Selected Topics in Rehabilitation and Radiology

1. 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

2. 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

3. 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

4. American Academy of Neurology Practice Parameters • Available on line – http://www.aan.com/professionals/practice/index.cfm?a=0&fc=1 • 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

5. Imaging of the Neonate • Ultrasound on all < 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

6. 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

7. Myelination – Delayed vs abnormal(PVL vs leukodystrophy)

10. Hypoplasia Corpus Callosum + Septo-Optic Dysplasia (mild)

11. Hypoplasia Corpus Callosum + Septo-Optic Dysplasia (mild)

12. Vermian and CC hypoplasia Term baby, smart, bulbar-CP like picture

13. Amino acid non-ketotic hyperglycinemia glycine synthase maternal phenylketonuria methyl malonic acidemia Mitochondrial pyruvate dehydrogenase pyruvate decarboxylase fumarase Organic Acid glutaric acidemia congenital disorder of glycosylation 3-hydroxyisobutyric aciduria Peroxisomal Zellweger Refsum adrenoleukodystrophy Metabolic errors – Agenesis CC

14. 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 Metabolic errors - Cerebellar

15. 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

16. 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

17. 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 Aug;131(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.

18. Lissencephaly Microgyria Pachygyria Schizencephaly Migrational Defects

19. 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

20. 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?”]

21. 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

23. T2, gadolinium enhancementhttp://www.med.ege.edu.tr/norolbil/2000/NBD12600.htmlhttp://www.annalsofian.org

24. Representative axial T2- (upper left), noncontrast T1- (upper middle), postcontrast T1- (upper right), and fast fluid-attenuated inversion recovery (FLAIR) (bottom row)

28. 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

29. 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

30. 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)

32. Craniosynostosis

33. Post TBI with parietal fracture

34. Socially Responsive Child

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

37. Teen with SMA and knee pain

38. 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

39. 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

40. SMA - obstruction