Case Presentation  Kamlesh JobanputraP.D.C.C(Neuroradiology)
CLINICAL NOTES•   1 YR/M OPD•   CEREBRAL PALSY•   MENTAL RETARDATION•   TONIC SEIZURES.
T1 W IMAGES
T2 W IMAGES
DISCUSSION• INITIAL IMPRESSION• FIGURE OF 8 APPEARANCE• ?COLPOCEPHALY• LISSENCEPHALY
REVIEW OF LITERATURE• TYPES OF LISSENCEPHALY• Classic (Type I) Lissencephaly (4-Layer Lissencephaly)• Patients with classi...
LISSENCEPHALY 1
LISSENCEPHALY TYPE II• Cobblestone lissencephaly is characterized by a nodular  brain surface, ocular anomalies, and conge...
LISSENCEPHALY II
NEWER CLASSIFICATION
WHY NOT LISSENCEPHALY• This detailed review of a large number of MR images of  patients with various types of lissencephal...
CAUSES OF MEGA CORPUS CALLOSUM• A mega-corpus callosum is an exceptional  finding in the neurologic setting, with only a  ...
POLYMICROGYRIA• PMG is characterized by many small prominent convolutions  separated by shallow sulci with an irregular ap...
POLYMICROGYRIA CLASSIFICATION• Pattern I is characterized by a small fine undulating  cortex with normal cortical thicknes...
HOW DIFFERENT IS THIS CASE• Poly microgyria• Enlarged Corpus callosum• Psychomotor retardation.
PMMC• A constellation of findings such as megalencephaly, a mega-  corpus callosum, a complete lack of motor  development,...
PMMC• Megalencephaly may or may not be  associated.• Mega corpus callosum, polymicrogyria, epilepsy and psychomotor  retar...
EXTRA FINDINGS• ENLARGED COMMISURES (AC, CC, PC)• REQUIRES FURTHER RESEARCH.
THANK YOU
Neurorad 2
Neurorad 2
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Neurorad 2

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Neurorad 2

  1. 1. Case Presentation Kamlesh JobanputraP.D.C.C(Neuroradiology)
  2. 2. CLINICAL NOTES• 1 YR/M OPD• CEREBRAL PALSY• MENTAL RETARDATION• TONIC SEIZURES.
  3. 3. T1 W IMAGES
  4. 4. T2 W IMAGES
  5. 5. DISCUSSION• INITIAL IMPRESSION• FIGURE OF 8 APPEARANCE• ?COLPOCEPHALY• LISSENCEPHALY
  6. 6. REVIEW OF LITERATURE• TYPES OF LISSENCEPHALY• Classic (Type I) Lissencephaly (4-Layer Lissencephaly)• Patients with classic lissencephaly may have a smooth brain surface in the complete form, or more commonly, they have a smooth surface with some gyral formation along the inferior frontal and temporal lobes in the incomplete form.• This anomaly results from arrest of the migration process. Patients The malformation is characterized by complete agyria in the complete form or parieto-occipital agyria with frontotemporal pachygyria in the incomplete form. The cortex is thick because it encompasses radial columns of the arrested cells.• The subcortical white matter is thin, with a lack of the normal gray-white matter interdigitation.• There is a circumferential band of high signal intensity on T2-weighted images, most prominent in the parieto-occipital cortex, corresponding to a sparse cell zone with increased water content.
  7. 7. LISSENCEPHALY 1
  8. 8. LISSENCEPHALY TYPE II• Cobblestone lissencephaly is characterized by a nodular brain surface, ocular anomalies, and congenital muscular disorders.• Cobblestone cortex results from overmigration of the neuroblasts and glial cells beyond the external glial limitations into the subarachnoid space .• It includes a spectrum of anomalies with Walker-Warburg syndrome (WWS) being the most severe form; Fukuyama congenital muscular dystrophy (FCMD), the mildest form; and muscle-eye-brain (MEB) disease, the intermediate form
  9. 9. LISSENCEPHALY II
  10. 10. NEWER CLASSIFICATION
  11. 11. WHY NOT LISSENCEPHALY• This detailed review of a large number of MR images of patients with various types of lissencephaly showed developmental abnormalities of the major forebrain commissures in almost all patients;• The CC was the commissure most commonly involved.• Although many variations were seen, the most common anomaly consisted of an absent rostrum, a small inferior genu, and a small or abnormally shaped splenium: this configuration has been defined as hypogenetic.• In addition, malformations secondary to specific mutations and presumed mutations seemed to have a characteristic overall shape of the CC.• Kara AJNR 31 Oct 2010 www.ajnr.org
  12. 12. CAUSES OF MEGA CORPUS CALLOSUM• A mega-corpus callosum is an exceptional finding in the neurologic setting, with only a few reports in the literature.• In most cases, the abnormal development of the corpus callosum results in complete or partial agenesis and thinning.• Two genetic syndromes merit specific mention: 1. neurofibromatosis type1 and 2.Cohen syndrome
  13. 13. POLYMICROGYRIA• PMG is characterized by many small prominent convolutions separated by shallow sulci with an irregular appearance of the cortical surface and cortical white matter.• It results from prenatal infection, ischemia, or exposure to toxins or chromosomal abnormality.• The malformation is characterized by a heterogeneous collection of neurons and derangement of the normal 6-layer lamination of the cortex. The sulci may be obliterated and bridged by fusion of their superficial cellular layers, particularly the molecular one.• The anomaly can be unilateral or bilateral, small or large, symmetric or asymmetric.• It is commonly located in regions adjacent to the Sylvian fissures (ie, the posterior frontal lobe, the superior temporal lobe, and the inferior parietal lobe).
  14. 14. POLYMICROGYRIA CLASSIFICATION• Pattern I is characterized by a small fine undulating cortex with normal cortical thickness (3–4 mm). This pattern is seen in the anterior frontal region, mainly in infants younger than 12 months.• Pattern II has a bumpy cortical appearance with less undulation. The cortex is abnormally thickened (5–7 mm) with an irregular cortical–white matter junction. This pattern is noted in infants older than 18 months. It involves mainly the frontal, parietal, and perisylvian regions. Differentiation between the cortex and underlying white matter is poor on T1- weighted images.
  15. 15. HOW DIFFERENT IS THIS CASE• Poly microgyria• Enlarged Corpus callosum• Psychomotor retardation.
  16. 16. PMMC• A constellation of findings such as megalencephaly, a mega- corpus callosum, a complete lack of motor development, perisylvian polymicrogyria, and distinct facies has been designated megalencephalypolymicrogyria- mega-corpus callosum syndrome (Online Mendelian Inheritance in Man 603387)• Bindu PS, Taly AB, Sinha S, Bharath RD. Mega-corpus callosum, polymicrogyria, and psychomotor retardation syndrome. Pediatr Neurol 2010;42:129-132.• Mega-Corpus Callosum, Polymicrogyria, and Psychomotor Retardation: Confirmation of a Syndromic EntityT. M. Pierson1, R. A. Zimmerman2, G. I. Tennekoon3, C. G. Bönnemann4
  17. 17. PMMC• Megalencephaly may or may not be associated.• Mega corpus callosum, polymicrogyria, epilepsy and psychomotor retardationGyurgyinka Gergev, Nóra Szabó, Éva Romhányi, Sándor Túri, László Sztriha European Paediatric Neurology Society Congress, 9th EPNS Congress
  18. 18. EXTRA FINDINGS• ENLARGED COMMISURES (AC, CC, PC)• REQUIRES FURTHER RESEARCH.
  19. 19. THANK YOU

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