慢性炎症性脱髓鞘性多发性神经根神经病最新进展PPT课件
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length dependent axonal neuropathy.
DML: distal motor latency
.
10
The Lewis–Sumner syndrome (LSS), or multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), has been reported in up to 15% of patients fulfilling the EFNS/PNS criteria for CIDP
severe autonomic dysfunction should be regarded as a red flag when considering the diagnosis of CIDP.
.
5CIDP PHENOTYPSThe clinical presentation of CIDP is variable
.
7
Atypical CIDP
.
8
Distal paresthesia and hypesthesia are the most frequent symptoms, followed by proprioceptive ataxia
.
9
DADSP(distal acquired demyelinating symmetric polyneuropathy ) : distal or predominantly sensory large fibre neuropathy
The disease evolves over more than 8 weeks, thus distinguishing the condition from GBS which has an acute onset.
The time course maybe relapsing, chronic progressive, monophasic or GBS like onset.
.
2
prevalence of around 6 cases per 100,000 predominantly affect males, and typically occur in middle
to old age CIDP can also occur in children. The incidence of CIDP increases with age, rising to 1.5
times the overall average in people over 65 years of age
.
3
Clinical presentation
Classic CIDP is characterized by the occurrence of symmetrical weakness in both proximal and distal muscles, impaired sensation and parasthesia and absent or diminished tendon reflexes
Fatigue can be the main complaint in CIDP patients(75%)
Activity-induced weakness
Severe pain is present in only a minority of CIDP patients;
Tremor can be a disabling symptom in CIDP (50%)
Department of Neurology, Fujian Provincial Hospital Xingyong Chen
.
1
Introduction
Traditionally, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been considered a heterogeneous disorder including a broad spectrum of clinical phenotypes.
determined by the number and distribution of the demyelinating peripheral nerve lesions
.
6
SM: sensory and motor. PE: plasma exchange; RR:relapsing–remitting; CS:corticosteroids;
The European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) CIDP treatment guideline has defined several clinical pictures as atypical CIDP phenotypes, additional to the classical (typical) picture of CIDP
a higher incidence of almost 40% of restless legs syndrome in patients with CIDP
Autonomic symptoms : 23%, of which gastrointestinal and genitourinary symptoms were most frequent (mild)
.
4
Newly recognised clinical features in CIDP
Acute onset of CIDP may occur in up to 18% of CIDP patients, resembling the GBS
diagnosis was changed to CIDP in 5% of GBS patients
DML: distal motor latency
.
10
The Lewis–Sumner syndrome (LSS), or multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), has been reported in up to 15% of patients fulfilling the EFNS/PNS criteria for CIDP
severe autonomic dysfunction should be regarded as a red flag when considering the diagnosis of CIDP.
.
5CIDP PHENOTYPSThe clinical presentation of CIDP is variable
.
7
Atypical CIDP
.
8
Distal paresthesia and hypesthesia are the most frequent symptoms, followed by proprioceptive ataxia
.
9
DADSP(distal acquired demyelinating symmetric polyneuropathy ) : distal or predominantly sensory large fibre neuropathy
The disease evolves over more than 8 weeks, thus distinguishing the condition from GBS which has an acute onset.
The time course maybe relapsing, chronic progressive, monophasic or GBS like onset.
.
2
prevalence of around 6 cases per 100,000 predominantly affect males, and typically occur in middle
to old age CIDP can also occur in children. The incidence of CIDP increases with age, rising to 1.5
times the overall average in people over 65 years of age
.
3
Clinical presentation
Classic CIDP is characterized by the occurrence of symmetrical weakness in both proximal and distal muscles, impaired sensation and parasthesia and absent or diminished tendon reflexes
Fatigue can be the main complaint in CIDP patients(75%)
Activity-induced weakness
Severe pain is present in only a minority of CIDP patients;
Tremor can be a disabling symptom in CIDP (50%)
Department of Neurology, Fujian Provincial Hospital Xingyong Chen
.
1
Introduction
Traditionally, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been considered a heterogeneous disorder including a broad spectrum of clinical phenotypes.
determined by the number and distribution of the demyelinating peripheral nerve lesions
.
6
SM: sensory and motor. PE: plasma exchange; RR:relapsing–remitting; CS:corticosteroids;
The European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) CIDP treatment guideline has defined several clinical pictures as atypical CIDP phenotypes, additional to the classical (typical) picture of CIDP
a higher incidence of almost 40% of restless legs syndrome in patients with CIDP
Autonomic symptoms : 23%, of which gastrointestinal and genitourinary symptoms were most frequent (mild)
.
4
Newly recognised clinical features in CIDP
Acute onset of CIDP may occur in up to 18% of CIDP patients, resembling the GBS
diagnosis was changed to CIDP in 5% of GBS patients