Approach to peripheral neuropathy

Approach to peripheral neuropathy

1. Approach to peripheral neuropathy Dr Vaishal Shah SR Neurology GMC Kota

2. Types of nerves and their function

3. Sensory symptoms

4. Motor symptoms

5. Autonomic symptoms

6. Causes Systemic disorders Endocrine – DM, hypothyroidism, acromegaly Connective tissue – Sjogren, RA, SLE, MCTD, Vasculitis Nutritional – B complex ( B1, niacin, B6, B12 ), vitamin E, copper, B6 overdose Inflammatory – AIDP, CIDP, Amyloidosis, Sarcoidosis, hypereosinophilic syndrome, IBD, celiac disease Metabolic – CLD, Uremia , Porphyria Infective – Leprosy, HIV, Diphtheria, Lyme, Hep B & C Malignancy – Paraneoplastic, Infiltration by leukemia & lymphoma, Plasma cell disorders Critical care neuropathy

7. Causes Toxic ( Drugs/toxin ) Hereditary – CMT –M/C Environmental – Vibration induced, prolonged cold exposure, hypoxemia Idiopathic – 46%. Most cases > 50 years. Progession – slowly over months to years. Predominant sensory symptoms. Proposed but unproven causes are HTn , dyslipidemia, increased oxidative stress.

8. Drugs

9. Toxins

10. Classification

11. Evaluation Mild symptoms with known underlying lesion like DM, chemotherapy, alcohol abuse – No evaluation is required Feature warranting a full evaluation Assymetry Non length dependence Motor predominance Acute onset Predominant autonomic involvement Rapidly progressive symptoms Sensory ataxia

12. Acute / abrupt onset GBS Vasculitis Porphyria Infectious disease ( Lyme disease, diphtheria ) Toxin / Drug – arsenic, thallium, chemotherapeutic agents, depsone

13. Subacute/chronic CIDP DM Vasculitis HIV Vitamin B12 deficiency Copper deficiency Paraneoplastic Sjogren syndrome Toxin, Drug

14. Relapsing and remitting course CIDP Porphyria HNPP

15. General physical examination Purpura Vasculitis Angiokeratoma Fabry disease Hypopigmentation Leprosy Orange tonsil Tangier’s disease

16. General physical examination 1)Skin hyperpigmentation 2)Clubbing POEMS syndrome

17. General physical examination Mess line Arsenic or thallium intoxication

18. General physical examination R etinitis pigmentosa, Icthyosis Refsum’s disease ( AR ) RP PN Cerebellar ataxia Elevated CSF protein

19. General physical examination Thickened nreves Leprosy Neurofibromatosis Refsum disese Amyloidosis HMSN DM Sarcoidosis

20. General physical examination Purpura, Livodereticularis – Vasculitis, Cryoglobulinemia Angiokeratomas – Fabry’s disease Skin pigmentation – Leprosy, POEMS, adrenoleukodystrophy Icthyosis – Refsum’s Disease Mees ’ line – Arsenic / Thallium Intoxication Alopecia – Thallium Poisoning, Hypothyroidism, SLE Maculoanaesthetic patches with thickened nerves - Leprosy Orange Tonsils – Tangier’s disease Pes cavus , high-arched feet and mutilation – Hereditary neuropathy Macroglossia – Amyloidosis Chelosis /Glossitis – Multivitamin deficiency

21. Electrodiagnosis - Mononeuropathy Direct trauma Compression or entrapement Neoplastic compression or infiltration

22. Electrodiagnosis – Mononeuritis multiplex Simultaneous / sequential damage to multiple noncontiguous nerves. Ischemia caused by systemic vasculitis. Painful (90%) neuropathy progresses over weeks ). Can be patchy. Sensory/sensorimotor. Lower > upper. Distal > proximal. Constitutional symptoms present in most of the patients. Microangiopathy in DM – Diabetic amyotrophy

23. Electrodiagnosis – Mononeuritis multiplex Ischemic – Sickle cell, cholesterol emboli, atrial myxoma Inflammatory – Sarcoidosis, CIDP variants ( MADSAM, MMN ), Eosinophilic disorders, A/W crohn’s , UC and celiac disease Infectious – Leprosy, Lyme, Viral ( Hep B, Hep C, other viral infection in immunocompromised patients ) Genetic – HNPP, Familial amyloid neuropathy Neoplastic – Lymphoma, leukemia , paraneoplastic

24. Electrodiagnosis Polyneuropathy Axonal vs demyelination Distal>Proximal vs Distal=proximal LL>UL vs UL=LL Wasting vs weakness>wasting CMAP vs Latency, CV

25. Clue – Painful polyneuropathy DM Alcohol Paraproteinemia Vasculitis associated and connective tissue associated Amyloidosis ( familial, acquired ) Paraneoplastic sensory neuropathy ( Mainly Lung ) Idiopathic small fibre neuropathy

26. Clue – Motor predominant GBS Porphyria Lead poisoning Variant of CIDP

27. Clue – Significant autonomic involvement DM GBS Amyloidosis ( Familial, acquired ) Uremic Porphyria ( descending neuropathy ) HIV related autonomic neuropathy Hereditary sensory and autonomic neuropathy Vincristine

28. Clue - Neuropathies with cranial nerve involvement Diabetes mellitus GBS HIV/AIDS Lyme disease Sarcoidosis Neoplastic invasion of skull base or meninges Diptheria Friedrich’s ataxia

29. Clue – Drugs with demyelinating neuropathy Amiodarone Chloroquine Suramin Gold

30. Nerve biopsy Vasculitis, amyloid, leprosy, hereditary cause. Sural nerve. Occasional – superficial peroneal nerve. Biopsy of peroneous brevis through same incision – Increases yield in vasculitis.

31. Skin biopsy Small fibre neuropathy Very small piece of skin just proximal to ankle is removed. Special stains are applied - Qualitative assessment or by careful counting to determine intraepidermal nerve fibre.

32. Thank you

33. References Clinical Approach to Peripheral Neuropathy: Anatomic Localization and Diagnostic Testing Adina R. Alport et al : Continuum Lifelong Learning Neurol 2012;18(1):13–38. An Approach to the Evaluation of Peripheral Neuropathies;Mark B. Bromberg; SEMINARS IN NEUROLOGY/VOLUME 30, NUMBER 4 2010 www.uptodate.com Harrison 20 th edition