Peripheral Neuropathy

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Presentation Transcript

Peripheral Neuropathy:

Peripheral Neuropathy Dr Rahul Chakor Dept of Neurology

Slide 2:

PERIPHERAL NEUROPATHY - A GENERAL TERM Disorders affecting peripheral nerve cells and fibers Motor, Sensory or Autonomic fibers Small-fiber neuropathy (SFN) - involvement of autonomic and pain fibers Mononeuropathies - Focal involvement of a single nerve Mononeuritis multiplex syndrome - involvement of multiple nerves asymmetrically Generalized sensorimotor polyneuropathies Polyneuropathy syndrome Sensory-motor involvement in a relatively symmetric fashion distal-to-proximal gradient of involvement

Prevalence:

Prevalence Peripheral Nerve disorders - 2.4% Diabetic Sensorimotor polyneuropathy -1% Carpal tunnel syndrome - 3 to 5% Charcot-Marie Tooth disease, type 1a -approximately 30 per 100,000 population

Pathophysiology:

Pathophysiology Stereotype response to an insult or disease Wallerian degeneration- focal trauma, compression, infarction Axonal degeneration - most common, distal to proximal, dying back, metabolic Segmental demyelination - acquired inflammatory demyelination, AIDP

Pathological Events:

Pathological Events Wallerian degeneration

Pathological Events:

Pathological Events Axonal degeneration

Pathological Events:

Pathological Events Neuronopathy

Slide 8:

Saltatory conduction Demyelination 0.2 to 2 m/s 12 to 120 m/s

Symptoms:

Symptoms Polyneuropathy Bilateral symmetrical Distal Sensory Motor Autonomic Mononeuropathy Single nerve distribution Motor Sensory Mononeuritis multiplex Multiple nerve distribution Motor Sensory

Slide 10:

Positive Sensory Tingling Band like sensation Tightness Paresthesias - spontaneous Dysesthesias - on stimulation Allodynia - on non noxious stimulus Hyperalgesia - on noxious stimulus Hyperpathia Deep burning, cutting, gnawing, shooting, jabbing, electric like flashes, nocturnal and at rest Negative sensory Numbness- loss of sensation Cotton wool sensation Walking on ice Feels different Painless ulcerations Foot deformities

Slide 11:

Negative Motor Weakness in feet toe extensor and hands Tripping, Falls Difficulty in walking Wasting Positive Motor Cramps Fasciculations Tremor Myokimia

Slide 12:

Autonomic Lightheadedness Orthostatic hypotension Syncope Sweating disturbance Heat intolerance Autonomic Bladder bowel disturbance Erectile dysfunction Gastroparesis - nausea, early satiety, vomiting Constipation Diarrhoea

Slide 13:

Demyelination Weakness without wasting Early loss of reflexes Generalized arreflexia Proximal > distal weakness Preserved pain and temperature Axonopathy/ Dying back neuropathy/ Length dependent neuropathy Distal followed by proximal weakness & sensory loss Symmetric weakness and wasting Sensory loss in Stocking and glove distribution Absent ankle jerks

Axonopathies vs Demyelination:

Axonopathies vs Demyelination Axonoapathy Reduced amplitudes of SNAP, CMAP Conduction velocities, latencies normal EMG shows distal muscle denervation Recovery slow & incomplete (regeneration 2 - 3 mm/day) Common pathology Demyelination NCV reduced to < 70% of lower limit of normal Conduction block, delayed distal latencies Temporal dispersion Recovery better than axonopathies May coexist with axonopathies Few causes

Neuronopathies:

Neuronopathies Sensory Neuronopathy Pure sensory syndrome Loss of sensation, gross sensory ataxia Loss of pain, temperature sensation Arreflexia Paraneoplastic, autoimmune, pyridoxine toxicity, cisplatinum, HIV, idiopathic Motor Neuronopathy Pure motor syndrome Focal Weakness and wasting Fasciculations Loss of app reflexes Poliomyelitis, Motor neuron disease, inherited neuronopathies

Axonopathies :

Axonopathies Acute Toxins Axonal GBS Tick paralysis Critical illness neuropathy Subacute/Chronic Metabolic Toxic Hereditary CMT II Systemic disorders

Axonopathies:

Axonopathies Metabolic, Toxic Diabetes Renal failure/Uremia Deficiency states - B1 B6, B12, Niacin, Vit E, folic acid, PEM, Copper Hypothyroidism Alcohol Toxins - acrylamide, solvents, OPC compounds, Arsenic, Lead, Thallium

Axonopathies:

Medications Metronidazole - S, Ax Cisplatin - S, Ax Anti HIV - Stavudine, didanosine, lamivudine, Zalcitabine - S, Ax INH - SM, Ax Statins - SM, Ax Phenytoin - SM, Ax Amiodarone - SM Demyelination Pyridoxine - S, Ax, N Axonopathies

Demyelination/Myelinopathies :

Demyelination/Myelinopathies Acute AIDP Diptheria Chronic/Relapsing CIDP POEMS Multiple myeloma Paraproteinemia (MGUS) HIV Inherited CMT I, III, X Refsum disease Leukodystrophies

Motor neuropathies:

Motor neuropathies Pure motor neuropathies ( Normal SNAPs) MMN AMAN MND Predominantly Motor Neuropathies (SNAPs impaired) GBS CIDP Porphyric Neuropathy Lead intoxication POEM Syndrome Diabetic Lumbar plexopathy HMSN (Charcot -Marie-Tooth disease)

Small fiber neuropathies (Painful):

Small fiber neuropathies (Painful) Diabetes mellitus Idiopathic small fiber neuropathy HIV- distal symmetrical Amyloid neuropathy Uremic Alcohol Drugs -ART (Stavudine), cisplatin Hereditary - Fabrys disease, Tangier disease

Sensory ataxic neuropathies, Neuronopathies (Predominantly sensory):

Sensory ataxic neuropathies, Neuronopathies (Predominantly sensory) Malignant inflammatory ganglionopathy (Paraneoplastic) Sjogrens syndrome Idiopathic Cisplatinum Pyridoxine toxicity Miller fisher syndrome MGUS associated neuropathy

Autonomic Neuropathies:

Autonomic Neuropathies GBS/AIDP Diabetes Mellitus Porphyria Idiopathic acute panautonomic neuropathy Paraneoplastic HSAN Amyloidosis (familial & acquired) HIV related Vincristine, amiodarone, cisplatin, organic solvents, metals

Mononeuritis Multiplex:

Mononeuritis Multiplex Ax Vasculitis DM Sarcoidosis Leprosy HIV Myelinopathies MMN MADSAM HNPP Multiple pressure palsies (DM, Hypothyroidism)

Facial neuropathy:

Facial neuropathy GBS CIDP Leprosy HIV Sarcoidosis HSV, VZV Lyme disease

APPROACH:

APPROACH DISTRIBUTION OF WEAKNESS SINGLE NERVE MONONEUROPATHY ULNAR NERVE RADIAL NERVE MULTIPLE NERVES MONONEURITIS MULTIPLEX SLIDE 25 SYMMETRIC DISTAL > PROXIMAL AXONOPATHY SLIDES 16 TO 18 SYMMETRIC PROXIMAL > DISTAL MYELINOPATHY ACUTE CHRONIC AIDP SLIDE 19 CIDP SLIDE 19 PRESSURE, STRETCH INJURY

Slide 28:

3 Goals Where is the lesion Etiology Therary 6 Questions 1) Systems involved Motor, Sensory, Autonomic, Mixed 2) Distribution Distal or Proximal & Distal Focal/Asymmetric or Symmetric 3) Sensory involvement Pain, burning, stabbing or proprioceptive loss 4) ? UMN involvement With or without sensory loss 5) Temporal profile Acute, Subacute, Chronic 6) Family history Sensory signs without symptoms 3 -6- 9 APPROACH TO PERIPHERAL NEUROPATHY 9 Phenotypic Patterns

Pattern recognition approach of Peripheral Neuropathy:

Pattern recognition approach of Peripheral Neuropathy 1) Symmetric proximal and distal weakness with sensory loss - Myelinopathies (slide 19) 2) Symmetric distal weakness with sensory loss - Metabolic, drugs, toxins, HMSN, amyloidosis 3) Asymmetric distal weakness with sensory loss - Multiple nerves (vasculitis, HNPP, leprosy, sarcoid, HIV). Single nerve (compression, radiculopathy) 4) Asymmetric distal weakness without sensory loss - MMNCB, MND (with UMN signs) 5) Asymmetric proximal and distal weakness with sensory loss - plexopathy, HNPP, idiopathic, meningial carcinomatosis) 6) Symmetric sensory loss without weakness -Metabolic (diabetes), cryptogenic, drugs, toxins 7) Symmetric distal sensory loss with UMN signs - B12 deficiency and SCD 8) Proprioceptive loss without weakness - ganglionopathy (slide 23) 9) Autonomic neuropathy - (slide 24)

Thank you:

Thank you