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NEUROPATHY
ALDY S. RAMBEDEPARTEMEN NEUROLOGI FK USU/DEPARTEMEN NEUROLOGI FK USU/
RSUP H. ADAM MALIK MEDAN
DEFINITION
Neuropathy is defined as a disease ori j f th i h l tinjury of the peripheral sensory, motor, or autonomic nerves.Can be : - pure motor
- pure sensorypure sensory- mixed sensorimotor
t i- autonomic
CategoryUsually categorized separately :
N th l ti i j t thNeuronopathy : selective injury to the cell body of the axonRadiculopathy : selective injury to the nerve roots distal to their originPlexopathy : injury to the brachial or lumbosacral plexusp
CLASSIFICATION
1. BASED ON THE ONSET OF NEUROPATHY:
ACUTE NEUROPATHYeg. : ACUTE IDIOPATHIC POLYNEUROPATHYCHRONIC NEUROPTHYeg. : BERI BERI
DIABETES MELLITUSLEPROSY
2. BASED ON SEVERITY
1. MILD NEUROPATHY :SENSORY ONLY
2. MODERATE NEUROPATHY :SENSORY MOTOR AND DECREASE OFSENSORY, MOTOR, AND DECREASE OF TENDON REFLEXES
3. SEVERE NEUROPATHY :3. SEVERE NEUROPATHY :SENSORY, MOTOR, DECREASE OF TENDON REFLEXES, MUSCLE ATROPHY
3. BASED ON THE NUMBER OF NERVES INVOLVEDNERVES 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.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.
ETIOLOGY1. 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 DEFICIENCYVIT B1, OR VIT B12 DEFICIENCY
ETIOLOGY
3. INFECTIVE AND GRANULOMATOUS NEUROPATHIES:AIDS, LEPROSY. DIPHTHERY, SARCOIDOSIS
4. VASCULITIS NEUROPATHIES:POLYARTERITIS NODOSA- POLYARTERITIS NODOSA
- RHEUMATOID ARTHRITIS- SYSTEMIC LUPUS ERYTHEMATOSUSSYSTEMIC 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- TOXINS : ORGANOPHOSPHAT
ARSENICLEADTHALIUMGOLD
ETIOLOGY (cont.d)
7. HEREDITARY NEUROPATHIES- IDIOPATHIC
HEREDITARY MOTOR AND SENSORY NEUROPATHIESHEREDITARY SENSORY NEUROPATHIESFAMILIAL AMYLOIDOSIS
- METABOLICPORPHYRIAPORPHYRIAMETACHROMATIC LEUCODYSTROPHYABETALIPOPROTEINEMIA
ETIOLOGY
8. ENTRAPMENT NEUROPATHIES- UPPER LIMBS
MEDIAN NERVE (CARPAL TUNNEL SYNDROME)ULNAR NERVERADIAL NERVE
- LOWER LIMBSPERONEAL NERVEPERONEAL NERVEFEMORAL NERVEOBTURATOR NERVE
PATHOGENESISCan be divided into 4 major categories :1. Neuronal degeneration : results from damage to the motor or
ll b di i h b d isensory nerve cell bodies, with subsequent degeneration2. Wallerian degeneration : results from damage to the axon at
a specific point below the cell body, with degeneration distal t th i jto the injury.
3. Axonal degeneration : results from diffuse axonal damage. The distal portion undergoes the earliest and most severe h f ll d b d l i l t ith ti dchange followed by gradual proximal ascent with continued
injury (dying back phenomenon)4. Segmental demyelination : results from injury to the myelin
h th ith t i j t thsheath without injury to the axon
PATHOPHYSIOLOGY
1 NEUROPRAXIS1. NEUROPRAXIS :- the mildest form- conduction disruption only- intact nerve continuityy- recovery in minutes or weeks
PATHOPHYSIOLOGY
2. AXONOTMESIS:AXONAL DAMAGE FOLLOWED BY- AXONAL DAMAGE FOLLOWED BY DEGENERATION
- ENDONEURAL SHEATH REMAINS INTACTINTACT
- POSSIBLE REGENERATION
PATHOPHYSIOLOGY
3. NEUROTMESIS:
- PARTIAL OR TOTAL NERVE DAMAGESURGICAL INTERVENTION IS NEEDED- SURGICAL INTERVENTION IS NEEDED
- 50% RECOVER
CLINICAL SYMPTOMS
1. SENSORY SYMPTOMS :Involvement of sensory axons producesInvolvement of sensory axons produces impairment of sensation with dysesthesias or paresthesias.
CLINICAL SYMPTOMS
2. MOTOR SYMPTOMS :Involvement of motor axons produces muscleInvolvement of motor axons produces muscle wasting and weakness followed by atrophy and fasciculations
- LMN TYPE MUSCLE WEAKNESS- FOOT DROP- WRIST DROPWRIST DROP
CLINICAL SYMPTOMS
3. CHANGE OF TENDON REFLEXESThe tendon reflexes supplied by theThe tendon reflexes supplied by the affected nerve are depressed or absent.
decreased or absent of tendon reflexesdecreased or absent of tendon reflexes
CLINICAL SYMPTOMS
4. AUTONOMIC :Involvement of axons supplying autonomicInvolvement of axons supplying autonomic function produces loss of sweating, alteration in bladder fuction, constipation, and impotencein bladder fuction, constipation, and impotence
in male
DIAGNOSIS
1. CLINICAL SYMPTOMS AND SIGNS2. LABORATORY STUDIES3. CHEST X-RAY4. LP5 ECG5. ECG6. BIOPSY : sural nerve or radial cutaneus nerve
7. ELECTROPHYSIOLOGY: EMGNCV
DIABETIC NEUROPATHYNeuropati diabetik :adanya gejala dan atau tanda disfungsi saraf perifer pd orang dgn diabetes setelah dieksklusikan penyebab lain orang dgn diabetes setelah dieksklusikan penyebab lain.
Prevalence : 10 - 20 % (symptomatic)Diabetic Neuropathy :Diabetic Neuropathy :
▫ 50% of diabetic patients ▫ type 1 than type 2 ▫ the most common : chronic sensorimotor 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-myoinositol1. Hyperglycemia polyol myoinositol hypothesis.2. Microvascular hypothesis3. Structural changes at the node of Ranvier.4. Vasculitic neuropathy.
DIABETIC NEUROPATHIC SYNDROMES
DISTAL SYMMETRIC NEUROPATHY :- large fiber sensory neuropathy- large fiber sensory neuropathy
numbness, paresthesias, dysesthesias, hyperesthesias ataxiahyperesthesias, ataxia.
- sensorimotor neuropathy any of the above plus distal weaknessany of the above plus distal weakness
DIABETIC NEUROPATHIC SYNDROMES
Small Fiber Neuropathy :- “pure” small fiber neuropathy
numbness, paresthesias, painful dysesthesias, hyperesthesias.
- Diabetic neuropathy cachexiaDiabetic neuropathy cachexiasubacute, severe neuropathic pain, rapid weight loss
- Autonomic neuropathyerectile dysfunction, orthostasis, cardiac dysrhythmia, diarrhea, constipationy y , , p
DIABETIC NEUROPATHIC SYNDROMES
Ischemic Mononeuropathy.- cranial (eg. CNs III, VI,VII)
diplopia, pupil-sparing third nerve palsy, hemifacial weakness
- Radicular (thoracic lumbosacral)Radicular (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 nerveboth in territory of a single nerve
DIABETIC NEUROPATHIC SYNDROMES
Regional Neuropathic Syndromes.- Diabetic amyotrophy- Diabetic amyotrophy
subacute weakness and atrophy of proximal leg musclesproximal leg muscles
- Diabetic thoracoabdominal neuropathy.subacute weakness numbness andsubacute weakness, numbness, and atrophy in thorax and abdomen
THERAPY
Intensive diabetic therapyM i t i id l b d i htMaintain ideal body weightAdjuvant analgetics :
TCA antidepressantscarbamazepinecarbamazepinegabapentini t lid i tintravenous lidocaine, etc
CARPAL TUNNEL SYNDROME
80% in WOMEN, A COMMON TEMPORARY PHENOMENON DURING PREGNANCYPRESSURE TO THE NERVE WHEN PASSING BENEATH THE FLEXORPASSING BENEATH THE FLEXOR RETINACULUM OBSTRUCTION OF VENOUS CIRCULATION AND EDEMA ISCHEMIA INCREASING PRESSURE ON THE NERVE ISCHEMIC ATROPHY OF NERVE FIBERSNERVE FIBERS
Etiology
1. Hereditary : HMSN type III2. Traumatic : dislocation, fracture, hematoma, wrist
sprainsprain3. Infection : tenosynovitis, tbc, sarcoidosis4. Metabolic : amyloidosis, gout5 Endocrine : acromegaly DM hypothyroidism5. Endocrine : acromegaly, DM, hypothyroidism,
pregnancy6. Neoplastic : ganglion cysts, lipoma , myeloma7 Collagen vascular diseases : RA polymyalgia7. Collagen vascular diseases : RA, polymyalgia
rheumatica, SLE8. Degenerative disease : OA9. Iatrogenic : radial artery puncture, shunt for dialysis,9. Iatrogenic : radial artery puncture, shunt for dialysis,
anticoagulant therapy
Clinical Symptoms
The earliest symptoms : numbness and paresthesias in the sensory distributionparesthesias in the sensory distribution of the median nerve in the hand (thumb, index, middle and lateral half ( , ,of the ring finger)Later on : pain, worst at nightp , gLate : inability to screw bottle caps or grip properly g p p p y
SIMPLE CLINICAL TESTS FOR CTS
PHALEN’S SIGN (PHALEN’S 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)
TINEL’S SIGN (HOFFMANN-TINEL’S SIGN) :is present when tingling (paresthesia)is experienced when tapping lightly with a finger or a tendonwhen 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 ne edistribution of the damaged nerve.
Therapy
Identified causes should be treatedCorticosteroid injection around the medianCorticosteroid injection around the median nerve in the carpal tunnel.Surgical division of the transverse ligamentSurgical division of the transverse ligament (flexor retinaculum)Endoscopic carpal tunnel releaseEndoscopic carpal tunnel release
GUILLAIN - BARRE SYNDROME (GBS)( )ESSENTIALS of DIAGNOSIS :
Rapidly progressive paralysis, often diascending
AreflexiaIncreased CSF protein without increased cellIncreased CSF protein without increased cell count (albuminocytologic dissociation)Evidence of demyelination on nerve
d ti t di ( b d l d)conduction studies (may be delayed)A neurologic emergency that may rapidly progress to respiratory compromiseprogress to respiratory compromise
GBS : Subtypes and Clinical Findings
Acute inflammatory demyelinating polyneuropathy (AIDP) :polyneuropathy (AIDP) :- ascending paralysis- minor sensory symptomsminor sensory symptoms- nonspecific antibody (Ab)- EMG/NCV : demyelination on NCS absent- EMG/NCV : demyelination on NCS, absent
F waves
GBS : Subtypes and Clinical Findings
Acute motor axonal neuropathy (AMAN) :- flaccid paralysis- flaccid paralysis- often with Campylobacter jejuni infection
IgG anti GM1 IgG anti GD1a- IgG anti-GM1, IgG anti-GD1a- EMG/NCV : reduced motor amplitudes
l t dinormal sensory studies
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 amplitudesaxonal injury by EMGaxonal injury by EMG
GBS : Subtypes and Clinical Findings
Miller Fisher syndrome :- ataxia- ataxia- areflexia
opthalmoplegia- opthalmoplegia- IgG anti-GQ1b
EMG/NCV d d AP- EMG/NCV : decreased sensory nerve APmotor conductions often normalnormal
GBS : SYMPTOMS AND SIGNS
AIDP often begins 1-3 weeks after an infection or inciting event such as surgery70% cases initially have paresthesias or vague70% 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 weeksprogress over days to a few 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 isAscending weakness beginning in the distal legs is typical, although descending paralysis with predominant proximal muscles weakness rarely appearsappears.
GBS : SYMPTOMS AND SIGNS
Facial weakness occurs in half of patients with AIDP and ophthalmoparesis and lower cranial nerves
thi d th i d d h ineuropathies can cause dysarthriaand dysphagia.¼ AIDP require mechanical ventilationWeakness is symmetric, and ranges from mild toWeakness is symmetric, and ranges from mild to severe flaccid quadriparesis.Sensation is usually normal, despite sensory symptoms although mild distal vibratory loss may besymptoms, although mild distal vibratory loss may be found Reflexes are diminished or absentSphincter tone is normal
GBS : DIAGNOSTIC STUDIES
Imaging studies of the spinal cord to rule out myelopathic diseaserule out myelopathic disease.LP (after spinal cord disease excluded)Evaluation for infectionECGChest radiographsEMG/NCVEMG/NCV
GBS : DIFFERENTIAL DIAGNOSIS
Acute spinal cord disease (acute myelopathy, transverse myelitis)transverse myelitis)Brainstem ischemiaAcute disorders of neuromuscular junctionAcute disorders of neuromuscular junction (myasthenia gravis, botulinum intoxication)Acute neuropathies (porphiric neuropathy,Acute neuropathies (porphiric neuropathy, diphtheritic neuropathy, mononeuropathy multiplex, toxic neuropathy)
THERAPY
PLASMAPHARESIS (5-6 exchanges over 1- 2weeks) or)IMMUNOGLOBULIN IV (0,4 g/kg/day for 5days)
Equally effective when given within the first 2 weeks after onsetCombination of both no additional benefitCombination of both – no additional benefit
RCTs on oral or IV corticosteroid – failed to showRCTs on oral or IV corticosteroid failed to show benefit