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Marginal Glioneuronal Heterotopia

Presentation

Cystic components may be present. Best imaging clues: Wedge- shaped cortically based mass with bubbly appearence exceeding normal cortical thickness. Lack of surrounding edema, no contrast enhancement. b. [epos.myesr.org]

Epilepsy is very common and typically presents in childhood 3. It is usually associated with variable developmental delay 3. [radiopaedia.org]

A breach of the neuropial border seems to be the most acceptable pathomechanism for our presented cases. [pubmed.ncbi.nlm.nih.gov]

We have presented 32 patients who underwent cortical recectiom for intractable seizures and demonstrated the neuropathologic features, which could be explained by a disturbance in the process of neural development in the farm. [jkna.org]

Heterotopic neurons were present in the molecular layer as well as in the subcortical white matter. [bioline.org.br]

Respiratoric

  • Cheyne-Stokes Respiration

    […] vestibulospinal tract, reticulospinal tract, tectospinal tract motor cortex- coticobulbar fiber(cranial motor n. nu.), corticopinal fiber consciousness(의식)- arousal(각성)+awareness(인식) arousal-alert, drowsy, stuporous, comatose vestibulo-ocular reflex-COWS 호흡-Cheyne-Stokes [m.blog.naver.com]

Gastrointestinal

  • Regurgitation

    Arch Pathol 78:533–539 Google Scholar Shuangshoti S, Yenrudi S, Netsky MG (1981) Heterotopias of brain as a cause of infantile regurgitation. [link.springer.com]

  • Chronic Diarrhea

    diarrhea, and ambiguous genitalia with micropenis and cryptorchidism. [karger.com]

Musculoskeletal

  • Muscle Weakness

    ABBREVIATIONS: DIR double inversion recovery FCMD Fukuyama congenital muscular dystrophy Patients with Fukuyama congenital muscular dystrophy (FCMD) have generalized muscle weakness, hypotonia, and developmental delay beginning in early infancy. [ajnr.org]

  • Joint Stiffness

    stiffness [Rivière et al., 2012a; Di Donato et al., 2014; Verloes et al., 2015]. [karger.com]

Skin

  • Angiomatosis

    A study of 13 cases including combinations with medulloblastoma, colloid and epidermoid cysts, angiomatosis and other malformations. [link.springer.com]

Urogenital

  • Cryptorchidism

    […] cortical thickness (only 6-7 mm), atrophic striatal and thalamic nuclei, postnatal microcephaly, neonatal-onset epilepsy, hypothalamic dysfunction including deficient temperature regulation, chronic diarrhea, and ambiguous genitalia with micropenis and cryptorchidism [karger.com]

Neurologic

  • Spastic Quadriplegia

    Patients with severe LIS have early developmental delay, early diffuse hypotonia, later spastic quadriplegia, and eventual severe or profound mental retardation. [karger.com]

  • Febrile Seizures

    Only one female patient (# 2 – Table 1) within this group had had a son who was affected by febrile seizures. [scielo.org.ar]

Workup

EEG

  • Focal Epileptiform Discharges

    Interictal EEGs showed focal epileptiform discharges in 7 cases (53.8%). [scielo.org.ar]

Treatment

Response to pharmacological treatment Patients were considered refractory to treatment when seizure control was not achieved despite of having been medicated with one of the traditional drugs included within the first-line group of antiepileptic drugs [scielo.org.ar]

• ANATOMY • DEFINITION • TYPES –Defn, C/F, MRI • TREATMENT 4. DEVELOPMENT OF CEREBRAL CORTEX • It’s a complex and tightly regulated process and divided into 1. Neural cell proliferation and differentiation 2. Neuronal migration 3. [slideshare.net]

Rapamycin has anti-epileptic action, and the rapamycin analog everolimus has been used successfully for the treatment of SEGA. Further Reading Barkovich AJ, Kuzniecki RI Jackson GD, Guerrini R, and Dobyns WB. [neuropathology-web.org]

In : The treatment of epilepsy. Principles and Practice, Elaine Wyllie E (ed.), Lea and Febiger, O, Philadelphia. 1993; 41-54. Yasha TC, Santosh V, Das S et al : Hemimegalencephaly - morphological and immunocytochemical study. [bioline.org.br]

In : The treatment of epilepsy. Principles and Practice, Elaine Wyllie E (ed.), Lea and Febiger, O, Philadelphia. 1993; 41-54. 8. Yasha TC, Santosh V, Das S et al : Hemimegalencephaly - morphological and immunocytochemical study. [neurologyindia.com]

Prognosis

With time, it became obvious that it was a heterogeneous entity, with distinct clinical and imaging characteristics, and different surgical responses, the surgical indications and the prognosis being closely related with the pathologic subtype, so that [radiologykey.com]

Etiology

The child with a cephalocele: etiology, neuroimaging, and outcome.Childs Nervous System. 1996; 12: 540-550 Volpe J.J. Neurology of the Newborn. WB Saunders, Philadelphia2000 Sarnat H.B. deMello D.E. Blair J.D. Siddiqui S.Y. [ejpn-journal.com]

Focal cortical dysplasia or neuronal migration disorder could be due to prenatal vascular etiology.11 Association of CD with DNT has been well documented in literature. [bioline.org.br]

• Its having heterogeneous etiology - Genetic - CMV virus infection or Ischemia( MCA – In perisylvian PMG) 26. [slideshare.net]

The etiology and pathogenesis of PMG are diverse. Some forms of PMG are acquired and are caused by prenatal hypoxic-ischemic insults. In such cases, layer five is damaged and layers superficial to it overfold and fuse. [neuropathology-web.org]

Focal cortical dysplasia or neuronal migration disorder could be due to prenatal vascular etiology. [11] Association of CD with DNT has been well documented in literature. [neurologyindia.com]

Epidemiology

55-64 [Pubmed] 7 Epidemiology of hemimegalencephaly: A case series and review Tinkle, B.T. and Schorry, E.K. and Franz, D.N. and Crone, K.R. and Saal, H.M. American Journal of Medical Genetics. 2005; 139 A(3): 204-211 [Pubmed] [neurologyindia.com]

Prevention

The ectopic gray matter results from an insult that occurred during the late neuronal proliferating period, which allows the multiplication of neuroblasts but prevents their normal migration from the paraventricular region. [scielo.org.ar]

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