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Clinical case presentation August 2023

Prepared by: Dr Ho Yi Bin

Supervisor: Dr. Rizah Mazzuin Razali

Current posting: HKL


Case Scenario

Mdm NSL • 74 years, Chinese Female • ADL independent, CFS 3


Underlying

1) Hypertension

2) Dyslipidemia

3) Left eye cataract operation done on early July 2022


HOPI

  • Presented to ED HKL 26/7/2022 with dizziness x 4/52

  1. Spining sensation of the environment

  2. worsened upon movement of head

  3. partially resolving with rest

  4. associated with on and off nausea and vomiting

  5. not associated with changing postures

  6. not affecting ADL

  • Bloatedness and poor appetite x2/52

  • Flashes of light x 4/52 - claimed occurring post cataract surgery

Otherwise

  • denies chest pain, palpitation, dyspnea

  • denies syncope/presyncope

  • denies tinnitus/ear pain/ear discharge

  • denies head trauma

  • denies history of fall

  • denies fever

  • denies URTI


• Had visited ED x 3 past month - treated as Dyspepsia

• For current admission - ECG at ED HKL noted T inversion V1-6

• Diagnosis given at ED: Unstable Angina


Further history from pt’s daughter

  • Ambulation: previously independent, started using walking frame since end of June 22 due to dizziness - fear of fall

  • bADL: independent

  • iADL: independent - patient still able cook daily and manage her medications

  • Short term memory loss, forgetfulness - past 3 months

  • Visual hallucination x 1/12 – saw late husband

  • No abnormal mood changes or inappropriate behaviour

  • No incontinences / no constipation

  • Denies family hx of neurodegenerative, pyschosis disorder


O/e:

  • E4V5M6, orientated, obey command

  • BP 120/74

  • HR 78

  • Dxt: 7.2

  • Right eye immature cataract, no nystagmus


  • Lungs clear

  • CVS no murmur

  • Abdomen soft, no hepatopsplenomegally, non tender

Neurological examination

  • Tone Normal, no clonus

  • Power all 4 limbs 5/5

  • Reflex bilateral UL and LL Normal

  • Light touch sensation intact

  • Plantar reflex - downgoing bilaterally

  • Cerebellar sign negative bilaterally

  • Revised diagnosis in ward: Vertigo TRO Cerebellar stroke


  • CT brain plain (27/7/22): Right lentiform nucleus old infarct, ventricles and basal cisterns not effaced

  • Traced old notes: No evolving ECG changes since 2018, ECHO and myoview in 2018 - no ischemic changes

Referred to Opthalmolgy team

• Left eye post op vision - 6/9

• Right eye vision - 6/36, cataract

• Plan: for right eye cataract operation later


Referred to ENT

• Treat as BPPV

• Plan: started on betaserc


On 29/7/22 (day 4 of admisision), patient became delirious

  • Wanted to jump in ocean

  • Unable to identify hospital personnel

  • Started clapping her hands spontaneously


  1. Noted patient having low grade fever

  2. BP: 137/84, HR: 84, spo2: 96 % under RA

  3. Lungs Left Lower zone coarse crepts

  4. NBO x 2/7 - PR impacted stool

  5. Dx: Hyperactive delirium secondary to HAI

  6. Commenced on antibiotic and laxative


  • On 2/8/22 (day 8 of admission), patient developed visual hallucinations in ward

  • Also noted desaturate under room air (Type 1 respiratory failure) - put on Nasal prong 3l/min

  • CRP increased 7 -> 46, with worsening consolidation over Left LZ

  • She was treated for HAP – Antibiotic escalated to IV Tazosin

  • During rounds, noted patient had bilateral mycolonic jerking movements over bilateral UL and LL

  • Unable to perform full neurological examination as patient was still delirious. Noted myoclonic jerks resolve with iv diazepam 10mg stat

Imp: Cover for meningoencephalitis, ddx: TRO CJD

  • Covered with Acyclovir and Rocephine

  • Proceeded with CECT Abdomen and Brain on 11/8/22

  • CECT ABDOMEN: No intrabdomen collection/ascites, no bowel related mass or dilatation

  • CECT brain: No focal enhancing brain lesion/ no leptomeningeal enhancement, no subdural collection

  • Referred to neuromedical team E4V2M5, pupil 3/3 reactive,

  1. not obeying command Bilateral UL and LL increased tone,

  2. power at least 3/5 all limbs Plantar reflex downgoing bilateral Clonus negative

  3. Noted myoclonus jerk affecting limbs and trunk + stimulus sensitive

  4. Imp: Extrapydramidal syndrome TRO CJD

  5. Plan:

1) Loaded with IV Epilim 120mg/8 hrs and then 400mg bd

2) For EEG urgent



EEG urgent on 11/8/22 : Generalized frontally predominant periodic discharges occurring at frequency of 0.5-1 Hz on background of theta and delta wave PLEDS (Periodic lateralized epileptiform discharges) waveform

  • In view of GCS E2V2M5, patient look lethargic, was decided for intubation for airway protection

  • Was electively intubated on 11/8/22 at 11.30am. Post intubation, patient was started on single inotrope

  • Post intubation - noted on and off myoclonic jerk over the mouth and B/L upper limb upon sternal rub

  • Was repetead EEG on 12/8/22 (post intubation) -> generalized periodic discharges (GPEDS)

  • given IV Midazolam 2mg stat and the GPEDS attenuated

  • By neuromedical team:

  1. Increase to iv epilim 400mg tds

  2. Add clonazepam 1mg bd

  3. Cont ivi midazolam 3mg/hr

  • For MRI brain urgent

  1. In view of repeated spike of temperature, desaturation – was treated for HAP and escalated to IV Meropenem 2g TDS on 16/8/22.

  2. Wbc ranging 10-15, CRP: 56, procalcitonin 0.05 to 5

  3. LP was done on 16/8/22

  4. CSF: Blood stained; Cell count 0, No encapsulated yeast

  5. Biochem: Glucose: 4.0, Protein 0.2 g/L

  6. C&S: No growth

  7. Cytology: hemorrhagic CSF smears show scattered neutrophils admixed with a few lymphocytes. No atypical or malignant cells seen

  8. HSV-1 & HSV-2 DNA – not detected

  9. VZV DNA – not detected

  10. Was sent for CSF 14-3-3 protein and RTQuIc at private lab; however informed by lab - Unable to proceed with CSF 14-3-3 protein investigation in view high RBC in sample.

  11. (Family refuse for another LP)

MRI brain & MRA TOF on 19/8/22:

  • Bilateral symmetrical putamen, caudate nuclei, thalami and insular T2W/FLAIR hyperintensity with restricted diffusion

  • Right more than left cortical ribbon T2W hyperintensity with diffusion restriction on DWI at bifrontal, both parietal and right temporal and right occipital suggestive of sporadic CJD

  • In view poor GCS recovery - E1VTM1, no dolls eye reflex (sedation offed since 15/8/22), prolong intubation since 11/8/22, probable sporadic CJD -poor prognosis and persistent HAI with increasing intrope support, family was counselled by anaesth, neuromedical and medical team – was decided for NAR and terminal extubation done on 27/8/22

  • Patient asystole on 28/8/22

  • Cause of death: Ventilator Associated Pneumonia with Probable Sporadic CJD

Blood Ix



Other blood investigations

• Vit B12 (26/7/22): 188 (n)

• Folate (26/7/22): 35.9 (n)

• Am cortisol (27/7/22): 455

• TFT (26/7/22): TSH: 3.16, FT4: 21.8

• HIV/ anti Hep C/ HepBs Ag (11/8/22): negative

• VDRL (11/8/22): negative

• Anti TPO: negative

• Anti Thyroglobulin: negative


POINTS TO PONDER

  • How does the patient's clinical history and physical signs contribute to the diagnosis of CJD?

  • What are the diagnosis criteria for CJD?

  • What are the differential diagnosis for rapidly progressive dementia syndrome?

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As a summary....

This case demonstrates a complex and rapidly progressing neurological condition, ultimately diagnosed as sporadic Creutzfeldt-Jakob Disease.

The patient's clinical history and physical signs that contributed to the suspicion of CJDs are the rapid cognitive decline, visual hallucinations and myoclonic jerks.

The EEG showed periodic sharp wave complexes, and MRI changes further supported the diagnosis. Of course, the other differential diagnosis for rapidly progressive dementia also been work out

- Meningoencephalitis (lumbar pucture and CSF analysis)

- Hypo/hyperthyroidism (TFT)

- Folate, B12 defiency

- Syphilis (VDRL)

- RVD screening

- Hashimoto's Encephalopathy (Thyroid antibodies)

- Malignancy (CT TAP)


The diagnostic criteria for CJD was shared by Dr Angle in the link to CDC 💯


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Re-upload the video clip


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CDC diagnostic criteria for CJD


https://www.cdc.gov/prions/cjd/diagnostic-criteria.html

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Reading for RPD including definition and aetiology


https://www.nature.com/articles/s41582-022-00659-0

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