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Radiology Rounds

Radiology Rounds. Katherine Crook October 26, 2011. History. Precious Vallone Presented in February 2011 9 yr FS Maltese History of progressive vision loss, lethargy and ataxia One month prior to presentation– less responsive to commands

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Radiology Rounds

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  1. Radiology Rounds Katherine Crook October 26, 2011

  2. History • Precious Vallone • Presented in February 2011 • 9 yr FS Maltese • History of progressive vision loss, lethargy and ataxia • Onemonth prior to presentation– less responsive to commands • One week prior to presentation– right head tilt and intermittent rolling to the right

  3. Exam Findings • Physical Exam • Unremarkable • Neurological Exam • Mentation- QAR, appropriate • Posture- Right head turn • Gait- Non-ambulatory tetraparetic (R significantly weaker than left) • Palpation- Resents palpation of the cervical spine, but no overt pain response • Postural Reactions- Absent CP in all four limbs; intact hopping in left thoracic limb, but otherwise absent • Cranial Nerves- Absent menace OU, severely reduced PLR’s OU, decreased dazzle OU • Spinal Reflexes- Reduced withdrawal in right thoracic limb, all other reflexes intact • Sensation- intact

  4. Diagnostics • CBC/Chemistry • Thoracic radiographs • Ophthalmology Consult • MRI • CSF tap

  5. MRI findings • Mild T2 hypointensity within the dorsal aspect of the spinal cord • Lesion is hypo- to isointense on T1

  6. MRI Findings T1 Post-contrast T1 Post-contrast • Well defined contrast-enhancing lesion within the dorsal aspect of the spinal cord from the foramen magnum to the cranial aspect of C3 • Intracranial meningeal enhancement

  7. Results and Outcome • Lumbar CSF tap- • NCC: 22/µ/l, RBC: 648/µ/l, Protein: 563.8 mg/dl • Mild mononuclear pleocytosis • Placed on 1 mg/kg/day of Prednisone, Doxycycline and Clindamycin while waiting for results of Infectious disease testing • Infectious disease testing came back negative • Cyclosporine added to treatment plan • Currently tapering Prednisone

  8. MUE • GME, NME, NLE … • Common inflammatory conditions of the CNS • Definitive diagnosis on histopath • Collectively represent an aberrant immune response • Presumptive diagnosis based on history, neuro exam, imaging and CSF • Ante-mortem diagnosis complicated by overlap in diagnostic results– therefore termed MUE

  9. GME • aka Reticulosis, Inflammatory reticulosis of the brain and spinal cord, neoplastic reticulosis (reclassified as CNS lymphosarcoma or malignant histocytosisby Vandevelde) • Acute onset, progressive, can be multifocal or focal • Females, toy and terrier breeds are overrepresented; Mean age of onset approx. 4.5 yo • NME- younger; Pugs, Maltese, Yorkies, Chihuahua • CSF- Mild to moderate mononuclear pleocytosis with increased protein, but occasionally can be normal • Three forms described- focal, disseminated and occular

  10. GME • MRI- typically hyperintense on T2 with variable T1 intensity and variable contrast enhancement; lesions typically assume an infiltrative appearance, with a predilection for the white matter. • Pathology- Angiocentric, non-suppurative, mixed lymphoid inflammatory process predominately affecting the white matter of the brain and spinal cord • Disseminated- widely scattered lesions in cerebrum, caudal brainstem, cervical spinal cord and meninges • Focal- true mass lesion typically resulting from the coalescing of perivascular cellular infiltrates involving a large number of blood vessels in one region • Occular- perivascular infiltrates primarily localized to the retinal or post-retinal aspects of the optic nerve and optic chiasm • Prognosis- Varies with type, but generally poor without aggressive immunosuppression

  11. Thanks

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