Neurological vasculitis

Question 1

Define vasculitis

Answer 1

Inflammation of the blood vessels that may result in damage to the vessel walls
Fibrinoid necrosis - and vascular occlusion with subsequent ischaemic lesions in organ tissue

Question 2

What is secondary vasculitis?

Answer 2

Autoimmune inflammatory condition

Question 3

What is primary vasculitis?

Answer 3

De novo inflammation that is occurring in the vasovasorum (nerve that supply the nerve sheath)

Question 4

What is the treatment for vasculitis?

Answer 4

Secondary vasculitic neuropathy - treat underlying disease

Question 5

What is the treatment for non-systemic vasculitides?

Answer 5

Corticosteroid mini therapy (1mg/kg per day) tapered to 25mg by 3 months and to 10mg at 6 months; in severe cases combination therapy may be necessary

Question 6

What are the clinical spectrum of Vasculitis?

Answer 6

1. Peripheral nerve vasculitis

2. CNS vasculitis

Question 7

W/hat are examples of peripheral nerve vasculitis?

Answer 7

1. Primary
2. Secondary
3. Non-systemic vasculitis neuropathy (NSVN)

Question 8

What are examples of CNS vasculitis?

Answer 8

1. Primary

2. Secondary

Question 9

CASE 1

Answer 9

62 year old man

• Type II diabetes - controlled
• Hypercholesterolemia
• Diverticular disease
• Hypertension
• Depression

Question 10

History of Case 1

Answer 10

Increasing numbness of the right hand
New grip weakness of the left hand + left forearm pain.
One month of poor balance and falls
Skin papules involving his fingers
Occasional night sweats (every few days)
Neurological symptoms stabilised but developed skin
changes of his fingers progressed.
Systemic enquiry: 2 stone weight loss in the last year, transient joint pain.
No visual, speech, swallowing difficulties or sphincter disturbance
1-year ago numbness in the calf and knees (improved with better diabetes control).

Question 11

What are the clinical characteristic of vasculitic neuropathies?

Answer 11

Acute/subacute
Asymmetrical, non-confluent
Focal neuropathy (16.5%)
Multifocal neuropathy (56%)
Distal symmetrical sensory or sensorimotor polyneuropathy (25%)
May have cranial nerve involvement
Constitutional symptoms (weight loss, fever, rash, weight loss, fatigue or night sweat)

Question 12

What is Romberg positive

Answer 12

Ask patient to put feet together and close eyes and see if they sway left/right

It is a dorsal column dysfunction

Question 13

What are examples of primary systemic vasculitis neuropathy?

Answer 13

1. Small vessel
2. Middle vessel
3. Large vessel predominant

Question 14

What is an example of small vessel predominant?

Answer 14

Churge-Strauss syndrome

Microscopic polyangitis

Question 15

What is an example of medium vessel predominant?

Answer 15

Poly arthritis nodosa

Question 16

What is an example of large vessel predominant?

Answer 16

Giant cell arthritis

Question 17

What are examples of secondary systemic vasculitis?

Answer 17

1. Connective tissue disorder
2. Rheumatoid arthritis
3. Drugs
4. Infection
5. Malignancy

Question 18

What are examples of non systemic vasculitis?

Answer 18

Microvasculitis etiology e.g. diabetic
lumbosacral plexopathy
sicca syndrome

Question 19

What is the diagnosis for case 1?

Answer 19

Electro-diagnostic
Acute-to-subacute axonal loss of sensory and motor fibres
Patchy, multifocal
Asymmetrical or non-length dependent

Tissue diagnosis
Sural, superficial peroneal, median, ulnar, and radial sensory nerves
and peroneal, tibial, median, and ulnar motor nerves

Laboratory testing to exclude secondary vasculitis:
Blood: FBC, ESR, ANA, ENA, RHF, ANCA, Cryoglobulins, hepatitis B/C
and HIV
Cancer screen: paraneoplastic antibody, CT-PET or CT chest/abdomen/pelvis
Urine analysis
CSF analysis (normal >80%)

Question 20

What is the treatment ?

Answer 20

Secondary vasculitic neuropathy– treat
underlying disease

Non-systemic vasculitidies: corticosteroid monotherapy (1mg/kg per day) tapered to 25mg by 3 months and to 10mg at 6 months; in severe cases combination therapy may be necessary.

Question 21

What was the update on case 1?

Answer 21

Secondary vasculitic neuropathy
Neck lymph node was identified on screening
– confirmed non-Hodgkins lymphoma
Referred to Haematology and was given chemotherapy

Question 22

What are the clues for vasculitic neuropathy?

Answer 22

Non-confluent neuropathy
Painful
Subacute presentation

Question 23

What is the clinical history for case 2?

Answer 23

22 year old Caucasian man
Mild headache (for 24 hours)
Stuttering weakness of his left leg and arm (over 10
minutes)
Frequent cramping of his left arm (lasting 2 minutes
at a time) – this continued for 24 hours
Short term memory impairment and drowsiness

Spenomegaly 3 years ago – glandular fever

Question 24

What are the key examination findings for case 2?

Answer 24

Vital signs: BP 144/78, P 79 reg, RR 24, Sats 96% on
air, Temp 36oC

GCS 14 (E3, V5, M6)

Dysarthric speech

Left UMN sided weakness (involving the face/arm/leg)

Systemic examination was unremarkable

Question 25

What are the clinical presentation of cerebral vasculitis?

Answer 25

Stroke
Encephalopathy/Cognitive changes
Seizures
Headache
Movement disorders
Systemic features; fever, rash, night sweats,
weight loss, anorexia
Usually acute/subacute – can be chronic or
relapsing or remitting

Question 26

What are primary vasculitis of CNS?

Answer 26

1. Granulomatous
2. Lymphocytic
3. Angiographically defined
4. Mass lesion presentation
5. Amyloid B-related

Question 27

What are the treatment for primary CNS vasculitis?

Answer 27

No randomised studies
Some studies have shown effectiveness of cyclophosphamide in combination with corticosteroids
Monitoring is challenging, often MRI imaging
is used

Question 28

What does CNS vasculitis have?

Answer 28

Non-specific presentation thus making the diagnosis challenging

Biopsy is a gold standard