神经病学中枢神经系统脱髓鞘疾病资料课件

中枢神经系统脱髓鞘疾病Demyelinating

Diseases

of

the

CentralNervous

System1掌握MS概念、病因、发病机制、临床表现、辅助检查、治疗、诊断标准及鉴别诊断。熟悉视神经脊髓炎概念、临床表现、辅助检查、诊断及治疗。了解MS病理、预后；急性播散性脑脊髓炎概念、临床表现、诊断及治疗。Key

points-Demyelinating

Diseases

of

CNS2Chapter

1

Intraduction31. Concept:

A

group

of

diseasecharacterizedby

demyelinating

of

the

brain

andspinalcord.PATHOLOGY:

Demyelination髓

鞘

构

成CNSPNS4562. Pathologic

Findings7①

Destruction

of

the

myelin sheaths

ofCNS;

often primarily

in

white

matter,either

in

multiple

small

disseminatedfoci

orin

larger foci

;②

Infiltration

of

inflammatory cells

in

aperivenous

distribution;③

A

relative

integrity

of

the

axis

cylindersin

the

lesions

and

a

lack

of

wallerian,the

secondary

degeneration

of fibertracts.8临床常见脱髓鞘疾病多发性硬化症(multiple

sclerosis,MS)亚型视神经脊髓炎(Devic

diseases)急性播散性脑脊髓炎(acute

disseminated

encephalomyelitis,

ADEM

)9急性出血性白质脑病(acute

hemorrhage

leukoencephalitis,

AHLE)多发性硬化症(MS)10多发性硬化11Multiple

Sclerosis,MS1.

Concept:

Ms

is

a

kind

of

autoimmunediseases

characterized

bydemyelination

of

CNS.Due

to

its

high

incidence,

chronicity

andtendency

to

attack

young

adults,

it

has

becomeone

of

the

most

important

CNSdiseases.There

are

multipleareas

of

demyelinationwithin

the

CNS.The

episodesofdemyelination

are

separated

in

time

andplace,and

classically

the

disease

runs

arelapsing-remitting

course.(brain

and

spinal

cord)12是一种常见以中枢神经系统炎性脱髓鞘为特征的自身免疫性疾病病灶部位及时间上的多发性多数均以反复多次发作与缓解的病程具有免疫易感性、年轻人多见132.

Etiology

And

Pathogenesis14病毒感染及自身免疫反应：Since

the

exact

cause

isuncertain.

Immunological

mechanisms

undoubtedly

playa

role,although

the

causation

is

probably

multifactorial.麻疹病毒,人类噬

T淋巴细胞病毒(HTLV-I),分子模拟,细胞免疫及体液免疫。遗传因素(inherited

factor)环境因素(environment)15163.

Epidemiology17Incidence

of

MS

associated

with

latitude.On

moving

from

ahigh-prevalence

area

toalow-prevalence

area

prior

to

puberty,the

riskof

developing

MS

is

higher

than

in

the

low-prevalence

area;

However

the

move

is

madefollowing

puberty,

the

risk

of

the

high-prevalence

is

retained.Heredity

may

be

an

important

factor.MS

associated

with

the

HLA-DR

locus

onthe

sixth

chromosome,HLA-DR2expressstrongly

and

then

-DR3,

B7

and

A3.18194.

PathologicFindings20Characteristic:

Multiple

demyelinated

plaques.Position:

White

matteraround

thelateralventricles

and

spinal

cord,opticnerve,

brain

stem

andcerebellar.Acute

stage:

hyperemia,ondema,demyelination,infiltriation

of

inflammatory

cellsdistributed in

perivenous.Recovery

stage

:

Astrocyte

proliferition,

formingof

astrocytic

scab.急性期:充血、水肿、炎性脱髓鞘、血管周围Lc浸润。恢复期:星状细胞增生、胶质斑痕形成。肉眼观：CNS内脱髓鞘斑块215. Clinical

Manifestations22

Prodrome:

The

symptoms

evolvedmore

slowly,

over

several

weeksormonths.Acute

or

subacute

onset

(Relapsing- remitting).3)

Early

symptoms

andsigns:23①

Weakness

or

numbness;(1/2

patients

have

paresthesia

on

one

or

morelimbs)②

The

visual

loss

in

one

or

both

eyes;③

Nystagmus;4)

Common

symptoms

and

signs:24①

paralysis

and

paraplegia;②

The

visual

loss

in

one

or

both

eyes;(1/2

patients

have

visual

disorders,

relapsing-remitting)③

Nystagmus

and

palsy

of eye

muscles;(internuclear

ophthalmoplegia,

PPRFone

andahalf

syndrome)“一个半综合征”25垂直眼震26④

Sensation

disorder:

Romberg’s

sign,27(>1/2)Lhermitte’s

sign;⑤

Ataxia

(1/2), Charcot’s

syndrom

(later

stage);⑥

Impairmentof

PNS;⑦

Attacksyndrom;⑧

Other

clinical

feature.6.

Laboratory

and

assistant

Tests281)

CSF

Test①

Number

of

MNC<15×

106/L;②

IgG-Index

>

0.7(70%);oligoclonal

bands(OB)(95%);③

MBP,

PLP,

MAG,

MOG

Abs

andAb-secreting

cells

;④

CSF-Alb/serum-Alb>1.7(probability

of

MS)Evoked

potentials:50%-90%abnormal.①

visual

evoked

potentials(VEP);②

brain

stem

auditoryevoked

potentials(BAEP)

;③

somatosensory

evoked

potentials(SEP).MRI

:①

preiventricular

plaques;②

regular

plaques

in

brainstem,cerebellum

and

spinal

cord;③

atrophy

symptom.293031323334-

Abnormal

MRI

scans

are

found

in96%

with

a

definite

diagnosis

of

MS

70%

with

a

diagnosis

of

probable

MS3530

-

50%

with

a

diagnosis

of

possible

MS-

MRI

Criteria

for

diagnosing

MSAt

least

3

Lesions

and

two

of

thefollowing:123Lesions

abutting

the

Lateral

Ventricles

Lesions

with

diameters

greater

than

5mmLesions

present

in

the

Posterior

FossaSource(Offenbacher

H,

Fazekas

F,

Schmidt

R

et

al.Assessment

Of

MRI

Criteria

For

A

Diagnosis

Of

MS*Neurology

1993;

43:905-909)Diagnostic

criteria36Clinical

definite

MS

(CDMS):①

two

times

of

attack

and

two

lesions;②

two

attacks,

one

lesion

and

onesubclinical

evidence;Laboratory

supported

definite

MS

(LSDMS):①

Two

attacks,

one subclinical

evidence

andCSFOB/IgG;②

One

attack,

twolesions and

CSF

OB/IgG

;③

One

attack

,onelesion, one

subclinicalevidence

and

CSF

OB/IgG;3. Clinical

probable

MS

(CPMS):①

two

attacks,

one

lesion

;37②

one

attack,

two

lesions

;③

one

attack,

one

lesion

and

othersubclinical

evidence;4. Laboratory

supported

probable

MS

(LSPMS)Two

attacks

;

CSFOB/IgG;Two

attacks

involving

different

part

of

CNS,intermission

at lest

one month

;each

attack

must

continue

for24hs.多发硬化的诊断标准诊断38发作次数 临床病灶数 亚临床证据CSF

OB/IgG(LSPMS）CNS不同部位，

间隔至少一个月，每次持续24小时。临床确诊（

22CDMS）21及1实验室支持确诊21或1+(LSDMS）12+11及1+临床可能211+(CPMS)1211实验室支持可能2两次发作均累及+Differential

Diagnosis39急性播散性脑脊髓炎脑动脉炎、脑干炎、脊髓血管畸形颈椎病脊髓型热带痉挛性截瘫大脑淋巴瘤Treatment40目前尚无一种特效疗法，治疗的主要目的是：急性活动期抑制其炎症性脱髓鞘过程， 遏止病情的进展。尽量预防能促发的外因，减少复发次数，延长缓解间歇期。预防并发症。对症及支持疗法。1.

Relapsing-Remitting

MS：41①

Anti-inflammatory

treatment:methylprenisolone(high

dose

for

3d),prednison,

dexamethasone;②

Suppression

or

modulationof

the

immunesystem:

IFN-β1α

and

1b

;Azathioprine；Immuneglublin(Ig):0.4g/kg.dIVIg3-5d2.

ProgressiveMS：42①

Methotrexate,

MTX;②

Cyclosphoamide;③

Cyclosporine

A;④

Plasma

transplantation.3.

Symptomatic

treatment:43①

Spasticity:baclofen,dantrolene,diazepamandtizanidinecanbehelpful.②

Bladder

dysfunction:anticholinergicdrugs

urinary

catheter

may

berequired.预后分型良性型复发-缓解缓慢进展型慢性进展型44Examples45患者，女，32岁。主诉：行走不稳1年，左耳鸣、视物双影半年。走路不稳，踩棉花感左耳鸣复视快速细小水平眼震向右凝视时明显右侧指鼻试验、轮替试验、跟膝胫试验均欠佳Romberg征(+)，左Hoffmann征(+)四肢腱反射增高，以双下肢腱反射增高，右侧踝阵挛阳性头颅MRI未见异常46视神经和脊髓受累较多见，病灶中的软化、坏死较多见.视神经脊髓炎又称Devic病,为多发性硬化的一个亚型.中国,日本等东方人1.Introduction2.Etiology

And

Pathogenesis47视神经脊髓炎(Neuromyelitis

optica,

NMO)3.Patholgy主要侵犯视神经、视交叉、和脊髓胸颈段48Acute

stage:infiltration

ofinflammatory

cells.49Astrocyte

proliferitionClinical

Manifestations-NMO50年轻居多，21-41岁。特征：急性横贯性脊髓炎和双侧同时或相继出现的ON。70%可在数日内有截瘫。急性起病可在数小时或数日内单或双眼失明，眼眶痛。脊髓症状可横贯、不对称、或呈播散性；特征为快速进展的双下肢瘫，感觉脱失平面、括约肌障碍等，1/3病人有Lhermitte征、根痛。一、辅助检查51CSF细胞数增加，73%单相、82%复发。复发病人脊髓MRI88%出现纵向融合超过数个节段，钆强化和肿胀常见。二、鉴别诊断单纯球后神经炎MS表现为NMO临床模式。亚急性视神经病三、治疗大剂量甲强冲击疗法颈髓脱髓鞘52视神经炎急性播散性脑脊髓炎53(acute

disseminated

encephalomylitis,

ADEM)1.Introduction:

ADEM

is

a

kind

ofacuteinflammatory

disseminated

disease

involvedthewhiter

matter

of

brain

and

spinal

cord.(感染出疹或疫苗接种)爆发型：急性出血性白质脑炎(acute

necrotizing

hemorrhagic

encephalom