NEUROPATHY

ALDY S. RAMBE DEPARTEMEN NEUROLOGI FK USU/ RSUP H. ADAM MALIK MEDAN

DEFINITION Neuropathy is defined as a disease or i j injury of f th the peripheral i h l sensory, motor, t or autonomic nerves. Can be : - pure motor - pure sensory - mixed sensorimotor - autonomic t i

Category
Usually categorized separately : N Neuronopathy th : selective l ti i injury j to t the th cell body of the axon Radiculopathy : selective injury to the nerve roots distal to their origin Plexopathy : injury to the brachial or p lumbosacral plexus

CLASSIFICATION
1. BASED ON THE ONSET OF NEUROPATHY: ACUTE NEUROPATHY eg. : ACUTE IDIOPATHIC POLYNEUROPATHY CHRONIC NEUROPTHY

eg.

: BERI BERI DIABETES MELLITUS LEPROSY

2. BASED ON SEVERITY
1. MILD NEUROPATHY : SENSORY ONLY 2. MODERATE NEUROPATHY : SENSORY MOTOR SENSORY, MOTOR, AND DECREASE OF TENDON REFLEXES 3. SEVERE NEUROPATHY : SENSORY, MOTOR, DECREASE OF TENDON REFLEXES, MUSCLE ATROPHY

3.

BASED ON THE NUMBER OF NERVES INVOLVED

1. MONONEUROPATHY SIMPLEX :
ONLY ONE PHERIPHERAL NERVE INVOLVED. 2. MONONEUROPATHY MULTIPLEX (MULTIFOCAL NEURPATHY) ): MULTIPE, SCATTERED NERVES IN AN IRREGULAR DISTRIBUTION 3. POLYNEUROPATHY : SEVERAL NERVES INVOLVED, SYMMETRICAL, SAME ONSET AND DISTAL PREDOMINANT.

4. BASED ON LESION SITE

1 DISTAL AXONOPATHY : AXONAL LESION 2. MYELINOPATHY : DISORDER OF MYELIN SHEATH. 3. NEUROPATHY : DISORDER OF CELL BODY AT ANTERIOR HORN CELLS, SPINAL CORD OR DORSAL ROOT GANGLION.

ETIOLOGY
1. IDIOPATHIC INFLAMMATORY NEUROPATHIES
ACUTE IDIOPATHIC POLYNEUROPATHY (GUILLAIN BARRE SYNDROME) - CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY

2. METABOLIC AND NUTRITIONAL NEUROPATHIES - DIABETES, , HYPOTHYROIDI, , ACROMEGALY - UREMIA - LIVER DISEASES - VIT B1, OR VIT B12 DEFICIENCY

ETIOLOGY
3. INFECTIVE AND GRANULOMATOUS NEUROPATHIES: AIDS, LEPROSY. DIPHTHERY, SARCOIDOSIS 4. VASCULITIS NEUROPATHIES: - POLYARTERITIS NODOSA - RHEUMATOID ARTHRITIS - SYSTEMIC LUPUS ERYTHEMATOSUS

ETIOLOGY
5. NEOPLASTIC AND PARAPROTEINEMIC NEUROPATHIES: - COMPRESSION AND IRITATION BY TUMOR - PARANEOPLASTIC SYNDROME - PARAPROTEINEMIAS - AMYLOIDOSIS

ETIOLOGY
6. DRUGS INDUCED AND TOXIC NEUROPATHIES - DAPSONE, ISONIAZIDE, PHENYTOIN, PIRIDOXYNE, VINCRISTIN, HIDRALAZINE. - ALCOHOL - TOXINS : ORGANOPHOSPHAT ARSENIC LEAD THALIUM GOLD

ETIOLOGY (cont.d)
7. HEREDITARY NEUROPATHIES - IDIOPATHIC
HEREDITARY MOTOR AND SENSORY NEUROPATHIES HEREDITARY SENSORY NEUROPATHIES FAMILIAL AMYLOIDOSIS

- METABOLIC
PORPHYRIA METACHROMATIC LEUCODYSTROPHY ABETALIPOPROTEINEMIA

ETIOLOGY
8. ENTRAPMENT NEUROPATHIES - UPPER LIMBS
MEDIAN NERVE (CARPAL TUNNEL SYNDROME) ULNAR NERVE RADIAL NERVE

- LOWER LIMBS
PERONEAL NERVE FEMORAL NERVE OBTURATOR NERVE

PATHOGENESIS
Can be divided into 4 major categories : 1. Neuronal degeneration : results from damage to the motor or sensory nerve cell ll bodies, b di with i h subsequent b degeneration d i 2. Wallerian degeneration : results from damage to the axon at a specific point below the cell body, with degeneration distal t th to the i injury. j 3. Axonal degeneration : results from diffuse axonal damage. The distal portion undergoes the earliest and most severe change h followed f ll d by b gradual d l proximal i l ascent t with ith continued ti d injury (dying back phenomenon) 4. Segmental demyelination : results from injury to the myelin sheath h th without ith t injury i j to t the th axon

PATHOPHYSIOLOGY

1 NEUROPRAXIS : 1.
- the mildest form - conduction disruption only - intact nerve continuity y - recovery in minutes or weeks

PATHOPHYSIOLOGY
2. AXONOTMESIS: - AXONAL DAMAGE FOLLOWED BY DEGENERATION - ENDONEURAL SHEATH REMAINS INTACT - POSSIBLE REGENERATION

PATHOPHYSIOLOGY
3. NEUROTMESIS: - PARTIAL OR TOTAL NERVE DAMAGE - SURGICAL INTERVENTION IS NEEDED - 50% RECOVER

CLINICAL SYMPTOMS
1. SENSORY SYMPTOMS :
Involvement of sensory axons produces impairment of sensation with dysesthesias or paresthesias.

CLINICAL SYMPTOMS
2. MOTOR SYMPTOMS :
Involvement of motor axons produces muscle wasting and weakness followed by atrophy and fasciculations
- LMN TYPE MUSCLE WEAKNESS - FOOT DROP - WRIST DROP

CLINICAL SYMPTOMS
3. CHANGE OF TENDON REFLEXES The tendon reflexes supplied by the
affected nerve are depressed or absent.

decreased or absent of tendon reflexes

CLINICAL SYMPTOMS
4. AUTONOMIC :
Involvement of axons supplying autonomic function produces loss of sweating, alteration in bladder fuction, constipation, and impotence in male

DIAGNOSIS
1. 2. 3. 4. 5 5. 6. CLINICAL SYMPTOMS AND SIGNS LABORATORY STUDIES CHEST X-RAY LP ECG BIOPSY : sural nerve or radial cutaneus nerve

7. ELECTROPHYSIOLOGY: EMG NCV

DIABETIC NEUROPATHY
Neuropati diabetik : adanya gejala dan atau tanda disfungsi saraf perifer pd orang dgn diabetes setelah dieksklusikan penyebab lain. lain Prevalence : 10 - 20 % (symptomatic)

Diabetic Neuropathy : 50% of diabetic patients type 1 than type 2 the most common : chronic sensorimotor 50% asymptomatic 10-20% needs specific treatment

PATHOGENESIS The etiology is uncertain.

4 hypothesis (not necessarily exclusive) : 1 Hyperglycemia-polyol-myoinositol 1. Hyperglycemia polyol myoinositol hypothesis. 2. Microvascular hypothesis 3. Structural changes at the node of Ranvier. 4. Vasculitic neuropathy.

DIABETIC NEUROPATHIC SYNDROMES

DISTAL SYMMETRIC NEUROPATHY : - large fiber sensory neuropathy numbness, paresthesias, dysesthesias, hyperesthesias ataxia hyperesthesias, ataxia. - sensorimotor neuropathy any of the above plus distal weakness

DIABETIC NEUROPATHIC SYNDROMES

Small Fiber Neuropathy : - pure small fiber neuropathy numbness, paresthesias, painful dysesthesias, hyperesthesias. - Diabetic neuropathy cachexia subacute, severe neuropathic pain, rapid weight loss - Autonomic neuropathy erectile dysfunction, orthostasis, cardiac dysrhythmia, y y , diarrhea, , constipation p

DIABETIC NEUROPATHIC SYNDROMES

Ischemic Mononeuropathy. - cranial (eg. CNs III, VI,VII) diplopia, pupil-sparing third nerve palsy, hemifacial weakness - Radicular (thoracic, (thoracic lumbosacral) pain, followed by numbness or weakness in a radicular distribution - Peripheral (eg. Femoral) pain, followed by numbness, weakness or both in territory of a single nerve

DIABETIC NEUROPATHIC SYNDROMES

Regional Neuropathic Syndromes. - Diabetic amyotrophy subacute weakness and atrophy of proximal leg muscles - Diabetic thoracoabdominal neuropathy. subacute weakness, weakness numbness numbness, and atrophy in thorax and abdomen

DIAGNOSIS

THERAPY
Intensive diabetic therapy M i t i ideal Maintain id l body b d weight i ht Adjuvant analgetics : TCA antidepressants carbamazepine gabapentin i t intravenous lid lidocaine, i etc t

Adjuvant Analgetics

CARPAL TUNNEL SYNDROME

80% in WOMEN, A COMMON TEMPORARY PHENOMENON DURING PREGNANCY PRESSURE TO THE NERVE WHEN PASSING BENEATH THE FLEXOR RETINACULUM OBSTRUCTION OF VENOUS CIRCULATION AND EDEMA ISCHEMIA INCREASING PRESSURE ON THE NERVE ISCHEMIC ATROPHY OF NERVE FIBERS

Etiology
1. Hereditary : HMSN type III 2. Traumatic : dislocation, fracture, hematoma, wrist sprain 3. Infection : tenosynovitis, tbc, sarcoidosis 4. Metabolic : amyloidosis, gout 5 Endocrine : acromegaly 5. acromegaly, DM DM, hypothyroidism hypothyroidism, pregnancy 6. Neoplastic : ganglion cysts, lipoma , myeloma 7 Collagen vascular diseases : RA, 7. RA polymyalgia rheumatica, SLE 8. Degenerative disease : OA 9. Iatrogenic : radial artery puncture, shunt for dialysis, anticoagulant therapy

Clinical Symptoms The earliest symptoms : numbness and paresthesias in the sensory distribution of the median nerve in the hand (thumb, ( , index, , middle and lateral half of the ring finger) Later on : p pain, , worst at night g Late : inability to screw bottle caps or grip g p properly p p y

SIMPLE CLINICAL TESTS FOR CTS

PHALENS SIGN (PHALENS MANEUVRE): is present when tingling (paresthesia) is experienced in the distribution of the median nerve when the wrist is held in forced flexion (90o for 30-60 seconds) TINELS SIGN (HOFFMANN-TINELS SIGN) : is present when tingling (paresthesia)is experienced when tapping lightly with a finger or a tendon hammer over a compressed or regenerating peripheral nerve. The tingling is present in the dist ib tion of the damaged nerve. distribution ne e

Therapy
Identified causes should be treated Corticosteroid injection around the median nerve in the carpal tunnel. Surgical division of the transverse ligament (flexor retinaculum) Endoscopic carpal tunnel release

GUILLAIN - BARRE SYNDROME (GBS) ( )

ESSENTIALS of DIAGNOSIS :

Rapidly progressive paralysis, often ascending di Areflexia Increased CSF protein without increased cell count (albuminocytologic dissociation) Evidence of demyelination on nerve conduction d ti studies t di ( (may b be d delayed) l d) A neurologic emergency that may rapidly progress to respiratory compromise

GBS : Subtypes and Clinical Findings

Acute inflammatory demyelinating polyneuropathy (AIDP) : - ascending paralysis - minor sensory symptoms - nonspecific antibody (Ab) - EMG/NCV : demyelination on NCS NCS, absent F waves

GBS : Subtypes and Clinical Findings

Acute motor axonal neuropathy (AMAN) : - flaccid paralysis - often with Campylobacter jejuni infection - IgG anti anti-GM1, GM1 IgG anti anti-GD1a GD1a - EMG/NCV : reduced motor amplitudes normal l sensory studies t di

GBS : Subtypes and Clinical Findings

Acute motor sensory axonal neuropathy (AMSAN) ( ): - acute (< 1 week) - profound quadriparesis - ventilation often required - IgG anti-GM1
-EMG/NCV : reduced or absent motor amplitudes reduced or absent sensory amplitudes axonal injury by EMG

GBS : Subtypes and Clinical Findings

Miller Fisher syndrome : - ataxia - areflexia - opthalmoplegia - IgG anti-GQ1b - EMG/NCV : decreased d d sensory nerve AP motor conductions often normal

GBS : SYMPTOMS AND SIGNS

AIDP often begins 1-3 weeks after an infection or inciting event such as surgery 70% cases initially have paresthesias or vague numbness in their hands and feet. Symmetric weakness appears a few days later and progress over days to a few weeks weeks. Paralysis is maximal by about 2 weeks in> 50% cases, and by 1 month in > 90% cases. Ascending weakness beginning in the distal legs is typical, although descending paralysis with predominant proximal muscles weakness rarely appears. appears

GBS : SYMPTOMS AND SIGNS

Facial weakness occurs in half of patients with AIDP and ophthalmoparesis and lower cranial nerves neuropathies thi can cause dysarthriaand d th i d dysphagia. d h i AIDP require mechanical ventilation Weakness is symmetric, and ranges from mild to severe flaccid quadriparesis. Sensation is usually normal, despite sensory symptoms although mild distal vibratory loss may be symptoms, found Reflexes are diminished or absent Sphincter tone is normal

GBS : DIAGNOSTIC STUDIES Imaging studies of the spinal cord to rule out myelopathic disease. disease LP (after spinal cord disease excluded) Evaluation for infection ECG Chest radiographs EMG/NCV

GBS : DIFFERENTIAL DIAGNOSIS

Acute spinal cord disease (acute myelopathy, transverse myelitis) Brainstem ischemia Acute disorders of neuromuscular junction (myasthenia gravis, botulinum intoxication) Acute neuropathies (porphiric neuropathy, diphtheritic neuropathy, mononeuropathy multiplex, toxic neuropathy)

THERAPY
PLASMAPHARESIS (5-6 exchanges over 1- 2 weeks) ) or IMMUNOGLOBULIN IV (0,4 g/kg/day for 5 days) Equally effective when given within the first 2 weeks after onset Combination of both no additional benefit RCTs on oral or IV corticosteroid failed to show benefit