Selected Topics in Rehabilitation and Radiology

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Selected Topics in Rehabilitation and Radiology

• Neuroimaging of CP
• Neuroimaging of MS, ADEM, GBS
• More on MRI
• Some rare birds you can learn to recognize
• Osteopenia/Osteoporosis
• Interventional/GI

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Rehabilitation and RadiologyOBJECTIVES

• Demystify/explain fancy new neuroimaging
  techniques at a basic level
• Get a few more board questions right
• Choose well from available options, and know when
  to consult your radiologist
• Recognize and respect contraindications
• Recognize limitations and appropriately apply
  findings to patient management

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Cerebral Palsy

• Who remembers the definition?
• Do they still have idiopathic as most common
  etiology on the boards?
• The most common etiologic sequence and result is
  _________________________?
• Indications for neonatal imaging
• screening, therapeutics, prognostics,
• Indications for diagnostic imaging when they come
  to our clinics

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American Academy of Neurology Practice Parameters

• Available on line http//www.aan.com/professiona
  ls/practice/index.cfm?a0fc1
• Mar 2004Diagnostic Assessment of the Child with
  Cerebral Palsy
• Jun 2002Neuroimaging of the Neonate
• Sep 2003Utility of MRI in Suspected MS
• Jun 1993Magnetic Resonance Imaging in the
  Evaluation of Low-Back Syndrome

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Imaging of the Neonate

• Ultrasound on all lt 30 weeks preterm infants once
  between 7-14 days, again between 36-40 weeks
• MRI slightly better at picking up cystic white
  matter lesions
• Will pick up IVH, PVL, ventriculomegaly
• Non-contrast CT for sick term babies
• MRI between days 2-8 if not conclusive diffusion
  weighted if available
• Pick up major hemorrhages, BG/thalamic
• Research on MRS promising

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Imaging for Cerebral Palsy

• Get MRI if etiology not clearly established.
• Genetic and metabolic testing if
• Positive clinical findings (dysmorphology)
• Specific brain malformations
• Normal brain structurally and deterioration,
  episodic nature, FH, no etiology
• Skip the EEG unless possible seizures
• Check hearing, vision, nutrition, cognition,
  speech
• Consider coagulopathy workup for early CVA

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Myelination Delayed vs abnormal(PVL vs
leukodystrophy)
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Hypoplasia Corpus Callosum Septo-Optic
Dysplasia (mild)
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Hypoplasia Corpus Callosum Septo-Optic
Dysplasia (mild)
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Vermian and CC hypoplasia Term baby, smart,
bulbar-CP like picture
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Metabolic errors Agenesis CC

• Organic Acid
• glutaric acidemia
• congenital disorder of glycosylation
• 3-hydroxyisobutyric aciduria
• Peroxisomal
• Zellweger
• Refsum
• adrenoleukodystrophy

• Amino acid
• non-ketotic hyperglycinemia
• glycine synthase
• maternal phenylketonuria
• methyl malonic acidemia
• Mitochondrial
• pyruvate dehydrogenase
• pyruvate decarboxylase
• fumarase

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Metabolic errors - Cerebellar

• Menkes syndrome
• Smith-Lemli-Opitz syndrome
• Shapiro syndrome
• fetal alcohol syndrome
• acrocallosal
• ectodermal dyplasia
• Lhermitte Duclos Disease (PTEN mutations)
• Pontocerebellar hypoplasia

• CGDS - congenital glycosylation defect,
  (carbohydrate-deficient transferrin)
• Joubert
• recessive, several genes discovered
• hypotonia
• episodic hyperpnea/apnea
• abnormal eye movements
• facial, other dysmorphisms.
• developmental delay
• cerebellar ataxia

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Glutaryl-CoA dehydrogenase deficiency

• encephalopathic crises
• extrapyramidal symptoms
• Treatment
• Glucose/electrolyte IV for acute illness
• Carnitine supplementation
• Low protein, lysine restricted diet
• Neuroimaging
• frontotemporal and basal ganglia atrophy
• subependymal pseudocysts
• delayed myelination
• chronic subdural effusions and hematomas
• Could be msitaken for child abuse

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Other associations

• HIE, NEC, sepsis with either CC or vermian
• Congenital infections with cerebellar
• Toxoplasmosis
• Rubella
• Dandy-Walker and variants
• Chromosomal - trisomy 8, 13, 18, 21
• Peroxisomal disorders and fatty acid oxidation
  defects can produce migration defects
• Folate and neural tube defects

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HIE vs Metabolic

• HIE - end of term gestation -hyperintense signal,
  atrophy of putamen and thalamus, associated with
  static esxtrapyramidal CP
• Signal abnormalities, atrophy in the putamen,
  globus pallidus, or caudate associated with
  genetic-metabolic diseases
• J Pediatr. 1997 Aug131(2)240-5, "Brain magnetic
  resonance imaging in suspected extrapyramidal
  cerebral palsy observations in distinguishing
  genetic-metabolic from acquired causes," Hoon AH
  Jr, Reinhardt EM, Kelley RI, Breiter SN, Morton
  DH, Naidu SB, Johnston MV.

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Migrational Defects

• Pachygyria
• Schizencephaly

• Lissencephaly
• Microgyria

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BOTTOM LINE

• Brain malformations can be caused by
  environmental or genetic factors, by creating a
  toxic or energy-deficient intrauterine milieu,
  changes in membrane function, or disturbing
  normal expression of genes responsible for
  morphogenesis.
• Refer for detailed workup
• Refer urgently if having episodic or step-wise
  deterioration with/without illness
• Later referral may not be helpful

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Imaging of Neuro-Immunologic Disorders

• MS
• ADEM
• Transverse myelitis
• GBS and variants
• question What are several conditions that
  MRI is almost always the best study for?

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Multiple Sclerosis

• Ideal criteria 2 attacks, 2 lesions
• May not fit primary progressive types versus
  remitting-relapsing
• If fewer of either, look for corroboration with
  CSF and/or VEP studies
• Poser, McDonald, newer criteria
• Location of lesions
• Differential gadolinium enhancement
• Differential ADEM, CVD, HIV, sarcoid
• Easy differential Chiari I, cervical SCI

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T2, gadolinium enhancementhttp//www.med.ege.edu.
tr/norolbil/2000/NBD12600.htmlhttp//www.annalsof
ian.org
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Representative axial T2- (upper left),
noncontrast T1- (upper middle), postcontrast T1-
(upper right), and fast fluid-attenuated
inversion recovery (FLAIR) (bottom row)
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MRI variations you just saw

• FLAIR - special T2 sequence analysis
• Fast Fluid Attenuation Inversion Recovery
• CSF subtracted out, good for MS, PVL
• May enhance edges of ventricles as artifact
• Gadolinium MRI contrast material for T1
• highly paramagnetic material
• coordinates with protons of water molecules,
  changes them to a very bright signal

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More MRI variationsmanipulate slice, pulse
orientation and timing, Te Tr values,
post-processing

• Spin Echo
• Gradient Echo
• STIR short tau inversion recovery
• TSHIRT
• Fast Spin Echo
• Time of Flight Angiography 2D 3D
• Fat Separation (Dixon use for fractures)
• Diffusion Weighted Imaging (DWI)
• Echo Planar Imaging (EPI use for FMRI)
• FMRI - BOLD

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MRS (MR Spectroscopy)

• Normal spectrum of eight metabolites
• Reproducible, small files
• Peaks compared with creatine
• NAA higher, myo-inositol half
• Multivoxel and 3D spectroscopy
• Color map superimposed on diagnostic image
• Region of interest down to 2-3 mm, 4-9 min.
• Works reliably only at long TEs, leaving NAA,
  creatine, choline, lipid, and lactate as others
  decay
• Gliomas (choline cell membrane turnover)
• HIE (lactate)
• Dementia (low NAA, high myo-inositol)

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Craniosynostosis
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Post TBI with parietal fracture
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Socially Responsive Child
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Osteopenia

• Plain film not quanititative
• DEXA newer versions may subtract hardware,
  contractures a problem
• Ultrasound very rough screening
• Quantitative CT
• Urinary N-telopeptide

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Teen with SMA and knee pain
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GI Radiology

• KUB
• Stool pattern, pneumatosis
• Barium study upper, SBFT, lower
• Structural anomaly, IBD
• Radionuclide not as sensitive as pH probe but
  useful, quanititative
• No study sensitive for gastritis, esophagitis

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GI - Interventional

• Continent cecostomy
• Anterograde enema
• Brief admission, traning
• Non-surgical tube placement
• NJ by fluoro in cases of SMA
• GJ poor feeding tolerance via GT
• Limitations due to finer gauge tube may not be
  able to use crushed meds

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SMA - obstruction