Inflammatory Disease

Question 1

Name 4 infectious inflammatory CNS diseases (5)

Answer 1

–Viral

–Bacterial

–Protozoal

–Algae

–Parasitic

Question 2

Name 5 non infectious CNS inflammatory diseases (7)

Answer 2

–GME

–NME

–NE

–SRMA

–Cauda equina neuritis

–Pachimeningitis

–Eosinophilic

Question 3

Label

Answer 3

A) Grey matter

B) White matter

C) Meninges

D) Ventricles

Question 4

How do you diagnose:

• GME granulomatous meningoencephalomyelitis
• NME necrotising meningoencephalitis
• NLE necrotising leukoencephalitis

Answer 4

Histopathology

If no histopathology = MUA

Question 5

What are the clinical signsof inflammatory CNS disease?

Answer 5

–Acute/sub-acute in onset

–Progressive in nature

–Associated with multifocal/diffuse neuroanatomical localisation

–Systemic signs rarely reported

Question 6

A) What is GME?

B) What is characteristic?

Answer 6

A) Inflammatory (immune-mediated) disease of unknown aetiology

B)

•Characteristic granulomatose, angiocentric encephalitis

–Macrophages

–Lymphocytes

–Plasma cells

Question 7

With GME is there more:

White matter and meninges OR

Grey matter?

Answer 7

Grey

Question 8

What 3 things are seen with angeiocentric encephalitis with GME?

Answer 8

–Macrophages

–Lymphocytes

–Plasma cells

Question 9

What is this?

Answer 9

Angiocentric encephalitis

Question 10

What are the 2 classifications of GME?

Answer 10

• Clinical
• Histopathological

Question 11

What are the 3 clinical forms of GME?

Answer 11

•Disseminated

–Most common, acute rapidly progressive multifocal signs

–Cerebrum, caudal brainstem, cerebellum, or cervical spinal cord

•Focal

–Acute or slowly progressive signs reflecting single space-occupying mass lesion

•Ocular

Question 12

What are the 3 major pattern of lesion distribution with GME?

Answer 12

•Common disseminated form

–Spinal cord, brainstem and mid-brain (<

• Disseminated form with angiocentric expansion (multiple coalescing lesions)
• Focal form, single discrete mass lesion

–Spinal cord, brainstem and mid-brain, thalamus, optic nerves, cerebrum

Question 13

What are the clinical features of GME?

Answer 13

•Young adult dogs (average age 4.5yrs range from 6 months to 12 years)

• Any gender
• Female, toy and terrier breeds over- represented

Question 14

What do the clinical signs of GME reflect?

Answer 14

Lesion distribution

Question 15

What are the clinical signs of GME?

Answer 15

–Vestibulo-cerebellar

–Cranial nerves deficits

–Visual impairment

–Paresis (tetra)

–Cervical pain

–Body turn

–Altered mentation

–Seizures

Question 16

How do you diagnose GME?

Answer 16

–Rule out systemic dx

–Advanced imaging (MRI)

• Multiple hyperintensities on T2-weighted or (FLAIR) throughout the CNS white matter.
• Variable intensity in T1-weighted
• Variable contrast uptake on T1 post gadolinium
• Variable meningeal enhancement

–CSF

•Mononuclear mixed pleocytosis, increased TP

–Histopathology

• Angiocentric
• Mixed-lymphoid
• With matter encephalitis
• Lymphocytic meningitis

Question 17

How do you treat GME?

Answer 17

–Supportive

•Mannitol?

–Immunomodulatory

Question 18

A) What is NME?

B) Where is there pan-encephalitis?

C) Where is mainly affected?

Answer 18

A) Inflammatory (immune-mediated) disease of unknown aetiology

B)

–Meninges

–Grey matter

–White matter

C) Cerebral hemispheres mainly

Question 19

What is this?

Answer 19

•Non-suppurative, necrotising ME

Question 20

What are the clinical features of NME?

Answer 20

• Young adult dogs mean age 2Yr (6mo to 7Yr)
• No gender predisposition
• Breeds? Pug, Maltese, Pekingese…
• Rapidly progressive signs

–Seizures

–Depression

–Circling

–Visual deficits

Question 21

How do you diagnose NME?

Answer 21

–Rule out systemic dx

–Advanced imaging (MRI)

• hyperintense on T2-weighted and FLAIR
• Isointense to slightly hypointense on T1-weighted
• Variable contrast enhancement on T1-weighted
• Loss of grey/white matter demarcation

–CSF (lymphocytic), increased TP

–Histopathology

• Lymphocytic meningitis
• Polioencephalitis
• Necrotising leukoencephalitis

Question 22

How do you treat NME?

Answer 22

–Supportive

• Antiepileptic
• Mannitol?

–Immunomodulatory

Question 23

NLE:

A) What is it?

B) What does it cause?

C) What is seen?

Answer 23

A) Inflammatory (immune-mediated) disease of unknown aetiology

B) Non-suppurative, necrotising encephalitis

C)Hemispheric white matter, and brainstem

•Cortex and meninges not involved

Question 24

What is this?

Answer 24

•Non-suppurative, necrotising encephalitis

Question 25

What is this?

Answer 25

•Hemispheric white matter, and brainstem

Question 26

What are the clinical features of NLE?

Answer 26

* Young adult dogs mean age 4.5Yr (4mo to 10Yr) * No gender predisposition * Breeds? Yorkshire terrier, Pomeranian, French bulldog

Question 27

What are the signs of NLE?

Answer 27

Rapidly progressive signs * Depression * Circling * Vestibulocerebellar signs * Visual deficits * Seizures

Question 28

How do you diagnose NLE?

Answer 28

–Rule out systemic dx –Advanced imaging (MRI) * Hyperintense on T2-weighted and FLAIR images * Multiple cystic areas of necrosis * Hypointense or isointense on T1-weighted images, * Contrast enhancement is variable –CSF monocytic, increased TP –Histopathology * Asymmetric, bilateral necrotising encephalitis * Hemispheric white matter and brainstem * Overlying cortex and meninges not involved * Histiocyte, microglia and macrophages cell infiltrates and lymphocytic perivascular cuffing

Question 29

How do you treat NLE?

Answer 29

–Supportive •Mannitol? –Immunomodulatory

Question 30

How can you treat MUA?

Answer 30

Immunosuppression * Corticosteroids * Cytosine arabinoside * Ciclosporines * Azathioprine

Question 31

How can we treat MUA with corticosteroids?

Answer 31

* Prednisolone, 1.5mg/kg BID then progressively reduced over 6 months to a maintenance dose of 0.5mg/kg EOD * Dexamethasone, 0.2mg/kg SID then progressively reduced over about 6 months to a maintenance dose of 0.05mg/ kg EOD

Question 32

How can you treat MUA with Cytosine Arabinoside?

Answer 32

* Synthetic nucleoside analog, competes for incorporation into nucleic acidsà inhibits DNA polymerase in mitotically active cells * 50gm/m2 SC every 12 hours for two consecutive days, this protocol is repeated every 3 to 6 weeks (or longer intervals according to clinical signs) * Monitor haematology

Question 33

How can you treat MUA with ciclosporin?

Answer 33

* Suppressing T-lymphocyte activation and proliferation * 6mg/kg PO every 12 hours * Can be combined with ketoconazole * Adverse effects: * GI upset * Gingival hyperplasia * Change in coat (shedding or hirsutism)

Question 34

How can you treat MUA with azathioprine? Include the side effects

Answer 34

* Thiopurine, interferes with the productions of purine nucleotides = increased lymphocytes proliferation * =Hepatic metabolism * 2mg/kg SID * Tapered to 0.5-1mg/kg EOD long term Side effects: Bone marrow suppression and GI upset

Question 35

What is SRMA? What is seen?

Answer 35

SRMA steroid responsive meningitis arteritis * Inflammatory (immune-mediated) disease * Suppurative inflammation (neutrophilc) * Leptomeninges spinal cord * Necrotising fibrinoid arteritis

Question 36

What is the predisposition of SRMA?

Answer 36

* Young dogs 6-18months * No gender predisposition * Any breed but over represented –Beagle –Boxer –Bernese mountain dog –Weimaraner –etc

Question 37

What are the clinical features of SRMA?

Answer 37

* Profound cervical hyperaesthesia * Pyrexia * Depression * Neutrophilia

Question 38

What are the 2 forms of SRMA?

Answer 38

* Acute “typical” * Chronic, due to inadequate treatment or relapses of acute disease

Question 39

How do you diagnose SRMA?

Answer 39

–Neutrophilia –(MRI) longus colli myositis, altered CSF signal –CSF * Acute: marked neutrophilic pleocytosis and raised TP * Chronic: mixed or mononuclear pleocytosis and raised TP * Measure IgA in serum and CSF

Question 40

What is this?

Answer 40

SRMA steroid responsive meningitis arteritis

Question 41

What does this show?

Answer 41

SRMA steroid responsive meningitis arteritis

Question 42

How can you treat SRMA?

Answer 42

Immunosuppression * Corticosteroids * Cytosine arabinoside * Ciclosporines * Azathioprine

Question 43

What is the prognosis of SRMA?

Answer 43

Fair to good

Question 44

A) What is Cauda equina neuritis? B) What is occassionally affected? C) What is the likely origin? D) Who is most affected?

Answer 44

A) Rare and sporadic disease in horses and Involves the nerves on the cauda equina B) Occasionally affects CN, VII, VIII and rarely V C) Likely autoimmune or post infectious origin (herpes, adenovirus) D) Adult female mostly affected

Question 45

What does cauda equina neuritis lead to?

Answer 45

To tail paralysis and sphincters incompetence

Question 46

What makes Cauda equina neuritis lymphoplasmacytic?

Answer 46

•The inflammation starts in the intradural portion of the nerve root

Question 47

What makes Cauda equina neuritis granulomatous?

Answer 47

The inflammation continues in the extradural portion forming large “mass- like” lesions

Question 48

How do you diagnose Cauda equina neuritis?

Answer 48

•Compatible clinical signs –Tail paralysis, incontinence, gait deficits, CN •CSF (lumbar) –Non-suppurative pleocytosis –Elevated TP •Post mortem

Question 49

What is the prognosis of Cauda equina neuritis?

Answer 49

Poor

Question 50

A) What is Eosinophilic meningoencephalitis (EME)? B) Who is at risk? C) What is seen?

Answer 50

A) Inflammatory likely immune-mediated, allergic mechanism?!? B) Young, male Golden Retrievers and Rottweilers may be over-represented, any breed and gender can be affected C) Severe meningitis and periventriculitis with infiltration of underlying parenchyma

Question 51

How do you diagnose Eosinophilic meningoencephalitis (EME)?

Answer 51

* MRI multifocal changes * CSF = eosinophilic pleocytosis

Question 52

What is this?

Answer 52

Eosinophilic meningoencephalitis (EME) •eosinophilic pleocytosis

Question 53

What is the prognosis of Eosinophilic meningoencephalitis (EME)?

Answer 53

–Immunosuppressive doses of corticosteroids –Occasionally needs additional immunosuppressive drugs such as cytosine

Question 54

Pachimeningitis (hypertrophic chronic): A) What is this? B) Which breeds are at risk? C) What is it chracterised by?

Answer 54

A) Inflammatory likely immune mediated origin B) Greyhounds and crossed-sight hounds are over-represented C) Charaterised by diffuse thickening of the dura mater by fibrosing inflammatory process

Question 55

What do the clinical signs of Pachimeningitis (hypertrophic chronic) reflect?

Answer 55

Multiple CN deficits

Question 56

What is the most common sign of Pachimeningitis (hypertrophic chronic)?

Answer 56

Dropped jaw

Question 57

How do you diagnose Pachimeningitis (hypertrophic chronic)?

Answer 57

* MRI * CSF may be inflammatory

Question 58

How do you treat Pachimeningitis (hypertrophic chronic)?

Answer 58

–Immunosuppressive doses of corticosteroids –Occasionally needs additional immunosuppressive drugs such as cytosine

Question 59

What is the prognosis of Pachimeningitis (hypertrophic chronic)?

Answer 59

–Fair, most dogs achieve clinical remission however a cure is difficult to obtain