1. The patient is a 62-year-old male who presented with weakness, fever, and altered sensorium over several months. 2. Imaging shows multiple hemorrhagic and ischemic brain infarcts. The patient is diagnosed with possible CNS vasculitis, infection like TB or histoplasmosis, or a paraneoplastic condition. 3. Further workup reveals HSV2 meningoencephalitis. The patient is treated with acyclovir but shows no improvement in neurological status. The primary possibilities being considered are HSV2 vasculitis, CNS vasculitis, TB, or a paraneoplastic condition.

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