2. Peripheral Neuropathy.ppt
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Peripheral Neuropathy Zhongjie Chen Department of Neurology, The affiliated Zhongshan hospital, Xiamen University 1 Peripheral Neuropathy Outline rigeminal neuralgia Idiopathic facial palsy Guillain-Barre Syndrome...
Peripheral Neuropathy Zhongjie Chen Department of Neurology, The affiliated Zhongshan hospital, Xiamen University 1 Peripheral Neuropathy Outline rigeminal neuralgia Idiopathic facial palsy Guillain-Barre Syndrome 2 OUTLINE Anatomy Pathological Processes Clinical classification Symptoms Investigation 3 Central Nervous System: Brain Spinal Cord Peripheral Nervous System: Cranial nerve (exclusion of optic and olfactory nerve ) Spinal nerve Autonomic nerve nerve root ， nerve plexus ， nerve bundles ， nerve cord and terminal branch 4 5 6 Anatomy Peripheral nerves are made up of numerous axons bound together by three types of connective tissue— endoneurium, perineurium, epineurium. The vessel located in the epineurium provides the blood supply. Peripheral nerve trunks contain myelinated and unmyelinated fibres 7 vessel unmyelinated myelinated fat fibres fibres endoneurium perineurium epineurium Peripheral composite nerve 8 Pathological Processes Wallerian degeneration (injury,cut off) Axonal degeneration (toxicosis , metabolic ) Neuronal degeneration (poliomyelitis) Segmental demyelination (GBS 、 diphtheria) 9 Wallerian Axonal Segmental Neuronal degeneration demyelination Neuro soma nucleus axon myelin sheath Ranvier node Schwann cell motor end plate muscle Normal 10 Symptoms Sensory disturbance Motor deficits Tendon reflexes (lower or disappear) Autonomic disturbances Others(ataxia,deformity) 11 Investigation Nerve conduction velocity(NCV) Electromyography(EMG) Blood tests CSF examination Nerve biopsy 12 Trigeminal Neuralgia 13 Trigeminal Nerve 14 15 Nuclei Trigeminal Nerve 16 Etiology and Pathology A facial pain syndrome of unknown cause. Demyelination 17 18 Clinical Features It develops in middle to late life. It consists of severe paroxysms electric-shock-like pain(or prickling 、 cutting 、 bursting-like ） usually in the V3 and V2 division of the trigeminal nerve lasting for several seconds or minutes each time. Involvement of V1 division or bilateral disease occurs in less than 5% of cases. 19 Clinical Features Occurrence during sleep is rare. Painfree intervals may last for minutes to weeks, but long-term spontaneous remission is rare. Sensory stimulation of trigger zones about the cheek, nose, or mouth by touch, cold, wind, talking, or chewing can precipitate the pain. 20 · trigger zones 21 Clinical Features Tic douloureux Course could be periodic Physical examination is normal 22 Diagnosis and Differential Diagnosis Diagnosis depending on the clinical features ， no positive sign. Differential Diagnosis Secondary trigeminal neuralgia Tooth ache Glosspharyngeal neuralgia root of tongue ， soft palate 23 Treatment Drug is preferential AEDs: carbamazepine phenytoin, clonazepam Baclofen VitaminB12 Nerve block :absolute alcohol , glycerol surgery 24 Idiopathic Facial Palsy (Bell’s palsy) 25 Idiopathic Facial Palsy (Bell’s palsy) Anatomy Etiology and pathogenesis Clinical features Diagnosis and differential diagnosis Treatment 26 Anatomy Facial nerve is composite nerve consists of ： motor fibers sensory fibers parasympathetic fibers 27 28 29 30 Etiology and Pathology Uncertain etiology cold 、 virus infection, unstable of autonomic nerves contraction of nutrient vessel, expansion of capillary vessels tissue edema oppression of facial nerve facial nerve edema and demyelination ， axonal degeneration 。 31 Clinical features Onset □ Occurs in any age, usually unilateral. □ Paralysis: Progresses over 3 to 72 hours □ Pain (50%): Near mastoid process □ Excess tearing (33%) □ Other: Hyperacusis; Dysgeusia 32 Clinical features Signs □ Facial weakness Upper & Lower Unilateral Degree: Partial (30%); Complete (70%) □Stapedius dysfunction (33%): Hyperacusis □ Lacrimation: mildly affected in some patients 33 Bell sign 34 □ Taste: No clinically significant changes in most patients. □ There should be no sensory loss in the face. □ Hunt syndrome. geniculate ganglionitis caused by herpes zoster ； mastoid process pain ； external auditory canal herpes and hypesthesia 。 □ No abnormalities beyond the territory of the facial nerve. 35 Clinical features Prognosis Depend on the amplitude of CMAP Decreased ＜ 70% may recovery in 2 month Decreased 70% ～ 90% 2 ～ 8month Decreased ＞ 90% 0.5 ～ 1 year （ compared with the normal side ） 36 Clinical features Prognosis if denervation of facial nerve present in 10 days after onset ， recovery time will be prolonged,the average recovery time is 3 month 。 37 Clinical features Prognosis better □ Incomplete paralysis □ Early improvement □ Slow progression □ Younger age 38 Clinical features Prognosis better □ Normal taste □ Electrodiagnostic tests normal 39 Diagnosis and differential diagnosis Diagnosis ： depend on acute onset of peripheral facial palsy Differential diagnosis 1 Central facial palsy. 40 Quiet Frown and show teeth Close eyes Peripheral palsy Centre palsy 41 Diagnosis and differential diagnosis Differential diagnosis 2 GBS 3 Various kinds of aural facial palsy mastoiditis ， tympanitis 4 peripheral facial palsy caused by tumor in posterior cranial fossa or meningitis 42 Treatment Principle ： improve local blood circulation ， alleviate edema of facial nerve ， prompt function recovery 。 Corticosteroids: prednisone or dexamethason VitaminB1,B12 Baclofen Rehabilitation Physiotherapy 43 AIDP (acute inflammatory demyelinating polyneuropathies,) 44 Definition AIDP =Guillain-Barré Syndrome （ GBS ） 45 Guillain Barre 46 Definition GBS ： immunological mediated peripheral neuropathy characterized pathologically by demyelination of peripheral nerve and nerve root ， inflammatory reaction of lymphocytes and macrophage around small vessel 。 47 Epidemiology Incidence: 0.6 to 1.9/100,000/year Male: Female = 1.25: 1 Peak ages: 16~25 years old 45~60 years old Certain forms of GBS appear to occur more frequently in certain areas of China. 48 Etiology and pathogenesis The precise cause is unclear. GBS often follows minor infective illness, inoculations or surgical procedures. Clinical and epidemiologic evidence suggest an association with preceding Campylobacter Jejuni(CJ) infection. The pathogenesis resembles EAN （ experimental allergy neuritis ） Molecular mimicry 49 Campylobacter Jejuni(CJ) 50 Clinical features GBS Prodrome It often follows 1-4 weeks after a respiratory infection or diarrhea. Campylobacter jejuni(CJ) has been particularly implicated as a cause of the diarrhea. 51 Clinical features Weakness: Most often symptomatic in legs Distribution: Proximal + Distal; Symmetric Severity: Quadriplegia in 30%; Bedbound another 30% Respiratory failure. 52 Clinical features Sensory: usually less marked than motor symptoms. Paraesthesias: Initial symptom in 50%; Eventually occur in 70% to 90% Pain Loss: with classic glove-and-stocking pattern of sensory loss, but rarely occurs. 53 Clinical features Cranial nerve: Ⅶ, Ⅸ,Ⅹ facial weakness is present in 50% of cases. Autonomic dysfunction tachycardia, cardiac irregularitis, labile blood presure, disturbed sweating and so on. Monophase course 54 Clinical features Clinical classification AIDP AMAN （ acute motor axonal neuropathy ） AMSAN （ acute motor sensory axonal neuropath y） 55 Clinical features Clinical classification Fisher syndrome ophthalmoplegia +ataxia +tendon reflex disappears Unclassifiable GBS 56 Investigations CSF: a characteristic abnormality, with increased protein concentration but a normal cell count. Eletrophysiologic studies marked slowing of motor and sensory condu- ction velocity, evidence of denervation and axonal loss. F wave reflex is delayed or absent. Nerve biopsy: demyelination 57 Diagnostic criteria for GBS(1) Required for diagnosis Progressive weakness of more than one limb. Distal areflexia with proximal areflexia or hyporeflexia. 58 Diagnostic criteria for GBS(2) Supportive of diagnosis Progression for up to 4 weeks. Relatively symmetric deficits. Mild sensory involvement. Cranial nerve(especiallyⅦ)involvement. Recovery beginning within 4 weeks after progression stops. Autonomic dysfunction. No fever at onset. Increased CSF protein after 1 week. CSF white blood cell count10/l. Nerve conduction slowing or block by several weeks. 59 Differential diagnosis Hypokalemic periodic paralysis (Hopp) Poliomyelitis fever ， no sensory abnormality ， CSF cell count rise Myasthenia gravis(MG) 60 Treatment Assisting respiration: Patients who are severely affected are best managed in ICU where facilities are available for monitoring and assisted respiration if necessary. Sometimes antibiotic is necessary for preventing respiratory tract’s infection. Symptomatic therapy: The aim is to prevent such complications as respiratory failure or vascular collapse. 61 Treatment Preventing complications: hypostatic pneumonia bedsore Deep Vein Thrombosis 、 pulmonary embolism limbs contracture 、 deformity swallow paralysis retention of urine pain anxiety and depression 62 Treatment Etiological therapy: Plasma exchange(plasmapheresis) Intravenous immunoglobulin: 0.4g /kg /d for 5 days Corticosteroids: it has not been successful in acute GBS and can bring about adverse outcome. Rehabilitation 63 Prognosis The disorder is self-limiting,and improvement occurs over the weeks or months following onset. About 70-75% of patients recover completely, 25% are left with mild neurologic deficits, and 5% die, usually as a result of respiratory failure. The prognosis is poorer when there is evidence of preceding CJ infection. 64 Question 1. What is the clinical feature and treatment principle of Idiopathic Facial Palsy ？ 2. What is the diagnostic criteria 、 differential diagnosis and treatment principle of GBS ？ 65
