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Table 1 Clinical, radiological and laboratory findings in 15 MOG-IgG-positive patients with a history of brainstem involvement

From: MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome

 

#1

#2

#3

#4

#5

#6

#7

#8

#9

#10

#11

#12

#13

#14

#15

Sex

Ff

f

f

f

f

f

m

m

m

m

f

f

f

f

f

Ethnicity

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

Cauc

BSTI at onset

y

y

n

n

n

n

n

n

y

y

y

y

n

y

n

Age at first evidence for BSTI (years)

53

18

45

35

31

53

19

19

50

37

44

27

26

25

22

Time to first evidence for BSTI (years)

0

0

18

0.75

0.25

41

0.3

0.17

0

0

0

0

1.7

0

0

No of clinical BST attacks

5

1

2

3

1

0

1

1

0

1

1

1

1

2

1

No of attacks with subclinical BSTI

2

0

0

0

0

1

0

1

1

0

0

0

1

0

0

Clinical BST findings

Central hypoventilation, dysphagia, dysarthria, CN III and VII paresis

Respiratory impairment, difficulties coughing, dysphagia, dysarthria, diplopia

Cerebellar gait and upper limb ataxia

Impaired balance, vertigo

Intractable nausea and vomiting

None, subclinical BST involvement

Double vision and gait ataxia

Hearing loss

None, subclinical BST involvement

Trigeminal hypesthesia

None, subclinical BST involvement

Trigeminal hyp- and paresthesia, diplopia, nystagmus, unsteady gait

Hypesthesia tongue and face, impaired smooth pursuit

Hemihypesthesia including the face

INO

Infratentorial MRI findings

MRI1: Cerebral peduncle of the midbrain, MO, pons, MRI2: MO, new T2 lesions, Gd+, MRI3: pedunculus cerebellaris, crus cerebri, patchy Gd + pons, MO

Pontine tegmentum and cerebellar peduncles

Crus cerebri and entire pons, around the 4th ventricle, extending into the left cerebellar hemisphere

Pons and medulla oblongata

Right and left dorsal MO, ad-jacent to the 4th ventricle, incl. the area postrema

MO with patchy Gd enhancement

Lesions in the peri-aqueductal gray, ventral pons

T2 lesion in the pons

Median pontine lesion

N.d.

Cerebellar peduncle, single lesion

T2 lesions extending from the ponto-med. junction, throughout the MO to C5, incl. around the 4th ventricle

T2 lesions in the MO and pons, detectable over a period of at least 12 months

Bilateral pontine lesions and bilateral cerebellar peduncle lesions

Large, Gd + lesion: pons bilat., both pedunculi cerebelli, paramedian ponto-medullary junction

 Cerebral peduncles

y

n

y

n

n

n

n

n

n

n.d.

n

n

n

n

n

 Pons

y

y

y

y

n.d.

n

y

y

y

n.d.

n

y

y

y

y

 Cerebellar peduncles

y

y

n

n

n

n

n

n

n

n.d.

y

n

n

y

y

 Cerebellum

n

n

y

n

n

n

n

n

n

n.d.

n

n

n

n

n

 Medulla oblongata

y

n

y

y

y

y

n

n

n

n.d.

n

y

y

n

y

 Bulbo-spinal lesion, ever

y

n

n

y

y

y

n

n

n

n.d.

n

y

n

n

n

 Gd+, ever

y

n

y

y

y

y

y

y

y

n.d.

n.d.

n

n

n.d.

y

Supratentorial MRI findings

Normal

T2-hyperintense lesions in the frontal and parietal subcortical white matter

Crus cerebri, left subcort. white matter (adjacent to the temporal horn), corpus callosum, juxtacortical regions of parietal lobes

Lateral ventricular lesions

Normal

Normal

Confluent T2 hyperintense lesions in the right temporal lobe, pulvinar bilaterally

T2 lesions in basal ganglia, corpus callosum, periventr., pulvinar thalami, rostral putamen; leptomeningeal contrast enhancement

Normal

Normal

Single frontal lobe lesion

Juxta-cortical T2 lesion, insular region

Callosal, periventr., juxtacortical, deep white matter

Single small lesion directly adjacent to the left lateral ventricle

Peritrigonally and corona radiate, Gd+

Postinfectious/postvaccinal

n

y

n

n

n

y

n

y

y

n

n.d.

n

n

y

n

Simultaneous ON and BSTI

y

n

n

n

n

n

n

y

y

y

n

y

n

n

n

Simultaneous MY and BSTI

y

y

n

y

y

y

y

y

y

y

y

y

y

n

y

History of both ON and MY

y

n

n

y

y

y

y

y

y

y

y

y

y

n

y

Recurrent disease

y

n

y

y

y

y

y

y

y

n

y

y

y

y

y

NMOSD 2015

n

n

y

y

y

n

y

y

n

n

y

y

y

n

y

CSF-restr. OCB

n

n

n

y

n.d.

n

y

y

n

n

n

n

y

n

n

CSF WCC

normal

360

50

normal

122

30

22

60

normal

8

n.d.

59

33

150

8

CSF neutrohils

n.a.

7 %

6 %

n.a.

3 %

n.d.

6 %

26 %

n.a.

34 %

n.d.

n.a.

n.d.

26 %

n.d.

QAlb elevated

n.d.

y

n.d.

n

n

y

n

y

n

n

n.d.

n

n

y

y

Last EDSS

10

1

7.5

3

3

4.5

1

0

1

0

2.5

0

3.5

3

0.5

  1. Abbreviations: BSTI brainstem involvement, y yes, n no, n.a. not applicable, n.d. no data, f female, m male, Cauc Caucasian, MO medulla oblongata, Gd + gadolinum enhancing, CN cranial nerve, MRI magnetic resonance imaging, MY myelitis, ON optic neuritis, NMOSD 2015 neuromyelitis optica spectrum disorder according to Wingerchuk et al. (2015), CSF cerebrospinal fluid, OCB oligoclonal bands, QAlb albumin CSF/serum ratio, EDSS extended disability status scale, INO internuclear ophthalmoplegia