concept ID:
C0238052
Disease or Syndrome
Cerebrotendinous xanthomatosis (CTX) is a lipid storage disease characterized by infantile-onset diarrhea, childhood-onset cataract, adolescent- to young adult-onset tendon xanthomas, and adult-onset progressive neurologic dysfunction (dementia, psychiatric disturbances, pyramidal and/or cerebellar signs, dystonia, atypical parkinsonism, peripheral neuropathy, and seizures). Chronic diarrhea from infancy and/or neonatal cholestasis may be the earliest clinical manifestation. In approximately 75% of affected individuals, cataracts are the first finding, often appearing in the first decade of life. Xanthomas appear in the second or third decade; they occur on the Achilles tendon, the extensor tendons of the elbow and hand, the patellar tendon, and the neck tendons. Xanthomas have been reported in the lung, bones, and central nervous system. Some individuals show cognitive impairment from early infancy, whereas the majority have normal or only slightly impaired intellectual function until puberty; dementia with slow deterioration in intellectual abilities occurs in the third decade in more than 50% of individuals. Neuropsychiatric symptoms such as behavioral changes, hallucinations, agitation, aggression, depression, and suicide attempts may be prominent. Pyramidal signs (i.e., spasticity) and/or cerebellar signs almost invariably become evident between ages 20 and 30 years. The biochemical abnormalities that distinguish CTX from other conditions with xanthomas include high plasma and tissue cholestanol concentration, normal-to-low plasma cholesterol concentration, decreased chenodeoxycholic acid (CDCA), increased concentration of bile alcohols and their glyconjugates, and increased concentrations of cholestanol and apolipoprotein B in cerebrospinal fluid.
X-linked intellectual disability-psychosis-macroorchidism syndrome
MedGen UID:
163232
Concept ID:
C0796222
Disease or Syndrome
The spectrum of MECP2-related phenotypes in females ranges from classic Rett syndrome to variant Rett syndrome with a broader clinical phenotype (either milder or more severe than classic Rett syndrome) to mild learning disabilities; the spectrum in males ranges from severe neonatal encephalopathy to pyramidal signs, parkinsonism, and macroorchidism (PPM-X) syndrome to severe syndromic/nonsyndromic intellectual disability. Females: Classic Rett syndrome, a progressive neurodevelopmental disorder primarily affecting girls, is characterized by apparently normal psychomotor development during the first six to 18 months of life, followed by a short period of developmental stagnation, then rapid regression in language and motor skills, followed by long-term stability. During the phase of rapid regression, repetitive, stereotypic hand movements replace purposeful hand use. Additional findings include fits of screaming and inconsolable crying, autistic features, panic-like attacks, bruxism, episodic apnea and/or hyperpnea, gait ataxia and apraxia, tremors, seizures, and acquired microcephaly. Males: Severe neonatal-onset encephalopathy, the most common phenotype in affected males, is characterized by a relentless clinical course that follows a metabolic-degenerative type of pattern, abnormal tone, involuntary movements, severe seizures, and breathing abnormalities. Death often occurs before age two years.
Neurodevelopmental disorder with or without seizures and gait abnormalities
MedGen UID:
1645968
Concept ID:
C4693391
Disease or Syndrome
Neurodevelopmental disorder with or without seizures and gait abnormalities (NEDSGA) is an autosomal dominant disorder characterized by global developmental delay apparent from infancy or early childhood, resulting in variably impaired intellectual development that can range from profound with absent speech to mild with an ability to attend special schools. Most affected individuals show irritability, stiffness, and hypertonia early in life, which progresses to spasticity and impaired gait later. Some patients may develop seizures of variable severity early in life (summary by Martin et al., 2017).
Developmental and epileptic encephalopathy, 2
MedGen UID:
1663579
Concept ID:
C4750718
Disease or Syndrome
Developmental and epileptic encephalopathy-2 (DEE2) is an X-linked dominant severe neurologic disorder characterized by onset of seizures in the first months of life and severe global developmental delay resulting in impaired intellectual development and poor motor control. Other features include lack of speech development, subtle dysmorphic facial features, sleep disturbances, gastrointestinal problems, and stereotypic hand movements. There is some phenotypic overlap with Rett syndrome (312750), but DEE2 is considered to be a distinct entity (summary by Fehr et al., 2013). For a discussion of genetic heterogeneity of DEE, see 308350.
Neurodevelopmental disorder with microcephaly, epilepsy, and hypomyelination
MedGen UID:
1684142
Concept ID:
C5193057
Disease or Syndrome
A rare, genetic, neurometabolic disease characterized by microcephaly, short stature, epilepsy, cerebral hypomyelination, severe global developmental delay, and progressive spasticity. Macrocytic anemia and hyperthermia have also been reported in association. Brain imaging reveals delayed myelination with minimal progression over time, mild cerebellar atrophy and/or thin corpus callosum.
Developmental and epileptic encephalopathy 105 with hypopituitarism
MedGen UID:
1823963
Concept ID:
C5774190
Disease or Syndrome
Developmental and epileptic encephalopathy-105 with hypopituitarism (DEE105) is an autosomal recessive disorder characterized by the onset of seizures and pituitary insufficiency in the first weeks or months of life. Affected individuals have profoundly impaired development with almost no acquisition of skills. They are hypotonic, unable to sit or speak, and have poor or absent visual fixation. Endocrine workup shows central pituitary dysfunction with low hormone levels. Brain imaging shows cerebral atrophy, thin corpus callosum, and small pituitary gland (Schanzer et al., 2021). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350.

Professional guidelines

PubMed

Lagarde S, Boucraut J, Bartolomei F
Rev Neurol (Paris) 2022 Sep;178(7):675-691. Epub 2022 Feb 4 doi: 10.1016/j.neurol.2022.01.007. PMID: 35131107
Kondziella D, Bender A, Diserens K, van Erp W, Estraneo A, Formisano R, Laureys S, Naccache L, Ozturk S, Rohaut B, Sitt JD, Stender J, Tiainen M, Rossetti AO, Gosseries O, Chatelle C; EAN Panel on Coma, Disorders of Consciousness
Eur J Neurol 2020 May;27(5):741-756. Epub 2020 Feb 23 doi: 10.1111/ene.14151. PMID: 32090418
Asztely F, Kumlien E
Acta Neurol Scand 2012 Dec;126(6):365-75. Epub 2012 Jun 19 doi: 10.1111/j.1600-0404.2012.01691.x. PMID: 22713136

Recent clinical studies

Etiology

Koch G, Casula EP, Bonnì S, Borghi I, Assogna M, Minei M, Pellicciari MC, Motta C, D'Acunto A, Porrazzini F, Maiella M, Ferrari C, Caltagirone C, Santarnecchi E, Bozzali M, Martorana A
Brain 2022 Nov 21;145(11):3776-3786. doi: 10.1093/brain/awac285. PMID: 36281767Free PMC Article
Ujma PP, Szalárdy O, Fabó D, Erőss L, Bódizs R
Neuroimage 2022 Aug 15;257:119325. Epub 2022 May 20 doi: 10.1016/j.neuroimage.2022.119325. PMID: 35605767
Lucey BP, Wisch J, Boerwinkle AH, Landsness EC, Toedebusch CD, McLeland JS, Butt OH, Hassenstab J, Morris JC, Ances BM, Holtzman DM
Brain 2021 Oct 22;144(9):2852-2862. doi: 10.1093/brain/awab272. PMID: 34668959Free PMC Article
Baud MO, Proix T, Rao VR, Schindler K
Curr Opin Neurol 2020 Apr;33(2):163-172. doi: 10.1097/WCO.0000000000000798. PMID: 32049738
Asadi-Pooya AA
Neurol Sci 2018 Mar;39(3):403-414. Epub 2017 Nov 9 doi: 10.1007/s10072-017-3188-y. PMID: 29124439

Diagnosis

Lagarde S, Boucraut J, Bartolomei F
Rev Neurol (Paris) 2022 Sep;178(7):675-691. Epub 2022 Feb 4 doi: 10.1016/j.neurol.2022.01.007. PMID: 35131107
Ferrarelli F
Am J Psychiatry 2021 Oct 1;178(10):903-913. Epub 2021 Mar 17 doi: 10.1176/appi.ajp.2020.20070968. PMID: 33726524Free PMC Article
Kondziella D, Bender A, Diserens K, van Erp W, Estraneo A, Formisano R, Laureys S, Naccache L, Ozturk S, Rohaut B, Sitt JD, Stender J, Tiainen M, Rossetti AO, Gosseries O, Chatelle C; EAN Panel on Coma, Disorders of Consciousness
Eur J Neurol 2020 May;27(5):741-756. Epub 2020 Feb 23 doi: 10.1111/ene.14151. PMID: 32090418
Asadi-Pooya AA
Neurol Sci 2018 Mar;39(3):403-414. Epub 2017 Nov 9 doi: 10.1007/s10072-017-3188-y. PMID: 29124439
Clark I, Landolt HP
Sleep Med Rev 2017 Feb;31:70-78. Epub 2016 Jan 30 doi: 10.1016/j.smrv.2016.01.006. PMID: 26899133

Therapy

Liu T, Zhang X, Li A, Liu T, Yang X, Zhang H, Lei Y, Yang Q, Dong H
BMC Anesthesiol 2023 Jun 23;23(1):221. doi: 10.1186/s12871-023-02170-5. PMID: 37353750Free PMC Article
Koch G, Casula EP, Bonnì S, Borghi I, Assogna M, Minei M, Pellicciari MC, Motta C, D'Acunto A, Porrazzini F, Maiella M, Ferrari C, Caltagirone C, Santarnecchi E, Bozzali M, Martorana A
Brain 2022 Nov 21;145(11):3776-3786. doi: 10.1093/brain/awac285. PMID: 36281767Free PMC Article
Baud MO, Proix T, Rao VR, Schindler K
Curr Opin Neurol 2020 Apr;33(2):163-172. doi: 10.1097/WCO.0000000000000798. PMID: 32049738
Clark I, Landolt HP
Sleep Med Rev 2017 Feb;31:70-78. Epub 2016 Jan 30 doi: 10.1016/j.smrv.2016.01.006. PMID: 26899133
Dulac O
Epilepsia 2001;42 Suppl 3:23-6. doi: 10.1046/j.1528-1157.2001.042suppl.3023.x. PMID: 11520318

Prognosis

Brown CW, Chen HY, Panegyres PK
BMC Neurol 2023 May 23;23(1):202. doi: 10.1186/s12883-023-03248-w. PMID: 37221470Free PMC Article
Koch G, Casula EP, Bonnì S, Borghi I, Assogna M, Minei M, Pellicciari MC, Motta C, D'Acunto A, Porrazzini F, Maiella M, Ferrari C, Caltagirone C, Santarnecchi E, Bozzali M, Martorana A
Brain 2022 Nov 21;145(11):3776-3786. doi: 10.1093/brain/awac285. PMID: 36281767Free PMC Article
Lucey BP, Wisch J, Boerwinkle AH, Landsness EC, Toedebusch CD, McLeland JS, Butt OH, Hassenstab J, Morris JC, Ances BM, Holtzman DM
Brain 2021 Oct 22;144(9):2852-2862. doi: 10.1093/brain/awab272. PMID: 34668959Free PMC Article
Ferrarelli F
Am J Psychiatry 2021 Oct 1;178(10):903-913. Epub 2021 Mar 17 doi: 10.1176/appi.ajp.2020.20070968. PMID: 33726524Free PMC Article
Baud MO, Proix T, Rao VR, Schindler K
Curr Opin Neurol 2020 Apr;33(2):163-172. doi: 10.1097/WCO.0000000000000798. PMID: 32049738

Clinical prediction guides

Koch G, Casula EP, Bonnì S, Borghi I, Assogna M, Minei M, Pellicciari MC, Motta C, D'Acunto A, Porrazzini F, Maiella M, Ferrari C, Caltagirone C, Santarnecchi E, Bozzali M, Martorana A
Brain 2022 Nov 21;145(11):3776-3786. doi: 10.1093/brain/awac285. PMID: 36281767Free PMC Article
Lucey BP, Wisch J, Boerwinkle AH, Landsness EC, Toedebusch CD, McLeland JS, Butt OH, Hassenstab J, Morris JC, Ances BM, Holtzman DM
Brain 2021 Oct 22;144(9):2852-2862. doi: 10.1093/brain/awab272. PMID: 34668959Free PMC Article
Ferrarelli F
Am J Psychiatry 2021 Oct 1;178(10):903-913. Epub 2021 Mar 17 doi: 10.1176/appi.ajp.2020.20070968. PMID: 33726524Free PMC Article
Frohlich J, Toker D, Monti MM
Brain 2021 Sep 4;144(8):2257-2277. doi: 10.1093/brain/awab095. PMID: 33693596
Kondziella D, Bender A, Diserens K, van Erp W, Estraneo A, Formisano R, Laureys S, Naccache L, Ozturk S, Rohaut B, Sitt JD, Stender J, Tiainen M, Rossetti AO, Gosseries O, Chatelle C; EAN Panel on Coma, Disorders of Consciousness
Eur J Neurol 2020 May;27(5):741-756. Epub 2020 Feb 23 doi: 10.1111/ene.14151. PMID: 32090418

Recent systematic reviews

Rossi S, Romoli M, Urbinati G, Benini M, Russo M, D'Anna L, Abu-Rumeileh S, Sacco S, Querzani P, Foschi M
Neurol Sci 2022 Aug;43(8):4671-4683. Epub 2022 Apr 28 doi: 10.1007/s10072-022-06088-7. PMID: 35482160Free PMC Article
Lagarde S, Boucraut J, Bartolomei F
Rev Neurol (Paris) 2022 Sep;178(7):675-691. Epub 2022 Feb 4 doi: 10.1016/j.neurol.2022.01.007. PMID: 35131107
Roberto KT, Espiritu AI, Fernandez MLL, Gutierrez JC
Epilepsy Behav 2020 Nov;112:107462. Epub 2020 Sep 22 doi: 10.1016/j.yebeh.2020.107462. PMID: 32971385
Clark I, Landolt HP
Sleep Med Rev 2017 Feb;31:70-78. Epub 2016 Jan 30 doi: 10.1016/j.smrv.2016.01.006. PMID: 26899133
Galderisi S, Mucci A, Volpe U, Boutros N
Clin EEG Neurosci 2009 Apr;40(2):62-77. doi: 10.1177/155005940904000206. PMID: 19534300

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