dyke david off mason syndrome
TRANSCRIPT
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Case presentation
Dr Nani LampungJR-1 Dept of RadiodiagnosisJNMCH AMU, Aligarh
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Case
A 12 year old boy presented with weakness in right upper limb ,abnormal movements and seizures since early childhood. he had learning difficulties, cognitive impairment and global developmental delay. the boy presented with normal vision and hearing, no facial asymmetry was appreciated. No h/o of fever, cough, headache, neck rigidity
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there was no clear history of apparent perinatal and antenatal complications SYSTEMIC EXAMINATIONS- WNL
Blood investigations-normalchest x ray normalCECT Head advised
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CECT FINDINGSShows prominent cortical sulci and gyri overlying convexity of left cerebral hemispheres with evidence of volume loss with prominent ipsilateral lateral ventricle & pulling of lateral ventricles to ipsilateral side s/o ? hemicerebral atrophyB/L cerebellar hemispheres & brainstem appear normalB/L basal ganglia & thalami appear normalSellar & suprasellar regions appear normalBONE WINDOW : shows mild calvarial thickening of left side
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DIFFERENTIAL DIAGNOSIS
1. DDMS2. RASMUSSEN ENCEPHALITIS3. STURGE WEBER SYNDROME4. HEMIMEGAENCEPHALY
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DykE-DAvIDOFF-mASON SyNDROmE(DDmS)
Cerebral hemiatrophy
characterised by :Thickening of skull vault(compensatory)Enlargement of PNSElevation of petrous ridgeIpsilateral falcine displacementCapillary malformations
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ETIOLOGy
Underlying etiology- cerebral infarctPrenatal- congenital anomalies, vascular malformation, infectionsNatal- birth trauma, hypoxia, I/C bleedPost natal- secondary to cerebral trauma, tx, infections, febrile seizures
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CLINICAL PRESENTATION
Seizures Facial assymmetry Contralateral hemiparesis Mental retardation
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ImAGING FINDINGS
General features :The affected hemisphere demonstrates diffuse volume loss with encephalomalacia and gliosisLeft sided hemiatrophy-more common (70%)
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CT FINDINGS
NECT :atrophic hemisphere with enlarged sulci and dilatation of the I/L ventricleSuperior sagittal sinus & interhemispheric fissure are often displaced across the midlineBONE WINDOW :variable degrees of calvarial thickening,elevation of the sphenoid wing & petrous temporal bone with expanded sinuses & mastoids
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mRI FINDINGS
T1WI shows hemispheric volume loss with prominent sulci and cisterns
T2/FLAIR- demonstrate encephalomalacia with shrunken hyperintense gyri & subcortical WM I/L cerebral peduncle is usually smallAtrophy of the C/L cerebellum is common secondary to crossed cerebellar diaschisisDDMS neither enhances on T1 C+ nor demonstrates restricted diffusion
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Figure 1 :(A) Axial T2-weighted image: Left cerebral hemiatrophy, ipsilateral occipital horn dilatation, ipsilateral midline shift, hypoplasia of thalamus, caudate nucleus, and lentiform nucleus are demonstrated. In addition,ipsilateral pneumosinus dilatans (frontal) is seenFigure 1B: Axial T2-weighted image: Hypoplasia of left cerebral peduncleFigure 1C: Axial T1-weighted image: Left cerebral hemiatrophy with calvarial thickening. Hypointensity in white matter represents gliosis
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POINTS
AgeC/F- seizure, hemiparesisImaging :HemiatrophyProminent ventriclesThickened calvaria
no involvement of pns No displacement of
SSS & IHF
IN FAVOUR AGAINST
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RASmUSSEN ENCEPHALITIS‘chronic focal encephalitis’Rare progressive encephalitis characterised by drug-resistant epilepsy, progressive hemiparesis & mental impairmentInvolvement –focal encephalomalacia typically in the medial temporal lobe & around the sylvian fissure
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ETIOLOGY
Exact unknown Chronic inflammation of brain with T-cell infiltration of the
tissue (mostly only one cerebral hemisphere affected ) Permanent damage to the cells leading to atrophy Possible etiologies- viral infections/autoimmune disease
involving glutamate receptors with glutamine toxicity Biopsy findings are nonspecific,with leptomeningeal &
perivascular lymphocytic infiltrates, microglial nodules, neuronal loss and gliosis
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CLInICaL fEaTurEs
Clinically normal until seizures begin14 mnths to 14 yrs ( peak-3-6 yrs)Two main stages- 1. Acute stage 2.Chronic stage
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Acute stage- (4 to 8 months) Inflammation is active and the symptoms become progressively worse Seizures – focal motor seizures( epilepsia partialis continua)hemiparesis, hemianopia & cognitive difficulties( learning memory or language)Neurological deficits are progressive, and the seizure become often medically refractory
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Chronic or residual stageinflammation is no longer activeEpilepsy , paralysis or cognitive problems
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TrEaTMEnTAcute stage- Aim is to reduce the inflammationRx- steroidsIV IG –effective in both short & long term
Chronic/residual stage Aim -improving the remaining symptomsAntiepilepticsRefractory cases- hemispherectomy
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IMaGInG fIndInGs
Initially normalMRI- T2/FLAIR hyperintensity develops in cortex & subcortical white matter with evidence of volume loss of the affected hemisphere basal ganglia atrophy is seen in majority of the casesMRS findings are non specific with decreased NAA & CHO.myoinositol may be mildly elevated
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Brain MRI of an 8 year old female with Rasmussen's encephalitis.A. December 2008, the patient was presented with headache and epilepsia partialis continua. There are lesions with local brain swelling in the right parietal and occipital lobes and right cerebellar hemisphere.B. April 2009, the same patient, now she is comatose with epilepsia partialis continua. There is progression of the encephalitis - the left cerebral hemisphere has been involved with severe brain swelling and shift of the midline structures.
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Points
AgeC/FImaging : hemiatrophy
Normal Basal ganglia calvarial changes No focal
encephalomalacia
IN FAVOUR AGAINST
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sTurGE wEbEr sYndrOMENeurocutaneous syndromeUnlike most phakomatosis it is sporadicEncephalotrigeminal angiomatosis
Hallmarks- Capillary malformation of the skin( port wine mark) in the distribution of the trigeminal nerveRetinal choroidal angioma(+/- glaucoma) andA cerebral capillary-venous leptomeningeal angioma
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paThOLOGYThe leptomeningeal hemangioma results in the vascular steal affecting the subjacent cortex & white matter producing localised ischaemiaMost common location in is the parietooccipital region f/b frontal & temporal lobes Unilateral in 80% cases& is typically Ipsilateral to the the facial angioma ASSOCIATIONS1. COA
2.paragangliomas
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Epidemiology- rare 1 case in 40000-50000 LBNo gender predilection
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CLInICaL prEsEnTaTIOnCongenital facial cutaneous hemangioma (port wine stain/facial naevus flammeus)U/L in 63% or B/L – 31%Usually involves (V1) ophthalmic division of trigeminal nerveIn majority (72%) the naevus is unilateral & ipsilateral to the intracranial abnormalityMC clinical manifestation- seizure ( 1st yr 75 to 90%) medically refractory and worsen with time
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hemiparesis & migraine like headaches Approx 1/3rd of the patients have choroidal or
scleral angiomatous involvement Complicated with retinal detachment, buphthalmos
or glaucoma Most are mentally retarded
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radIOGraphIC fEaTurEs
X-RAY- gyriform calcification of the subcortical white matter (Tram-track appearance)finding usually become evident between 2 & 7 yrs of age
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CT fIndInGs
NCCTDystropic cortical/subcortical calcification( hall mark)Cortical calcifications, atrophy and enlargement of the I/L choroid plexus are typical findings in older children and adults with SWSBONE CT- thickening of diploe & enlargement with hyperpneumatisation of the I/L frontal sinuses secondary to longstanding volume loss in the underlying brain
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CECT Dense cortical calcifications may obscure
enhancement of the pial angioma on CECT however enlarged enhancing choroid plexus can
usually be identified
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MrI fIndInGs
T1WI-Signal of affected region largely normal, with anatomic volume loss evident at older age
T1 C+(gd)Prominent leptomeningeal enhancement in affected areaFLAIR scan may demonstrate serpentine hyperintensities in the sulci, ‘ ivy sign’
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Post contrast T1WI OR FLAIR sequences best demonstrate pial angioma
Serpentine enhancement covers the underlying gyri, extending deep into the sulci & sometimes almost filling the SA space
The I/L choroid plexus is almost always enlarged & enhances intensely
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T2WI: low signal in white matter subjacent to angioma representingPostulated accelerated myelination in neonateCalcification in later lifeAbnormal deep venous drainage seen as flow voids
MR SPECTROSCOPY- Decreased NAA
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DSA
In most cases (82%) angiography is abnormal & demonstrates absent superficial cortical veins with abnormal and enlarged deep venous drainage
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TreaTmenT and prognosis
Primary –seizure control Surgical resection in refractory cases Ophthalmological examination is also essential
to identify & treat ocular involvement
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Points
AgeC/FImaging :HemiatrophyThickening of calvaria
No cutaneous lesionsNo MRImaging :no cortical or SC calcificationsNormal I/L choroid plexusNo involvement of frontal sinuses
IN FAVOUR AGAINST
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hemimegalencephaly
Rare congenital disorder of cortical formation with hamartomatous overgrowth all or a part of a cerebral hemisphere
Results from either increased proliferation or decreased apoptosis(or both) of developing neurons
Abnormal mTOR signaling may play a role
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epidemiology Rare represent less than 5 % of MCDs Cryptogenic congenital d/o does not have a
recognised racial or gender predilection
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classificaTion
Classified into 3 forms1.Isolated2.Syndromic: Epidermal nevus syndromeKlippel trenaunay syndromeMccune-Albright syndromeProteus syndromeRare- NF1 & TS
3. Total hemimegalencephaly: hypertrophy also involves the brainstem & cerebellum
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clinical presenTaTion
Usually presents in infancyMacrocrania, developmental delay & seizuresMajority(90%) present with focal or generalised infantile spasmsExtracranial hemihypertrophy of part or all of the iplsilateral body may be present
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imaging findings
GENERAL FEATURESHMEG is characterised by enlarged, dysplastic-appearing hemisphere with abnormal gyration, thickened cortex,& white matter abnormalitiesLateral ventricles enlarged & deformedIn rare cases- dyplastic changes involves only a part of hemisphere(focal/localised/lobar HMEG)
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cT findings
NECT shows enlarged hemisphere and hemicranium Posterior falx often appears displaced across the
midline Abnormal white matter myelination may increase in
attenuation, making the C/L normal WM appear unusually hypodense
Dystrophic calcification may be seen Abnormal, uncondensed primitive appearing
superficial veins over regions of severely dysplastic cortex
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mri findingsT1WIOften Cortex appears thickened “lumpy-bumpy” on T1 scansNeuronal heterotopias are commonI/L ventricle is usually enlarged and deformedIn severe cases almost no normal hemispheric architecture can be discerned
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T2WI – shows areas of patchy & polymicrogyria with indistinct borders between gray & white matterWhite matter signal intensity on T2/FLAIR is often heterogenous with cysts and gliosis-like hyperintensity
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Figure 1: NCCT of head (a) Axial shows diffuse enlargement of left cerebral hemisphere (*) with midline shift towards right while the right hemisphere is normal.
Non-contrast magnetic resonance imaging T2W (b) Images (axial) show diffuse gyral thickening (thin arrow) in the left cerebrum causing midline shift and scalloping of inner table (arrow head) of left calvarium. White matter in both cerebral hemispheres show normal myelination. Linear hyperintensities involving the subcortical U fibers of left fronto-parietal region seen on fluid attenuation inversion recovery image (c) Post-gadolinium T1W image in axial plane (d) shows no abnormal enhancement
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TreaTmenT and prognosis
Rx Targeted to control epilepsy In refractory cases –Hemispherectomy
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Points
C/F- seizure/develpmt delay Imaging :assymetry in b/l hemisphereProminent ventricles
Age (infancy)No extracranial hemihypertrophyImaging :No abnormal enlargement of hemisphere seen
IN FAVOUR AGAINST
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diagnosis
DDMS
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THANK YOU….