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CCM inter-hospital grand-round 15 th March 2011

CCM inter-hospital grand-round 15 th March 2011. Cannot eat, cannot breathe then cannot see! Dr Alfred Chan Caritas Medical Centre. Clinical History (1). 21 year old girl, born in HK, student Normal full-term spontaneous delivery Unremarkable past health Non drinker and never smoke

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CCM inter-hospital grand-round 15 th March 2011

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  1. CCM inter-hospital grand-round15th March 2011 Cannot eat, cannot breathe then cannot see! Dr Alfred Chan Caritas Medical Centre

  2. Clinical History (1) • 21 year old girl, born in HK, student • Normal full-term spontaneous delivery • Unremarkable past health • Non drinker and never smoke • No history of drug abuse • “Flu-like illness” in early June 2010 •  weak left face + nasal regurgitation on fluid drinking ~ 2 weeks later

  3. Clinical History (2) • Visited acupuncture experts  Reluctant to quit from school examination • Attended AED of hospital A on 9th July 2010 for choking after drinking • Facial weakness became bilateral • Subjective weak voice and slurred speech

  4. AED of Hospital A • BP 106/85; P 112 regular; temp 37; • GCS 15/15; PERL • No ophthalmoplegia • Limb power and sensation normal • Otoscopy and hearing normal • Bilateral VII palsy, LMN type • Markedly diminished gag reflex

  5. Admit M&G of hospital A • No ocular movement deficit • Normal facial sensation/ hearing • Tongue movement intact • Poor gag reflex + bilateral LMN VII palsy • Tandem-walking  unsteady • WC 13.4 (80% neutrophil); normal R/LFT • ESR 73; CRP 27 • CT Brain: ? Right pons hypodense spots

  6. Initial list of differential diagnosis • Viral brainstem encephalitis • Gullain-barre syndrome • Myasthenia gravis • Vertebral artery dissection • Sarcoidosis • Nasopharyngeal carcinoma  lower cranial nerve invasion

  7. CSF analysis Essentially all normal • Protein 0.42; Glucose 3.2 • WCC 22; RBC 57 • Cryptococcal antigen negative • Bacterial/ AFB/ fungus culture negative • Cytology  no malignant cell • Virus: Enterovirus/ VZV/ HSV all negative • VDRL negative

  8. Is it MG crisis? 20 Jan 2009, CCM grand-round

  9. Private MRI • Hyperintense spots in antero-superior part of lateral end of both IAM • Short hyperintense line in labyrinthine portion of right facial nerve • Suggestive of bilateral facial neuritis • MRA showed normal vasculature • Incidentally retention change over bilateral paranasal sinuses

  10. Neurology workup • Tensilon test negative • Anti-Ach receptor antibody negative • Immune markers and ANCA negative • Urine porphobilinogen  negative • Nerve conduction test • Conduction block at Lt tibial/ Rt peroneal • Sural nerve intact bilaterally

  11. Treat as ?Bickerstaff encephalitis • IV ampicillin + IV Acyclovir > 9/7 to 15/7 • IVIG at 0.4g/ kg weight > 14/7 to 18/7 • Hydrocortisone 100mg Q8H > 20/7 to 25/7 • Prednisolone (tapering dose)> 26/7 to 5/8 • CT brain 7th Aug  putamen lesions+ • Rt dilated pupil FU eye as uveitis • She wants to withhold invasive treatment/ workup if cancer diagnosed

  12. End Cannot Eat

  13. Unexpected problems (1) • TSH <0.01 and T4 30.4 • Carbimazole 10mg BD + inderal 10mg tds since 10th Aug to 14th Aug • Complete heart block on 15th Aug  temp pacing at CCU on 16th Aug • Sputum retention and respiratory failure  intubated at ICU on 17th Aug • Started Sulperazon + Amikacin for HAP

  14. Sputum culture 15th Aug Acinetobacter species (Heavy growth) • Gentamicin Resistant • Ciprofloxacin Resistant • Cotrimoxazole Resistant • Unasyn Resistant • Piperacillin + Tazobactam Resistant • Tienam Resistant • Sulperazon Resistant • Amikacin Susceptible • Colistin Susceptible

  15. Unexpected problems (2) • Weaned off ventilator since 21st Aug • Fever 40 degree + unconscious 25th Aug  resume ventilator support • Change pacing wire to LIJV • Stop Sulperazon and Amikacin  change to Cloxacillin (ETA grew MSSA 24th Aug) • EEG  generalized encephalopathy

  16. End Cannot Breath

  17. Unexpected problems (3) • Seen by eye for increasing redness • Right endophthalmitis diagnosed 25th Aug • Bedside Intravitreous Vancomycin + Amikacin performed • CT orbit 25th Aug • Increased Rt vitreous chamber • hypodensities at bilateral basal ganglia

  18. Eye review on 26th Aug • Vision-saving procedure has to be done in CMC due to restriction on the portability of instruments. • Hopefully to remove sepsis focus • Patient was escorted to ICU of CMC on 27th Aug, under specialty of M&G, to manage ophthalmological emergency • Plan back to Hospital A after eye OT

  19. CMC ICU admission 27th Aug • GCS E2VtM1 • Under sedation of Dormicum/ fentanyl • Pulse rate 90 sinus, not pacing dependent • Minimal motor response to pain for limbs • Lines not inflamed and CXR clear • Eye OT not available on same day • ? Primary sepsis foci

  20. Contrast CT brain on 27th Aug

  21. Blood result on CMC arrival • Hb 7.2 NCMC; Platelet 146 • WCC 5.9 (neutrophil 5.1; lymphocyte 0.5) • INR 1.0; APTT 28 • RFT normal, A/G= 19/34, ALT 56 • TSH 0.13, T4 at 23.9 • ABG pH 7.49; CO2 31.5; pO2 185 (0.3 O2) • G6PD negative (May need Septrin later)

  22. Management strategy • Eye intervention as planned • Sepsis: second lumbar puncture for CSF sample + taking other specimens • Consult ENT for assessment • Consult neurology for (a) Primary event leading to bulbar palsy; and (b) Workup of current CNS severe dysfunction

  23. (1) Eye intervention 28th Aug • Right eye: dense fibrin/ pus in anterior chamber + dense vitritis + inferior rhegmatogenous retinal detachment (RRD) • Left eye: chronic RRD • Impression: Rt endophthalmitis + Lt RRT • Procedure: right eye AC washout + Phacoemulsification of cataract + endolaser vitrectomy + Repair of RRD + Intravitreal Vanco & Amikan

  24. (1) Eye intervention: plan • Poor visual prognosis of right eye • Aim to preserve left eye vision • Local antimicrobial to right eye  Hourly Gutt Vancomycin and Gutt Gentamycin • Right vitreous matter for bacterial and fungal culture  subsequently negative • Need daily assessment at ICU

  25. Never end Cannot see

  26. (2) Sepsis  workup • CSF glucose 2.0; protein 2.57; WCC 3; no bacterial/ fungus/ AFB growth • Nasal fluid aspirate: Candida albicans + MRSA + Enterococci + Acinetobacter • ETA  Acinetobacter species • CSU  Candida albicans + Enterocci • Blood culture negative for bacteria/ fungus • Vitreus matter  nil bacteria/ fungus

  27. (2) Sepsis  what to cover? • After a bit discussion: • Vancomycin 1g Q8H • Fluconazole 400mg stat then 200mg daily • Netromycin 150mg daily IV • Serum Beta-D-Glucan 173.7 (<80)  Possible systemic candidiasis

  28. (3) ENT intervention • 27th Aug: to refer back hospital A when fit • 30th Aug: bedside endoscopy showed tiny streak of mucus in Rt middle meatus  swab taken for culture (MDR-Acinetobacter + MRSA + enterococci) • 3rd Sept: Persistent fever despite broad coverage  Right maxillary sinus antrotomy only straw colour fluid !!

  29. (3) ENT intervention • Comments 3rd Sept 2010: “Based on the clear colour character of antral washout and negative local sign around he head & neck/ peri-nasal • Chance of frank sinusitis is low • Further procedure e.g. biopsy not helpful”

  30. Bulbar palsy CNS inflammation Sinusitis Endophthalmitis with retinal detachment Persistent severe sepsis Uncertain cause Nil culture result ENT not support Dx Poor prognosis but need QD assessment Limited choice of antimicrobials Dilemma as a CCM physician Antral washout grew MDR-Acinetobacter Will you treat accordingly?

  31. (4) Neurologist assessment • Review the initial diagnosis leading to admission to Hospital A • Assess the current neurological status

  32. Bulbar dysfunction: DDx Brainstem pathology Infiltration, infection, vascular Nerve Cranial nerve disorders Acute neuropathy with bulbar dysfunction Motor neuron disorders ALS SMA Isolated pseudobulbar palsy tetanus Myopathy Inflammatory (PM, DM, IBM) OPMD Thyroid disorders Distal myopathy Neuromuscular junction Myasthenia gravis

  33. Miller Fisher Syndrome A clinical variant of GBS; acute post-infectious paralytic illness Triad: ophthalmoplegia, ataxia, areflexia Pupillary abnormalities, ptosis, bulbar symptoms, facial weakness CSF: Albumino-cytological dissociation Anti-GQ1b anti-ganglioside antibodies: sensitive for FS and its variants

  34. Bickerstaff’s brainstem encephalitis (BBE) Closely related to MFS Additional: Altered consciousness, long tract signs Similar CSF and anti-GQ1b Antibody findings as in MFS MRI brain abnormality present in 30%

  35. Anti-GQ1b IgG antibody syndrome: clinical and immunological range Source: JNNP 2001;70:50-55

  36. What is unusual in this patient? No ophthalmoplegia ever documented No alteration of consciousness on presentation til very late phase CSF: normal protein + raised WCC Nerve conduction study: normal Fever persists

  37. (4) Neurologist assessment: summary Initial presenting illness • Bulbar palsy with normal NCT, but clinically nil response to IVIG and steroid Deterioration right now • Cerebral dysfunction with raised CSF protein • Hypointensities in bilateral temporal and Rt BG Need to consider other diagnosis • Sinusitis with orbital + CNS extension • For contrast MRI brain + skull base

  38. Searching for an answer…. • Anti-HIV negative, VDRL negative • Blood fungal culture/ bacterial negative • Paired serum viral titer not raised • CMV pp65 antigen negative • 2nd porphyria screen negative • Immune markers all negative • Anti-Ganglioside Q1b & anti-GM1 not raised • Serum Vancomycin trough 21.3 • Hb 9.9; Plt 123; WCC 5.6; ALT 63 (3/9/10)

  39. Searching for an answer, again….. • 9th July: Plain CT Brain (Hospital A) • 7th Aug: Plain CT Brain (Hospital A) • 25th August: Plain CT Orbit (Hospital A) • 27th August: Contrast CT brain + Sinus • 4th September: MRI brain + brainstem • 4th September: Plain CT brain • 10th September: Contrast CT brain • 11th September: Plain CT sinus • 15th September: MRI brain + DWI

  40. Brain was so ill, but no answer…. Condition since 4th September 2010 • Multi-focal inflammatory change over bilateral basal ganglia/ thalamus/ temporal lobes and brainstem on MRI, unlikely due to bacterial infection • GCS E4VtM1 and all limbs flaccid • Persistent high fever and tachycardia despite Vanco/netro/fluconazole 14 days

  41. While waiting, do something • Temporary tracheostomy on 6th Sept • Moderately enlarged thyroid • Milky discharge from left thyroid • Thyroid tissue sent for histology • Tracheostomy #8 inserted

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