Mononeritis multiplex

approach causes

1. MONONEURITIS MULTIPLEX Prashant Shringi Senior resident Neurology Govt. Medical College Kota

2. Generalized term including disorders of any cause affecting PNS May involve sensory nerves, motor nerves, or both or autonomic nerves May affect one nerve ( mononeuropathy ), several nerves together ( polyneuropathy ) or several nerves not contiguous ( Mononeuropathy multiplex ) PERIPHERAL NEUROPATHY

3. Mononeuritis multiplex is a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving simultaneous or sequential damage to two or more noncontiguous peripheral nerves . : Bradely’s neurology in clinical practice 7th edition DEFINITION

4. As the condition worsens, it becomes less multifocal and more symmetrical. Mononeuropathy multiplex syndromes can be distributed bilaterally, distally, and proximally throughout the body.

5. Mononeuritis multiplex actually is a group of disorders, not a true, distinct disease entity. Typically, the condition is associated with (but not limited to) systemic disorders such as the following Leprosy Diabetes mellitus Vasculitis

6. The damage to the nerves involves destruction of the axon and therefore interferes with nerve conduction. Pathophysiology

7. Common causes of damage include a lack of oxygen from decreased blood flow or inflammation of blood vessels causing destruction of the vessel wall and occlusion of the vessel lumen of small epineurial arteries.

8. Mononeuritis multiplex can be associated with many different conditions. Infections Rheumatological disorders Chronic conditions Malingnancy Hematological conditions ETIOLOGY

9. Mononeuritis multiplex can be associated with the following infections: Lyme disease  Leprosy  Acute viral hepatitis A  Hepatitis B  Hepatitis C  Acute parvovirus B-19 infection  Herpes simplex virus infection  AIDS and HIV infection  Infections

10. Mononeuritis multiplex can be associated with the following rheumatological disorders: Wegener granulomatosis   Henoch-Schönlein syndrome  Sjögren syndrome  Behçet’s disease  Temporal (giant cell) arteritis  Rheumatological Disorders

11. Systemic lupus erythematosus   Rheumatoid arthritis  Polyarteritis nodosa   Scleroderma

12. Mononeuritis multiplex can be associated with the following chronic conditions: Diabetes mellitus  Amyloidosis  Neurosarcoidosis   Celiac disease  Chronic Conditions

13. Mononeuritis multiplex can be associated with the following cancer-related conditions: Chronic graft versus host disease (GVHD)  Direct tumor invasion with intraneural spread – Lymphoma,    B-cell leukemia ,   carcinoid tumor  Paraneoplastic – Small cell lung cancer M alignancy

14. Mononeuritis multiplex can be associated with the following hematologic conditions: Churg -Strauss syndrome    Hypereosinophilia Cryoglobulinemia   Hypereosinophilia   Atopy -related peripheral neuritis   Idiopathic thrombocytopenic purpura   Hematological Disorders

15. Mononeuritis multiplex can be associated with the following miscellaneous conditions: Amphetamine angiitis   Gasoline sniffing  In addition, mononeuritis multiplex can be associated with the genetic disorder Tangier disease   and with multiple compression neuropathies.

16. Persons with one occurrence of mononeuritis multiplex are more prone to a recurrence. Mononeuritis multiplex can become progressively worse over time . Approximately 33% of cases originate from unidentifiable causes.

17. The actual incidence of mononeuritis multiplex is not known due to the widely varied etiologies that may lead to this disorder. The primary disease process often is so dominant that the symptoms of mononeuritis multiplex simply are attributed to the initial disease and remain undiagnosed. Epidemiology

18. The age of onset for mononeuritis multiplex depends on the patient's age at occurrence of the associated disease process. For unknown reasons, however, this condition does tend to occur in older patients with relatively mild or unrecognized diabetes.

19. If the cause of mononeuritis multiplex is identified early and is successfully treated, full recovery is possible, although it may take months to years. The same syndrome has a tendency to recur after an interval of months or years. The extent of disability varies, ranging from no disability to partial or complete loss of function and movement. Prognosis

20. Complications in mononeuritis multiplex include the following: Recurrent or unnoticed injury to any part of the body Deformity Atrophy Disturbances of organ functions that are autonomically controlled ( eg , cardiac, gastric, bladder) Decreased self-esteem and decreased social interaction due to an inability to participate in activities because of pain or incoordination Relationship problems associated with impotence COMPLICATIONS

21. The suspected cause of mononeuritis multiplex, as suggested by the patient’s history, symptoms, and pattern of symptom development, helps to determine which tests to perform. Approach Considerations

22. A detailed and complete medical history is vitally important in determining the possible underlying cause of mononeuritis multiplex. The pain usually is characterized as deep and aching, with superimposed lancinating jabs that are most severe at night. HISTORY & EXAMINATION

23. All patients should be questioned regarding - HIV risk factors possibility of a tick bite (Lyme disease) Constitutional symptoms ( malignancy) like weight loss, malaise, anorexia.

24. Individuals with Diabetes typically present with acute onset of severe, unilateral thigh pain that is followed rapidly by weakness and atrophy of the anterior thigh muscles and loss of the ankle reflex. Diabetes & Mononeuritis Multiplex

25. Other possible symptoms that may be reported by the patient include the following: Numbness Tingling Abnormal sensation Burning pain - Dysesthesia Difficulty moving a body part - Paralysis Lack of controlled movement of a body part

26. Loss of sensation and movement may be associated with dysfunction of specific nerves. Examination reveals preservation of reflexes and good strength except in regions that have been more profoundly affected. Physical Examination

27. Some common findings of mononeuritis multiplex are as follows Sciatic nerve dysfunction Femoral nerve dysfunction Common peroneal nerve dysfunction Axillary nerve dysfunction Radial nerve dysfunction  

28. Median nerve dysfunction Ulnar nerve dysfunction Peroneal nerve palsy Autonomic dysfunction - Dysfunction in the part of the nervous system that controls involuntary bodily functions, such as the glands, blood vessels, and heart.

29. Laboratory tests include the following : Complete blood count (CBC) with a differential Fasting blood glucose levels Borrelia burgdorferi  antibody titer - If Lyme disease is suspected Human immunodeficiency virus (HIV) blood tests - If HIV infection is suspected LAB TEST

30. Hepatitis screen - If hepatitis is suspected as a causative agent Erythrocyte sedimentation rate (ESR) and C-reactive protein level - If a systemic inflammatory process is suspected. Herpes viridae serology Parvovirus B-19 antibodies

31. Autoimmune profile Extensive vasculitic and rheumatological workup include ANA, RF, Anti- dsDNA , Anti-Ro, Anti-La, ANCA screen, cryoglobulins Serum protein electrophoresis

32. In some cases, a nerve biopsy may be appropriate to determine the underlying cause of mononeuritis multiplex (usually a combination of perivascular mononuclear inflammatory cells and multifocal axonal loss and axonal loss with multinucleated inflammatory cells) .   Histologic Findings

33.   A pattern of necrotizing vasculitis of epineural arteries may be observed in HIV-related mononeuritis .

34. Some studies have indicated that combining a nerve biopsy with a muscle biopsy can help clinicians to better diagnose peripheral nerve vasculitis , because it appears that in many cases, individuals with peripheral nerve vasculitis also have vasculitis in striated muscle.   Combined Nerve & Muscle Biopsy

35. Electrodiagnostic studies are used only in conjunction with an accurate and complete history and physical examination. The lesion or lesions are distal to the motor and sensory cell bodies and result in either axonal disruption/degeneration or abnormal axonal conduction. Electrodiagnostic Studies

36. Sensory nerve conduction studies (NCSs) show abnormalities of decreased amplitude in the presence of axonal disruption. H-reflex latencies may be prolonged or absent in mononeuritis multiplex. However, the H-reflex is typically performed only in the tibial nerves, limiting its usefulness in investigating mononeuritis multiplex. Sensory nerve conduction studies

37. Abnormalities are similar to those seen in axonal polyneuropathies, with the exception of the anatomic distribution. A reduction in the motor action potential amplitudes and minimal alterations in nerve conduction velocity will be seen. Motor nerve conduction studies

38. Findings on the needle electrode examination can vary, depending on the severity and time course of the disorder .. Needle electrode examination

39. Findings are typically neuropathic and may include abnormal spontaneous membrane activity (positive sharp waves and fibrillation potentials) and, during and after reinnervation , increases in motor unit action potential (MUAP) duration, amplitude, and polyphasic potentials.

40. Slow progression -Treat causative factors if possible Symptom Management TREATMENT

41. Tricyclic antidepressants Amitryptilin , nortryptilin Calcium channel alpha-2-delta ligands Gabapentin, pregabalin Calcium channel blocker Prialt ( Ziconotide ) SNRI’s Duloxetine, venlafaxine Topical Agents Lidocaine , Capsaicin Symptom Management

42. Antiepileptic Drugs Carbamazepine, phenytoin, lacosamide SSRI’s Opioid analgesics Tramadol Miscellaneous Botulinum toxin Mexiletine Alpha lipoic acid NMDA receptors unsuccessful Namenda, Dextromethorphan Symptom Management

43. First line drugs Lidoderm 5% patch Tricyclic antidepressants Gabapentin Pregabalin p.o. Duloxetine Second line Carbamazepine Phenytoin Venlafaxine Tramadol

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

45. If the causative factor for a patient's mononeuritis multiplex is discovered, education is directed toward avoidance of the initiating cause or pathogen. Additionally, recognition of early symptomology should be encouraged so that early treatment can be sought. Patient Education

46. THANK YOU

47. Bradely’s neurology in clinical practice 7th edition An Approach to the Evaluation of Peripheral Neuropathies;Mark B. Bromberg; SEMINARS IN NEUROLOGY/VOLUME 30, NUMBER 4 2010 Medscape.com References