Approach to a patient with CNS Vasculitis - Primary Vs Secondary

1. A CASE OF
?CNS VASCULITIS
? CNS INFECTION
Dr.ARUL SELVAN
Presenter : Dr.M.Ramesh Babu

2. History
• Mr. X 62 yrs old male, right handed person, occupation-
retired manager from air port authority from Chennai settled
in Seychelles.
• K/C/O AIHA - 2015 - No Comorbids, Presented with chief ℅
• Weakness of Rt.UL, LL -3months - 24 oct - 2017 & Lt.LL - 2
months
• Fever -3 months, intermittent, moderate degree, not a/w
chills & rigors & night sweats
• Altered sensorium- 2months
• Loss of appetite & weight loss - 3 months
• On 27/09/17 Presented with symptoms of tiredness,
slowness in daily activities, reduced memory, increased
sleep with LBA- 3 weeks

3. HOPI• Pt. was k/c/o Refractory AIHA on
Immunosuppressants - On 27/09/17 Presented with
symptoms of tiredness, slowness in daily activities,
reduced memory, increased sleep with LBA- 3
weeks- admitted and evaluated-
• ANA +ve, CSF - Lymphocytic pleocytosis, slight
raise in protein with normal glucose & Neg
back.pack., MRI-Multiple subacute hemorrhagic
infarctions in basal ganglia and thalamus and
watershed infarcts in ACA-MCA, MCA-PCA
territory on both sides, MRA- red. calibre in
intracranial portion of both ICA & MCA on both
sides- Diagnosed as CNS vasculitis - Treated with
Prednisolone & Azathioprine - Discharged on
5/10/17.

4. • On 24/10/17 Developed sudden onset of Rt.
UL&LL weakness found to have acute stroke -
admitted in Sychelles hospital, treated ,
partially recovered, on physiotherapy -
developed aspiration pneumonia - persistent
fever moderate degree, intermittent, no fixed
timing, without any chills, rigors & night sweats
.
• Developed altered sensorium - worsening of
sensorium & Lt.LL weakness ,not able to
respond commands, recognise wife & son -
taken him to Srilanka (18/12/17)for further
evaluation and treatment.

5. • No h/o headache, LOC, Seizures, starring look,
tongue bite, involuntary movements, nausea,
vomiting, skin rashes, joint pains, bleeding
manifestations
• No h/o chest pain /palpitations/ breathlessness
• No h/o abdominal pain, melena, loose stools,
• No Hematuria, swelling of lower limbs

6. • CT Brain done - left thalamic hemorrhagic
infarction , B/L multi infarcts.
• 20/12/17- acute haemorrhage in Lt. Frontal
lobe , thalamus, body of Lt.corpus callosum
with intraventricular haemorrhage c ventricular
dilatation.
• 23/12/17 - VP shunt was done
• PEG was inserted on 24/12/17
• In view of no improvement - brought here for
further management.

11. • Family history - Nil
• Personal history - Takes mixed diet, Dec.
Appetite, lost weight ~ 8-9 kg in last 3 months,
no habits, no high risk behaviour.
• No drug / toxic exposure
• No contact with TB persons

12. Series of Events
• Diagnosed AIHA - Dec 2015 - initiated on
steroids
• Steroids stopped in May - 2016
• Relapse - In Oct 2016 - Reinitiated on 60 mg of
Wysolone - PET CT done - enlarged spleen c
inc. uptake diffusely hyper metabolic marrow.
• Received 4 cycles of Rituxumab - followed by
steroid 40 mg with tapering dose.

13. • 03/10/17 - admitted with fever, memory disturbance,
constitutional symptoms of -1 month.
• ANA ++ , CSF - 10 lymphocytes with protein - 48, Glucose-
71mg/dl , Stains - ve, Xpert MTB -ve, AFB/Fungal c/s -neg,
Urine for Histoplasma- ve
• PET CT repeated - abnormal leptomeningeal enhancement
with mediastinal periportal lymphadenopathy.
• Treated as CNS vasculitis - with Azathioprine 50 g &
Prednisolone 20 mg
• 24/10/17 - MRI Acute stroke Lt. Basal ganglia infarction / Rt.
Hemiparesis
• 15/11/17 - Aspiration Pneumonia - 18/11/17 left to Srilanka.
• 23/12/17 - VP shunt was done
• Brought here and admitted on 15/1/18

14. On Examination
• Patient - Bed bound , Conscious, Markedly Disoriented,
spontaneous eye opening +, Moves left UL, GCS- E4V1M4-5
, not obeying commands, no eye contact
• Pupils - 3mm b/l reactive, EOM - no gaze preference,
• Facial lag Rt.side+
• Aphasic
• Motor system - Rigidity +
• Power 2/5 4-/5
• 2/5 2-3/5

15. • DTR’S - 1+
• B/L Plantar - Extensor
• Sensory - not tested
• No cerebellar signs noted
• Neck rigidity +
• No Involuntary movements
• Other systems - NAD
• Gr II pressure sore +

16. Provisional Diagnosis

17. • Provisional Diagnosis:
• AIHA/ Multiple hemorrhagic and ischemic
infracts
• ? Part of vasculitis
• ?TB
• ?Histoplasmosis
• ?Paraneoplastic

18. Investigations
• HB- 9.4gm%
• WBC- 7490cells/cumm, -P 83%, L-9%,M-7%
• ESR- 63mm/hr
• Reticulocytes- 2.0 %
• P.S - Occ. ellliptocytes, occ. tear drop cells seen, left meta myeloid
shift
• RBS - 183 mg/dl
• CUE - N
• RFT - N
• LFT - N
• S.Na - 143, k+ - 3.1, Mg- 2.1, LDH - 382u/l

19. • Blood c/s , Anaerobic c/s , Urine c/s - Yeast like cells
• HIV 1&2, HbsAg - Neg
• CSF - 25 cells, L-99%, Few RBC, Gl- 49mg/dl, protein-
80.7 mg/dl, Lac- 26.3 mg/dl, Bact. pack - Neg, Xpert
MTB- neg, Cryp. Ag - Neg., CSF c/s - SFNG, AFB c/s
- Awaited, C3 & C4 -N , CRP- 8.9mg/dl, PCT - N
• PT,APTT,INR- N
• Meningoencephalitis panel - HSV2 +ve, HSV PCR +ve
• TSH- N
• ECG- N
• 2D-Echo- EF 65%, GrII DD, No vegetations

21. Images

39. Treatment
• Inj.Acyclovir 500mg IV TDS,
• Inj.Meropenam 2gm IV TDS
• T.Prednisolone 40 mg OD
• Supportive medications
• Neurological status - same - No improvement

40. Possibilities
• HSV2 vasculitis
• CNS Vasculitis
• TB
• Paraneoplastic

42. Primary CNS vasculitis (PACNS)
• Defined as inflammation of the cerebral
vasculature without angiitis in other organs
affects small- and medium-sized arteries of
the brain parenchyma, spinal cord, and
leptomeninges
• 1. Granulomatous angitis of the CNS
(GACNS)
• 2. AtypicalPACNS

43. • GRANULOMATOUS ANGIITIS OF THECENTRAL NERVOUS
SYSTEM
• 20% of all patients with PACNS
• male-predominant (2:1) at any age
• mean age at diagnosis is 42 years,
• long prodromal period insidious onset of
symptoms

44. Clinical Manifestations
• Characterized by a long prodromalperiod
• Signs and symptoms of systemicvasculitis,
such as, fever, weight loss, or rash, are
usually lacking.
• highly variable and nonspecific
• PACNS should be suspected when strokes,
more often recurrent, occur in young patients
& unexplained diffuse neurologic dysfunction

46. Clinical
1.Headache, the most common
symptom, (generalized / localized,
slowly worsening, spontaneously
remitting for periods, and varies in
severity)
2.Cognitiveimpairment - insidious in
onset
3.Focal neurologicalmanifestations
!!!Constitutional symptoms (fever
and weightloss) are uncommon.
Salvarani C,Brown RDJr, Calamia KT,et al. Primary central
nervoussystem vasculitis: analysis of 101patients. Ann Neurol
2007;62:442–51.

47. †P, 0.05 versus 1983–2003cohort.
‡Defined asthe presence of at least 1of the following: fatigue, anorexia, weight
loss,arthralgia.

48. PCNSVworkup
Serology
CSF
Neuroimaging
i
Cerebral
angiography
BrainBiopsy
Imaging

49. Proposed criteria for PACNS
• The presence of an acquired and otherwise
unexplained neurologic deficit and with
• (a) the presence of either classic angiographic
orhistopathologic features of angiitis within the
CNS, and
• (b) no evidence of systemic vasculitis orany
condition that could elicit the angiographic or
pathologic features

50. • MR imaging =
• single or multiple, may include infarcts (both white
and gray matter) and hemorrhage, and may be
tumor-like
• Nonspecific high-intensity T2WI/FLAIR lesions in
white matter present in 42 percent of Secondary
vasculitis (low specificity )
• MR angiography — The resolution of MR
angiography (MRA) remains inadequate for the
demonstration of vasculitic changes

51. Neuroimaging
• Angiography
• positive findings if focal or diffuse areas ofarterial
stenosis, occlusion, dilatation, or beading were
detected
• findings of ectasia and stenosis referred to as"beading",
usually in the small arteries
• sensitivity of angiography in biopsy proven PACNS
cases was only 60 percent
• Thus, a negative angiogram cannot be used toexclude
the diagnosis of PACNS.

55. • Thank You