NEUROIMMUNOLOGY

TELECONFERENCE

A 52 year old male with suspected CNS

vasculitis

Sam Ratulangi University, Manado, Indonesia

IDENTITY
◻ Name : Mr. RP
◻ Age : 52 y.o
◻ Sex : Male
◻ Address : Palu, Central Sulawesi
◻ Admitted : Monday, October 2th 2017
CHIEF COMPLAINT

SUDDEN DEAFNESS
FOR 14 DAYS BEFORE ADMISSION
 PRESENT ILLNESS

14 days before admision Day of admision

• Sudden deafness on • Deafness on the left
the left ear followed by ear (+).
spinning sensation. • Spinning sensation (+)
• Brain CT  infarct in left • Both complaints were
cerebellum. not improved.
• Double vision, difficulty
to swallow, slurred
speech, gait
imbalance, one sided
extremity weakness or
cramps, and trauma
were denied
PAST ILLNESS
▪ (+) for high blood pressure & high cholesterol for 15
years; taken medication irregularly.
▪ No history of stroke, diabetes mellitus, tuberculosis,
recent head trauma, abnormal bleeding, ENT
disease, heart disease, kidney disease, muscle and
joint disease, or skin disease.
▪ No recent surgery.
▪ No allergy.
▪ No routine medication including traditional
medicine.
FAMILY & PERSONAL HISTORY
▪ No history of stroke, hypertension, diabetes mellitus,
trauma, abnormal bleeding, heart disease, or
kidney disease.
▪ No history of autoimmune diseases.
▪ No history of smoking or drug abuse.
▪ No history of bruise, joint pain, abdominal pain,
bloody diarrhea, perforation of intestines, anemia.
▪ A retired teacher with 1 wife and 2 children.
VITAL SIGNS
• Blood pressure : 140/90mmHg
• Heart rate : regular, 80 beats per minute
• Respiratory rate : regular, 20 times per minute
• Temperature : 36.7°C
• Body weight : 70kgs
• Body height : 165cm
• IMT : 25,71 kg/m²
• Visual Analog Scale : 0
GENERAL EXAMINATION
• Within normal limit.
• No specific skin and joint abnormality
were found.
 NEURO EXAM
• GCS: E4M6V5=15
• Pupils: Round, equal, direct light
reflex +/+, indirect light reflex
+/+, diameter 3mm/3mm
2 October 2017
• Meningeal signs: None
• Cranial Nerves:
• Horizontal jerk nystagmus on
right gaze
• Rinne test - /+; Weber test:
Lateralization to the right side
• Motor Examination :
• Muscle Strength :
5/5/5/5|5/5/5/5
5/5/5/5|5/5/5/5
• Muscle Tone: Normal
• Reflexes:
• Physiological :
• Pathological : Babinsky group -/-
and non-Babinsky group: -/-
• Sensory Examination:
Normesthesia for extero- &
proprioceptive modalities
• Coord. & Balance: Coord.:
dysdiadokokinesis -/+; dysmetria -/+
(undershoot); rebound phen. -/-;
Stance: Romberg test & sharpened
Romberg test swayed to the left on
open eyes; Gait: not tested
• ANS : No bowel & bladder incont;
normal hydrosis
 NO & NOT EXAM
• CN I : Normosmia • CN IX and X : Normal
• CN II : Acies Visus ODS: 6/6; • CN XI : Normal
Kampus visus ODS normal; color ODS: • CN XII : Normal
normal; Fundus ODS: Optic nerve papils • Coordination & Balance:
3 October 2017

are round, orange, with well-defined Coordination:

border, Aa:Vv=2:3, Macula: normal, dysdiadokokinesis -/+;
crossing phenomenon (-), bleeding (-), dysmetria -/+ (undershoot);
exudates (-), others (-) rebound phenomenon -/-;
• CN III, IV, VI : Normal eyelid, eye Stance: Romberg test and
movement: horizontal jerk nystagmus on sharpened Romberg test
right gaze, normal accommodation swayed to the left on open
• CN V : Normal eyes; Gait: not tested
• CN VII : Normal
• CN VIII : Acoustic: Rinne test - /+;
Weber test: Lateralization to the right
side; Vestibular: horizontal jerk
nystagmus on right gaze, head shaking
test -; dix-hallpike: not tested
 LAB RESULT (3 Oct 2017)
WBC 9.20 103/ul Na 139 mEq/L
RBC 5.39 106/ul K 4.47 mEq/L
Hb 15.5 g/dl Cl 100.4 mEq/L
Hct 44.2 %
Plt 329 103 /ul PT 14.1 (12.7) seconds
MCH 28,8 pg INR 1.16 (1.01) seconds
MCHC 35.1 g/dL APTT 36.2 (30.9) seconds
MCV 82.0 fl
SGOT 32 U/l Anti HIV Non-reactive
SGPT 72 U/l HBsAg Non-reactive
BUN 34 mg/dL Anti HCV Non-reactive
Creat 1.0 mg/dL
RBS 80 mg/dL
CXR (3 Oct 2017)
BRAIN CT SCAN (27 Sept 2017)
BRAIN CT SCAN
BRAIN CT SCAN
WORKING DIAGNOSIS
Sequele of Cerebral Infarct (POCS;
left AICA)
THERAPY
• ASA 80mg oral qd
• Betahistine mesylate 24mg oral every 12 hours
• Consult ENT Dept
• Plan : Brain MRI (not approved), Cerebral DSA 04
October 2017
CEREBRAL DSA (4 Oct 2017)

Cerebral DSA :
Right Internal
Carotid Artery (RICA)
injection revealed a
stenosis located in
petrous part of RICA
(yellow arrow). There
were also multiple
segments of
stenosis  ? beaded
sign in anterior
cerebral artery
territory (red arrow).
Cerebral DSA :
Contrast injection
from Left Spinal Artery
(LSA) was difficult
because there was a
tortuosity in the
ostium and proximal
segment of Left
vertebral Artery (LVA).
Intracranial projection
revealed a very slow
contrast filling in
Vertebrobasillary
circulation. There were
narrowings of the
AICA and PICA.
 NEURO EXAM POST-DSA
• Spinning sensation ↓
• GCS: E4M6V5=15
• MMSE: 28/30
• Pupils: Round, equal, direct light
5 October 2017
reflex +/+, indirect light reflex
+/+, diameter 3mm/3mm
• Meningeal signs: None
• Cranial Nerves:
• Horizontal jerk nystagmus on
right gaze
• Rinne test - /+; Weber test:
Lateralization to the right side
• Motor Examination :
• Muscle Strength :
5/5/5/5|5/5/5/5
5/5/5/5|5/5/5/5
• Muscle Tone: Normal
• Reflexes:
• Physiological :
• Pathological : Babinsky group -/-
and non-Babinsky group: -/-
• Sensory Examination:
Normesthesia for extero- &
proprioceptive modalities
• Coord. & Balance: Coord.:
dysdiadokokinesis -/+; dysmetria -/+
(undershoot); rebound phen. -/-;
Stance: Romberg test & sharpened
Romberg test swayed to the left on
open eyes; Gait: not tested
• ANS : No bowel & bladder incont;
normal hydrosis
ENT:
• Left sensorineural hearing loss due to
infarction.
• Advise: Audiogram.
• Continue previous therapy.
 THERAPY
• ASA is changed to Clopridogel 75mg qd oral.
• Paracetamol 500mg oral if needed.
• Ibuprofen 400mg oral if needed.
• Echocardiography.
• Consult to Neuroinfection, Neuroimmunology,
and Neuro-AIDS Division.
Neuroinfection, Neuroimmunology, and
Neuro-AIDS:
• Could be a spectrum of primary CNS
vasculitis.
• Advise: ANA Test.
• Will be presented in Neuroimmunology
Teleconference.
• Continue previous therapy.
 LAB RESULT (10 Oct 2017)
WBC 12.10 103/ul ANA Test Speckled pattern
RBC 5.81 106/ul Titer 1:100
HB 16 g/dl
HCT 48.0 %
PLT 345 103 /ul
MCH 27.5 pg
MCHC 33.3 g/dL
MCV 82.0 fl
Total 222 mg/dL
Cholesterol
HDL 53 mg/dL
LDL 141 mg/dL
Triglysceride 222 mg/dL
 NEURO EXAM
• Spinning sensation ↓
• Hearing improved
• GCS: E4M6V5=15
• Pupils: Round, equal, direct light
reflex +/+, indirect light reflex
14 Feb 2018
+/+, diameter 3mm/3mm
• Meningeal signs: None
• Cranial Nerves:
• Nystagmus improved
• Rinne test - /+; Weber test:
Lateralization to the right side
• Motor Examination :
• Muscle Strength :
5/5/5/5|5/5/5/5
5/5/5/5|5/5/5/5
• Muscle Tone: Normal
• Reflexes:
• Physiological :
• Pathological : Babinsky group -/-
and non-Babinsky group: -/-
• Sensory Examination:
Normesthesia for extero- &
proprioceptive modalities
• Coord. & Balance: Coord.:
dysdiadokokinesis -/+; dysmetria -/+
(undershoot); rebound phen. -/-;
Stance: Romberg test & sharpened
Romberg test swayed to the left on
open eyes; Gait: not tested
• ANS : No bowel & bladder incont;
normal hydrosis
 DISCUSSION
• Is this an accidental finding of vasculitis in the
CNS  No neuroanatomically related
findings.
• What test should we do next?