Peripheral neuropathy

ppt

1. Dr . Sabir K umar Khadka Dept of O rthopedics PERIPHERAL NEUROPATHY

2.  Refers to nerves outside the brain and spinal cord. Broken down into Sensory Motor Autonomic Parasympathetic Sympathetic

3. May affect one nerve ( mononeuropathy ), S everal nerves together ( polyneuropathy ) S everal nerves not contiguous ( Mononeuropathy multiplex ) Further classified into those that primarily affect the cell body (e.g., neuronopathy or ganglionopathy ), myelin ( myelinopathy ), and the axon ( axonopathy )

4. Pathology Acute axonal interruption Loss of motor and sensory function is immediate and complete Detectable at early stage by nerve conduction and EMG. Chronic axonal degeneration Symptoms tend to appear in feet and legs before arms and hands(stocking and glove distribution) NCV:reduction in size of CMAP and SNAP responses Demyelinating neuropathies Focal entrapment most common in nerve entrapment syndrome and blunt soft tissue trauma. Slowing of conduction and complete nerve block Eg : GBS and HMSN

5. P ossible mechanisms of peripheral nerve degeneration Demyelination – e.g. Guillain-Barre Syndrome Axonal degeneration - e.g. toxic neuropathies Compression – e.g. carpal tunnel syndrome Infarction – e.g. diabetes Infiltration – e.g. leprosy and granulomas

6. Causes of polyneuropathy Hereditary Hereditary motor and sensory neuropathy Friedreich ataxia Hereditary sensory neuropathy Infections Viral infections Herpes zoster Neuralgic amyotrophy Leprosy

7. Inflammatory Acute inflammatory polyneuropathy Guillain barre syndrome SLE Nutritional and metabolic Vitamin deficiencies Diabetes Myxoedema Amyloidosis Neoplastic Primary carcinoma Myeloma Toxic Alcohol lead

8.  Disease Diabetes Paraproteinaemia Alcohol misuse Renal failure Vitamin B-12 deficiency HIV infection Chronic idiopathic axonal neuropathy  Prevalence  11-41% (depending on duration, type,and control)  9-10%  7%  4%  3.6%  16% (depending on the population studied, usually much lower)  10-40% of different hospital series BMJ 2010:341:c6100

9. Loss of function “- symptoms” Disordered function “+ symptoms” Sensory “Large Fiber” ↓ Vibration ↓ Proprioception Hyporeflexia Sensory ataxia Paresthesias Sensory “Small Fiber” ↓ Pain ↓ Temperature Dysesthesias Allodynia The clinical response to sensory nerve injury

10. Loss of function “- symptoms” Disturbed function “+ symptoms” Motor nerves Wasting Fasciculation Large fibre Hypotonia Cramps Weakness Hyporeflexia Orthopedic deformity The clinical response to motor nerve injury

11. Loss of function “- symptoms” Disturbed function “+ symptoms” Autonomic nerves ↓ Sweating Hypotension ↑ Sweating Hypertension Urinary retention Impotence Vascular color changes The clinical response to autonomic nerve injury

12. Peripheral nerve compression and entrapment Carpal tunnel syndrome is a common mononeuropathy – Median nerve entrapement Clinical presentation Pain, tingling and paraesthesia on palmar aspect of hand and fingers Weakness of thenar muscles and wasting of abductor pollicis brevis Pain may extend to arm and shoulder Tinel’s and Phalen’s tests are positive.

13. Paraesthesia Numbness Burning pain Loss of vibration sense and position sense Difficulty using small objects e.g. needles Subacute with ataxia due to loss of sense of posture Feet are usually affected first – S t ock and Glove

14.  Clinical Presentation: Postural hypotension Urinary retention Erectile dysfunction Diarrhoea/constipation Diminished sweating Impaired pupillary response Cardiac arrhythmias Might occur in: Diabetes Amyloidosis Guillain-Barre syndrome

15.  Clinical presentation: Progressive weakness or clumsiness Difficulty walking (falling or stumbling) Respiratory difficulties (falling vital capacity) Wasting Foot or wrist drop might be seen Reflexes absent or reduced

16.  MONONEUROPATH Y Focal involvement of a single nerve Weakness & sensory loss in the territory of a single peripheral nerve Pain along the pathway of the nerve Direct tr a uma compression entrapment V a s c u lar lesions neoplasms

17.  Random pattern of nerve involvement In distribution of separate nerves,asymmetric May/may not be painful Not length dependent Isolated reflex loss  Mononeuropathy Multiplex

18. CAUSES —inflammatory- leprosy,sarcoid  Vascular-Diabetes Pressure,Trauma,Infiltration Vasculitis- PAN,SLE,RA,scleroderma Mononeuropathy Multiplex

19. MC type –Distal symmetric polyneurpathy Burning sensation,tingling,numbness Length dependent pattern Starts in feet,distal stocking glove pattern Fairly symmetric Symmetrically decreased reflexes Sensory>motor Polyneuropathy

20. Diabetes mellitus Alcohol Vit B12 deficiency HIV Although more than one nerve involved one will be prominant

21. P O LY R ADI C ULOPATHY Disease of multiple peripheral nerve roots Asymmetric with erratic distribution-proximal in one,distal in another Pain is a common feature MONORADICULOPATHY Root disease by disease of spinal column Changes in distribution of spinal nerve root

22. SENSORY NEURONOPATHY Ganglion cells predominantly affected Both proximal & distal involvement Sensory ataxia is common No weakness But awkward movement d/t sensory disturbances MOTOR NEURONOPATHY Disorder of ant horn cells Weakness,fasciculation,atrophy

23. PLEXOPATHY Asymmetric Painful onset Multiple nerves in a single limb Rapid onset of weakness,atrophy Isolated reflex loss Brachial plexus traction injury Lumbosacral plexopathy in pelvic trauma Compression by local tumor( pancoast’s tumor)

24. Directly related to the duration and degree of abnormal metabolic control – occurring relatively early in disease Due to metabolic disturbance and accumulation of fructose and sorbitol in Scwann cells Types of Diabetic neuropathy Symmetrical mainly sensory neuropathy Acute painful neuropathy Mononeuropathy and mononeuritis multiplex Diabetic amyotrophy Autonomic Neuropathy

25.  Chronic alcohol abuse leads to polyneuropathy  Calf pain is common Deficiency in thiamine due to alcoholism also causes neuropathy Can lead to Wernicke- Korsako ff syndrome Common presentation Eye signs Ataxia Cognitive change Delirium tremens Hypothermia and hypotension

26. Acute polyneuropathy – acute inflammatory or postinfective neuropathy Usually demyelinating but can be axonal F ollowing Campylobacter jejuni and CMV infections Infection induces antibody responses against peripheral nerves Paralysis 1-3 weeks following infection Weakness of distal limb muscles and/or distal numbness Symptoms progress proximally Loss of tendon reflexes Facial muscle weakness Autonomic features - uncommon Might need ventilatory support SC heparin is required to reduce risk of thrombosis Spontaneous recovery begins after several weeks

27. DM hypothyroidism chronic renal failure liver disease intestinal m a labsor p tion malignancy connective tissue diseases [HIV] drug use Vitamin B6 toxicity alcohol and dietary habits Weight loss, malaise, and anorexia.

28. Diabetes and Pre-Diabetes Alcohol neuropathy Chemotherapy Platinum-based Paraproteinemia Vasculitis and Connective Tissue Diseases Heavy metals and other toxins HIV Amyloidosis Porphyria

29.  A x o n al Vincristine Paclitaxel Nitrous oxide Colchicine Probenecid Isoniazid Hydralazine Me tronidaz o le Pyridoxine Didanosine Lithium Alfa interferon Dapsone  Axonal - continued.. Phenytoin Cimetidine Disulfiram Chloroquine Ethambutol Demyelinating Amiodarone Chloroquine Suram i n G o ld  N e ur on o p athy Thalidomide Cisplatin Amitriptyline Pyridoxine

30.  The temporal course of a neuropathy varies, based on the etiology. With trauma or ischemic infarction, the onset will be acute, with the most severe symptoms at onset. Inflammatory neuropathies have a subacute course extending over days to weeks. A chronic course over weeks to months is the hallmark of most toxic and metabolic neuropathies.

31. A chronic, slowly progressive neuropathy over many years occurs with most hereditary neuropathies or with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Neuropathies with a relapsing and remitting course include CIDP, acute porphyria, Refsum's disease, hereditary neuropathy with liability to pressure palsies (HNPP), familial brachial plexus neuropathy, and repeated episodes of toxin exposure.

32. Ischemic neuropathies often have pain as a prominent feature. Small-fiber neuropathies often present with burning pain, lightning-like or lancinating pain, aching, or uncomfortable paresthesias (dysesthesias).

33.  Peripheral neuropathy can present as restless leg syndrome.  Proximal involvement may result in difficulty climbing stairs, getting out of a chair, lifting and bulbar involvement can also be seen

34.  The clinical assessment should include: careful past medical history, looking for systemic diseases that can be associated with neuropathy, such as diabetes or hypothyroidism.

35.  All patients should be questioned regarding HIV risk factors diet (nutrition) vitamin use (especially B6) possibility of a tick bite (Lyme disease) Constitutional symtoms (malignancy)

36. A cranial nerve examination can provide evidence of mononeuropathies. Funduscopic examination may show abnormalities such as optic pallor, which can be present in leukodystrophies and vitamin B12 deficiency.

37. Tests with the highest yield of abnormality: blood glucose (fasting) serum B12 with metabolites (methylmalonic acid, homocysteine) SPEP(serum protein electrophoresis)

38. B L O OD TC,DC,ESR Urea,electrolytes,LFT RBS,HbA1C Serum protein electrophoresis Auto Ab=ANA,Antiganglioside,Antineuronal Vit B 12 level DNA analysis=chr 17 duplication-HMSN1&1A  =chr 17 deletion -HLPP

39. U R I N E BJ protein Porphyr ia Heavy metals CSF ANALYSIS

40. NERVE CONDUCTION STUDY Variation in axonal,demyelinating neuropathy Conduction block-CIDP,GBS EMG -muscle denervation changes

41. Antibodies against Gangliosides  GM 1 : GM 1 , GD1a : GQ1b : Multifocal motor neuropathy   Guillain-Barré syndrome Miller Fisher variant Antibodies against Glycoproteins  M y eli n - asso c iate d glycopro t ei n Antibodies against RNA-binding proteins  Anti-Hu, antineuronal nuclear antibody 1: Malignant inflammatory polyganglionopathy

42. (1) Confirming the presence of neuropathy, (2) Locating focal nerve lesions, (3) Nature of the underlying nerve pathology

43.  The limitations of EMG/NCS should be taken into account when interpreting the findings. There is no reliable means of studying proximal sensory nerves. NCS results can be normal in patients with small- fiber neuropathies Lower extremity sensory responses can be absent in normal elderly patients.  EMG/NCS are not substitutes for a good clinical examination.

44. IMAGES  CXR-sarcoidosis,malignancy Skeletal survey-multiple myeloma Screening for malignancy AUTONOMIC FUNCTION TESTS Diagnostic tests imp in  Asymmetric,motor predominant,rapid onset,demyelinating neuropathy

45. In vasculitis, amyloid neuropathy, leprosy, CIDP, Inherited disorders of myelin, and rare axonopathies The Sural nerve is selected most commonly The superficial peroneal nerve – alternative; :advantage of allowing simultaneous biopsy of the peroneus brevis muscle through the same incision. This combined nerve and muscle biopsy procedure increases the yield of identifying suspected vasculitis

46. “For symptomatic patients with suspected polyneuropathy, skin biopsy may be considered to diagnose the presence of a polyneuropathy, particularly SFSN.”

47.  Slow progression Treat causative factors if possible If rapidly progressing IVIG Immunomodulating agents

48.  Tricyclic antidepressants Amitryptilin, nortryptilin Calcium channel alpha-2-delta ligands Gabapentin, pregabalin SNRI’s Duloxetine, venlafaxine Topical Agents Lidocaine, Capsaicin

49.  Antiepileptic Drugs Carbamazepine, phenytoin, lacosamide SSRI’s Opioid analgesics Tramadol Miscellaneous Botulinum toxin Mexiletine Alpha lipoic acid

50.  Physical Therapy Gait and balance training Assistive devices Safe environment Footwear at all times Foot hygiene

51. Thank you

52. REFERENCES APLEY & SOLOMON’S SYSTEM OF ORTHOPAEDICS AND TRAUMA, 10 TH EDITION HARRISON’S PRINCIPLE OF INTERNAL MEDICINE,20 th EDITION