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CPC

CPC. Dr. Clayton wiley Madison Pilato, PGY-3 Neurology. Patient GM.

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CPC

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  1. CPC Dr. Clayton wiley Madison Pilato, PGY-3 Neurology

  2. Patient GM • 51 yo M with homelessness, HTN, DM, prior strokes without residual deficits, CAD s/p stenting who presented to an OSH 8/2 for AMS, progressed to becoming non-verbal, for which he was transferred to PUH on 8/4. Work up at OSH included CTH, Brain MRI, MRA head which demonstrated multiple old and new infarcts. • Recently unable to afford any of his medications

  3. Histories • Past Medical History: • HTN • DM c/b retinopathy • Multiple strokes, hemorrhagic and ischemic • CAD s/p stenting x2 2007 • CKD3 • Alcohol abuse • Past Surgical History: none • Family Medical History: non-contributory • Social History: • ETOH, polysubstance, homeless

  4. General exam • TempC 37.2 BP 207 (136-207/96 (71-96) Pulse 66 RR 14 SatO2 96 • Gen: Awake, alert and in no acute distress. • HEENT: Non-icteric. Moist MM. • CV: Regular rate and rhythm • Resp: Clear to auscultation bilaterally. • Ab: soft, non-tender and non-distended. • Ext: No peripheral edema.

  5. Neuro exam • MS: Awake, alert, slow but appropriate responses. Language fluent. • CN: PERRLA. EOMI without nystagmus. Facial sensation intact to LT. +slight facial asymmetry. Hearing intact to finger rub bilaterally. Uvula midline with symmetric palatal elevation. SCMs and shoulder shrug normal. Tongue protrudes midline. • MOTOR: Normal bulk and tone. No pronator drift. No adventitious movements or bradykinesia. • UE strength 5/5 deltoids, biceps, triceps, wrist flexors, wrist extensors, finger extensors and abductors, and hand grip bilaterally • LE strength 5/5 iliopsoas, gluteals, hamstrings, quadriceps, tibialis anterior, gastrocnemius • REFLEXES: 2+ at biceps, triceps, brachioradialis, patella, and achilles. No clonus. Flexor plantar response bilaterally • SENSORY: Intact to light touch • COORDINATION: Subtle dysmetria on finger-nose-finger and heel-shin on left • NIHSS=5 (LOC1 LOCq1 FP1 Ataxia 1 Extinction/Inattention 1)

  6. Labs • Na 136 K 4.4 Cl 102 HC03 26 BUN 26 Cr 1.5 • WBC 5.8 Hgb 13.7 Plt 211 • UDS negative

  7. Imaging

  8. Stroke Work up • Lipids LDL 153 • HgbA1c 7.7 • TEE: EF 60-65%, mild TR, no right to left intracardiac shunt, no vegetations, no cardiac source of emboli, no intracardiac thrombus or masses, atheromatous disease of the descending thoracic aorta, focal mildly thickened plaques

  9. Clinical course • 8/4-8/5 diaphoretic soaking sheets with worsening AMS • EEG: left frontal, right frontotemporal rhythmic delta activity • LP: WBC 1 RBC 1 Lymph 56 Monocytes 24 Mononuclear cells 20, Glucose 92, Protein 42 • CSF Culture NG CMV EBV HSV VZV all negative • CSF autoimmune/paraneoplastic negative • Blood cultures NG5D • TSH 0.884, B12 474, folate 19.2, HIV neg, ESR 42, CRP 8.0 • Repeat Brain MRI (8/5)

  10. Clinical course continued • 8/6-8/7 acutely agitated with tachycardia and ?posturing with requiring intubation. EtOH w/d? • ABG 7.27/34/271/15 Lactate 102.2 • CT/CTA no acute hemorrhage or vessel occlusion • DSA: diffuse vasculopathy • cEEG: one episode of tachycardia and posturing captured without EEG correlate. Generalized theta/delta slowing. • Tox screen negative for ethanol, acetone, isopropanol, methanol, ethylene glycol, salicylate

  11. Clinical course continued • 8/7-8/8 stopped following commands, began to triple flex to painful stimulation • Rheumatology consult: noted nephrotic range proteinuria, AKI (Cr peaked at 2.5) • Toxicology consult • Consider levamisole (cutting agent in cocaine) – induced vasculitis • Comprehensive drug screen (?never done) • Repeat Brain MRI (8/8)

  12. Vasculitis/vasculopathy work up • ANA <80 dsDNA 1.0 • ANCA neg • Anti-smooth muscle ab negative • Cryoglobulin screen negative • SPEP, IEP, serum free light chains – not done? • UPEP: no monoclonal immunoglobulins • RF <20 CCP • SSA/SSB <0.2/<0.2 • C3/C4 73 (range 79-152)/19 • HCV neg, HbsAg negative • Beta 2 macroglobulin (negative), anticardiolipinAb (negative), LAC panel: positive hexagonal lipid neutralization test is specific but isolated evidence for a lupus anticoagulant • RPR negative • Quantiferon GOLD not valid x 2

  13. Clinical course continued • 8/9 Brain biopsy by Neurosurgery • Mild hypertensive vasculopathy and mild diffuse gliosis . The hypertensive changes are most prominent in the white matter . There is no evidence of vasculitis, intravascular lymphoma or infection. Additionally, there are no PAS-positive granules within arterial walls to suggest CADASIL. • Skin biopsy: no rash/abnormal area to biopsy; final report was normal skin • Started on empiric steroids • Febrile, felt to be 2/2 dysautonomia • Continued to have paroxysmal tachycardia • CT CAP (without contrast): Areas of consolidation in the dependent portion of the lower lobes as well as scattered centrilobular groundglass nodules and tree-in-bud pattern areas of opacification in dependent portions of all lobes highly concerning for aspiration. No convincing evidence of neoplastic process. No evidence of pheochromocytoma.

  14. Clinical course continued • Repeat Brain MRI (8/14): mild progression in the confluent left cerebellar infarct and a new subcentimeter right caudate head infarct • 8/15: extremely poor neurologic exam (NIH2528) • EEG with lateralized periodic discharges, generalized periodic discharges, generalized slowing • s/p 5 days high dose steroids with no improvement • PLEX • Serum paraneoplastic/autoimmune panel negative • NMO Ab: negative • 8/18: Family arrived at bedside and made patient CMO, he passed ~3 hours after terminal extubation • Family requested autopsy

  15. Radiology / Pathology

  16. Radiology / Pathology

  17. Pathology A- Biopsy site • H&E

  18. Pathology B: Caudate, CC, Left • H&E

  19. Pathology C: Basal Ganglia, Left • H&E

  20. Pathology D: Cerebellum, infarct acute • H&E

  21. Pathology E: Hippocampus, acute infarct • H&E • CD68 • GFAP • Neurofilament • APP

  22. Pathology F: Cerebellum, infarct chronic • H&E

  23. Pathology G: Midbrain • H&E • CD68 • GFAP • Neurofilament • APP

  24. Pathology H: Pons • H&E

  25. Pathology I: Medulla and Pituitary • H&E

  26. Pathology J: Basal Ganglia Right, Infarct remot • H&E

  27. Final Neuropathology Diagnosis • Severe arteriolosclerosis with multifocal chronic and acute infarcts

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