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MS or Lyme or neither?

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Information about MS or Lyme or neither?

Published on May 6, 2008

Author: NeurologyGuru

Source: slideshare.net

Description

MS or Lyme or neither?
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40 year old lady married + 4 children, born in Israel, with presumptive diagnosis of MS presenting with blurred vision of the right eye 2002 Admitted with right leg weakness Myelopathic signs on examination MRI revealed inflammatory lesion T7-8 LP: TP 501, no cells, OCB negative Treated with steroids with marked improvement No neurological symptoms until current presentation At presentation mild sensory symptoms only

40 year old lady married + 4 children, born in Israel, with presumptive diagnosis of MS presenting with blurred vision of the right eye

2002

Admitted with right leg weakness

Myelopathic signs on examination

MRI revealed inflammatory lesion T7-8

LP: TP 501, no cells, OCB negative

Treated with steroids with marked improvement

No neurological symptoms until current presentation

At presentation mild sensory symptoms only

Current admission 3 days prior to admission, after slight bump to head, noticed blurred vision of R eye Vision deteriorated over following 3 days with pain on eye movement

3 days prior to admission, after slight bump to head, noticed blurred vision of R eye

Vision deteriorated over following 3 days with pain on eye movement

Past history No other neurological problems No other clinical features referable to auto-immune disease ( e.g. SLE, Bechets) No history of miscarriage Serological abnormalities ( to be discussed) Had traveled to New England but no history of tick bite

No other neurological problems

No other clinical features referable to auto-immune disease ( e.g. SLE, Bechets)

No history of miscarriage

Serological abnormalities ( to be discussed)

Had traveled to New England but no history of tick bite

Examination General examination unremarkable RAPD on the right Fundoscopy normal Remainder of examination normal with no evidence of myelopathy Steroids started for presumptive “ MS exacerbation”

General examination unremarkable

RAPD on the right

Fundoscopy normal

Remainder of examination normal with no evidence of myelopathy

Steroids started for presumptive “ MS exacerbation”

Laboratory investigation prior to admission Immunology ATG 87, 87 ( <100) 3.2003, 1.2004 ATPO 793, 538 ( <75)!! (TSH 3.56 - normal) ANA +2 / 4 (2002) –ve x2 (2003,2004) ENA +ve x1 (2002) –ve x2 ANCA –ve x2

Immunology

ATG 87, 87 ( <100) 3.2003, 1.2004

ATPO 793, 538 ( <75)!! (TSH 3.56 - normal)

ANA +2 / 4 (2002) –ve x2 (2003,2004)

ENA +ve x1 (2002) –ve x2

ANCA –ve x2

Laboratory investigation prior to admission Serology CMV past infection EBV past infection VZV past infection Toxoplasma neg Brucella neg VDRL neg Brucella neg Lyme +ve x2 ( 10.2003, 1.2004)

Serology

CMV past infection

EBV past infection

VZV past infection

Toxoplasma neg

Brucella neg

VDRL neg

Brucella neg

Lyme +ve x2 ( 10.2003, 1.2004)

Brain MRI

Laboratory investigation during current admission FBC, Bioch normal ESR 18 LP: - Pressure 12 cm H 2 O - TP 488, no cells, OCB negative Anticardiolipin Ab’s negative pANCA cANCA negative ANA negative TSH normal Anti TPO 397 ( 0-35) Anti TG normal Homocysteine Pending

FBC, Bioch normal

ESR 18

LP: - Pressure 12 cm H 2 O

- TP 488, no cells, OCB negative

Anticardiolipin Ab’s negative

pANCA cANCA negative

ANA negative

TSH normal

Anti TPO 397 ( 0-35)

Anti TG normal

Homocysteine Pending

Course Received high dose steroids Day 4: Ceftriaxone added, steroids tapered Day 5: improvement noted in vision Day 6 am : - mild left hemiparesis noted, steroid dose incresed Day 6 pm: witnessed tonic-clonic seizure. On examination, severe L hemiparesis Brain CT unremarkable

Received high dose steroids

Day 4: Ceftriaxone added, steroids tapered

Day 5: improvement noted in vision

Day 6 am : - mild left hemiparesis noted, steroid dose incresed

Day 6 pm: witnessed tonic-clonic seizure. On examination, severe L hemiparesis

Brain CT unremarkable

Course cont. Further seizures: Unresponsive to benzodiazepines phenytoin, phenobarbital (RSE) Intubation, propofol IV MRI: Right MCA infarct Anticoagulant treatment commenced

Further seizures: Unresponsive to benzodiazepines phenytoin, phenobarbital (RSE)

Intubation, propofol IV

MRI: Right MCA infarct

Anticoagulant treatment commenced

Brain MRI after Seizure

Further Investigation Angiograpghy - decreased perfusion in R MCA territory TEE - no clot detected

Angiograpghy - decreased perfusion in R MCA territory

TEE - no clot detected

Course cont. Weaned from propofol and BZD’s Extubated Marked improvement of hemiparesis

Weaned from propofol and BZD’s

Extubated

Marked improvement of hemiparesis

 

 

 

 

 

Neurological features of chronic Lyme disease

CSF in Chronic Lyme In early cases of neuroborreliosis, spinal fluid findings may still be negative. In cases of chronic disease, only mild elevations of protein may persist. In these circumstances, detection of B. burgdorferi DNA by PCR may be important to establish the diagnosis.

In early cases of neuroborreliosis, spinal fluid findings may still be negative.

In cases of chronic disease, only mild elevations of protein may persist.

In these circumstances, detection of B. burgdorferi DNA by PCR may be important to establish the diagnosis.

 

Serology Both IgG and IgM responses can persist for over 10 years, even after successful antibiotic treatment False positive ELISA results can be caused by other bacteria (e.g. Treponema denticulata ) or by a polyclonal B cell stimulation. Positive serology alone is not sufficient to make the diagnosis Cross-reactivities with syphilis tests do occur Direct detection methods (PCR) diagnostic

Both IgG and IgM responses can persist for over 10 years, even after successful antibiotic treatment

False positive ELISA results can be caused by other bacteria (e.g. Treponema denticulata ) or by a polyclonal B cell stimulation.

Positive serology alone is not sufficient to make the diagnosis

Cross-reactivities with syphilis tests do occur

Direct detection methods (PCR) diagnostic

Value of Serology

 

 

 

Proposed diagnostic criteria clouding of consciousness with reduced wakefulness, attention, or cognitive function no CSF evidence of bacterial or viral infection high serum concentration (or titer) of antithyroid microsomal, antithyroid peroxidase, or antithyroglobulin antibodies

clouding of consciousness with reduced wakefulness, attention, or cognitive function

no CSF evidence of bacterial or viral infection

high serum concentration (or titer) of antithyroid microsomal, antithyroid peroxidase, or antithyroglobulin antibodies

Thyroid function

 

 

MRI in Hashimoto Encephalopathy

Summary of Neurological Features Stroke-like signs in 23 patients (27%) seizures in 56 patients (66%) status epilepticus (10 patients [12%]) course relapsing and remitting in 51 patients (60%).

Stroke-like signs in 23 patients (27%)

seizures in 56 patients (66%)

status epilepticus (10 patients [12%])

course relapsing and remitting in 51 patients (60%).

CSF in Hashimoto Encephalopathy 65 patients (76%), the CSF contained 0 to 3 nucleated cells/mm3 In 3 patients (4%), it contained more than 100 cells/mm3 CSF protein concentration was high in 66 patients (78%), exceeding 0.01 g/dL in 18 patients (21%).

65 patients (76%), the CSF contained 0 to 3 nucleated cells/mm3

In 3 patients (4%), it contained more than 100 cells/mm3

CSF protein concentration was high in 66 patients (78%), exceeding 0.01 g/dL in 18 patients (21%).

Imaging 11 had cerebral atrophy 13 had nonspecific subcortical focal white matter abnormality 8 had diffuse subcortical abnormality, 7 had focal cortical abnormality 1 had transient bilateral narrowing of a middle cerebral artery

11 had cerebral atrophy

13 had nonspecific subcortical focal white matter abnormality

8 had diffuse subcortical abnormality,

7 had focal cortical abnormality

1 had transient bilateral narrowing of a middle cerebral artery

Antiphospholipid syndrome Can simulate MS especially in patients with myelitis and optic neuritis Especially in those without OCB’s ( Karussis et al, Annals of Neurology 1998 ) Associated with stroke in the young However: Anticardiolpin negative during current hospitalisation (steroids should not cause disappearance) Lupus anticoagulant not checked prior to heparin treatment

Can simulate MS

especially in patients with myelitis and optic neuritis

Especially in those without OCB’s

( Karussis et al, Annals of Neurology 1998 )

Associated with stroke in the young

However:

Anticardiolpin negative during current hospitalisation (steroids should not cause disappearance)

Lupus anticoagulant not checked prior to heparin treatment

MS Clinical and MRI features fit Against OCB –ve twice Stroke ( therefore other conditions must be excluded)

Clinical and MRI features fit

Against

OCB –ve twice

Stroke

( therefore other conditions must be excluded)

 

 

 

 

Out of 31 MS patients 5 (16%) had Anti TPO Ab’s Normal values: TPO-Ab 0–10 IU/mL;

 

 

CNS Vasculitis - GANS - PAN ( but no systemic features) GANS CSF and angiography normal MRI probably “ too severe” given mild clinical features prior to stroke No headache Clinical course typical of MS Stroke Optic nerve and spinal cord involvement rare Can simulate relapsing – remitting course of MS Against For

Is MS associated with alteration of platelet function? “ platelet aggregation and MS” Neu et al 1982 Acta neurologica Scand.

Measured in vitro platelet aggregation in 30 “ definite MS” patients and compared to 15 healthy subjects Both spontaneous and “ agonist-induced” aggregation was measured Agg % P < 0.01

Measured in vitro platelet aggregation in 30 “ definite MS” patients and compared to 15 healthy subjects

Both spontaneous and “ agonist-induced” aggregation was measured

Summary MS with stroke is diagnosis of exclusion Lyme disease unlikely but should be excluded via PCR because of the stroke Hashimoto Encephalopathy attractive diagnosis however no encephalopathy clinically or electrophysiologically. APLAS clinically fits..but no lab evidence ( Lupus Anticoagulant not checked) Granulomatous angiitis unlikely but cannot be ruled- out 100% without brain biopsy HIV should be tested for completeness

MS with stroke is diagnosis of exclusion

Lyme disease unlikely but should be excluded via PCR because of the stroke

Hashimoto Encephalopathy attractive diagnosis however no encephalopathy clinically or electrophysiologically.

APLAS clinically fits..but no lab evidence ( Lupus Anticoagulant not checked)

Granulomatous angiitis unlikely but cannot be ruled- out 100% without brain biopsy

HIV should be tested for completeness

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