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Mononeuropathy Multiplex


Presentation

  • Mononeuritis multiplex can sometimes be the initial presentation of SLE[ 1 ]. Confirmation of diagnosis is by nerve biopsy and nerve conduction studies.[escholarship.org]
  • Although other organ systems are often involved, the initial clinical presentation of systemic vasculitis may involve only the peripheral nervous system.[casemed.case.edu]
  • Cardiovascular autonomic neuropathy was present in two of the patients and there was improvement after treatment in the Ewing's tests – Valsalva manoeuvre, deep‐breathing test and lying‐to‐standing test.[onlinelibrary.wiley.com]
  • (See Presentation.) [1, 2, 3] Mononeuritis multiplex actually is a group of disorders, not a true, distinct disease entity.[emedicine.medscape.com]
  • Rheumatol., 34:184. [12] Shakir, R.A., Sulaimam K., Kahn, R.A., Rudwan, M. (1990)Neurological presentation of neuro-Behcet's syndrome: clinical categories. Eur.[kundoc.com]
Inflammation
  • Mononeuritis multiplex: Inflammation of two or more nerves, typically in unrelated parts of the body. Mononeuritis multiplex causes a loss of function in the muscle tissue that is innervated by the affected nerves.[medicinenet.com]
  • […] of the lymph nodes, lungs, liver, eyes, skin, or other tissues Sjögren syndrome, a disorder in which the glands that produce tears and saliva are destroyed Granulomatosis with polyangiitis, an inflammation of the blood vessel Symptoms depend on the specific[medlineplus.gov]
  • This is an inflammation around some blood vessels that can affect the vasa nervorum, or blood vessels around certain nerves.[aanem.org]
  • When the etiology involves inflammation, the term mononeuritis multiplex is used. In most cases, the individual neuropathies are of named nerves (i.e., median, ulnar, peroneal, etc.) as opposed to small nerve twigs.[casemed.case.edu]
  • The immediate cause of the vasculitic neuropathies is inflammation and occlusion of the vasa nervosum resulting in ischemia of the peripheral nerve.[escholarship.org]
Pain
  • Medicines that may help include: Over-the-counter or prescription pain drugs Antiseizure or antidepressant drugs to reduce stabbing pains A full recovery is possible if the cause is found and treated, and if the nerve damage is limited.[medlineplus.gov]
  • Treatment of neuropathic pain: an overview of recent guidelines. Am J Med. 2009 Oct. 122(10 Suppl):S22-32. [Medline]. Finnerup NB, Sindrup SH, Jensen TS. The evidence for pharmacological treatment of neuropathic pain. Pain. 2010 Sep. 150(3):573-81.[emedicine.medscape.com]
  • There was diminished sensory perception to touch, pain and temperature in the lower and upper limbs with motor weakness.[escholarship.org]
  • More information on Mononeuritis Multiplex Foundation for Peripheral Neuropathy American Chronic Pain Association[aanem.org]
  • As each subsequent nerve is infarcted, pain develops (often severe), followed hours or days later by weakness and numbness in the nerve’s distribution.[casemed.case.edu]
Amyloidosis
  • Common conditions include: Blood vessel diseases such as polyarteritis nodosa Connective tissue diseases such as rheumatoid arthritis or systemic lupus erythematosus (the most common cause in children) Diabetes Less common causes include: Amyloidosis,[medlineplus.gov]
  • Primary amyloidosis presenting as upper limb multiple mononeuropathies. Muscle Nerve. 2010 May. 41(5):710-5. [Medline]. [Full Text]. Le Clech L, Rizcallah MJ, Alavi Z, Hutin P.[emedicine.medscape.com]
  • […] includes (1) inflammatory cells in the vessel wall accompanied by pathologic evidence of acute or chronic vascular wall damage, and (2) no evidence of another primary disease that mimics vasculitis pathology (lymphoma, lymphomatoid granulomatosis or amyloidosis[ncbi.nlm.nih.gov]
Weight Loss
  • Unspecific symptoms, such as weight loss, fever or fatigue, have been reported in 80% of neuropathy with systemic vasculitis and in about 50% of patients with nonsystemic vasculitic neuropathy (NSVN).[ncbi.nlm.nih.gov]
Physician
  • A physician may recommend physical therapy or orthopedic interventions. If the cause of mononeuritis multiplex can be identified and treated, a patient may be able to fully recover.[aanem.org]
Arthritis
  • There are many causes of mononeuritis multiplex, including diabetes mellitus; infections, such as AIDS, Lyme disease, and leprosy ; sarcoidosis ; and connective tissue diseases, such as rheumatoid arthritis, systemic lupus erythematosus, vasculitis, Churg-Strauss[medicinenet.com]
  • Rheumatoid arthritis Neuropathy occurs in 15–50% of rheumatoid arthritis (RA) patients.[ncbi.nlm.nih.gov]
  • A clinical, electrophysiological, and pathological study of neuropathy in rheumatoid arthritis. Clin Rheumatol. 2008 Jul. 27(7):841-4. [Medline]. Florica B, Aghdassi E, Su J, Gladman DD, Urowitz MB, Fortin PR.[emedicine.medscape.com]
  • Differential Diagnosis of Mononeuropathy Multiplex • Vasculitis Polyarteritis nodosa Churg-Strauss syndrome Wegener's syndrome Hypersensitivity Cryoglobulinemia Systemic lupus erythematosus Rheumatoid arthritis Sjögren’s syndrome Chronic active hepatitis[casemed.case.edu]
Myalgia
  • The clinical symptoms include weight loss, livedo reticularis, testicular pain, myalgia, kidney involvement and visceral artery aneurysms. PAN is mostly a monophasic disease and the prognosis depends on the involved organ systems.[ncbi.nlm.nih.gov]
Mononeuropathy
  • Related to mononeuropathy multiplex: polyneuropathy, Diabetic amyotrophy mon·o·neu·rop·a·thy mul·'ti·plex nontraumatic involvement of two or more portions of the peripheral nervous system (for example, roots, plexus elements, nerve trunks), usually sequentially[medical-dictionary.thefreedictionary.com]
  • Multiple mononeuropathy is a nervous system disorder that involves damage to at least two separate nerve areas. Neuropathy means a disorder of the nerves. Multiple mononeuropathy is a form of damage to one or more peripheral nerves.[medlineplus.gov]
  • On the 10th day, we found significant improvement in the clinical signs of diabetic mononeuropathy – double vision, motility and position of the eyeball, ptosis of the upper eyelid and mydriasis.[onlinelibrary.wiley.com]
  • Mononeuropathy multiplex is associated with a limited differential diagnosis (see list below) and most often occurs in the setting of VASCULITIS and vasculitic neuropathy.[casemed.case.edu]
  • Sensory Mononeuropathy Multiplex in Chronic Graft versus Host Disease. J Clin Neuromuscul Dis. 2001 Jun. 2(4):184-6. [Medline]. Grisold W, Piza-Katzer H, Jahn R, Herczeg E. Intraneural nerve metastasis with multiple mononeuropathies.[emedicine.medscape.com]
Polyneuropathy
  • Related to mononeuropathy multiplex: polyneuropathy, Diabetic amyotrophy mon·o·neu·rop·a·thy mul·'ti·plex nontraumatic involvement of two or more portions of the peripheral nervous system (for example, roots, plexus elements, nerve trunks), usually sequentially[medical-dictionary.thefreedictionary.com]
  • Over time, a confluent pattern may develop, which may be difficult to distinguish from a generalized polyneuropathy. When the etiology involves inflammation, the term mononeuritis multiplex is used.[casemed.case.edu]
  • Most patients with paraneoplastic vasculitic neuropathy present clinically with mononeuropathia multiplex or asymmetric polyneuropathy.[ncbi.nlm.nih.gov]
Tingling
  • We report a case of 34-year-old male who had multiple joint pains for 10 years, hyperpigmented skin lesions on face with photosensitivity for 4 years, oral erosions for 2 years, and tingling and numbness of the lower and upper extremities of 3 weeks duration[escholarship.org]
  • Granulomatosis with polyangiitis, an inflammation of the blood vessel Symptoms depend on the specific nerves involved, and may include: Loss of bladder or bowel control Loss of sensation in one or more areas of the body Paralysis in one or more areas of the body Tingling[medlineplus.gov]

Workup

  • Typically, the condition is associated with (but not limited to) systemic disorders such as the following (see Causes, Presentation, Workup, and Treatment): Vasculitis [4, 5, 6, 7] Amyloidosis [8] Direct tumor involvement - Lymphoma, leukemia [9, 10][emedicine.medscape.com]

Treatment

  • On the 10th day, we established a decrease in total symptom score by an average of 2.7   1.4 points and by the end of the treatment period it was improved by 5.9   1.9 points (p 0.04).[onlinelibrary.wiley.com]
  • If no improvement can be seen, combination of antiviral and immunosuppressive treatment is necessary (see treatment).[ncbi.nlm.nih.gov]
  • . • Serology for Lyme, HIV (selected patients) • Systemic Evaluation for malignancy or sarcoid (depending on risks and other symptoms) Treatment The treatment ultimately depends upon the etiology.[casemed.case.edu]
  • Role of intravenous cyclophosphamide in treatment of neuropsychiatric systemic lupus erythematosus.Am J Med1995;98:32-41. 2005 Dermatology Online Journal[escholarship.org]
  • The treatment for mononeuritis multiplex depends on the underlying cause. CONTINUE SCROLLING OR CLICK HERE FOR RELATED ARTICLE SLIDESHOW Super Tips to Boost Digestive Health: Bloating, Constipation, and More See Slideshow[medicinenet.com]

Prognosis

  • A population-based and cross-sectional study of the long-term prognosis in multifocal motor neuropathy. J Peripher Nerv Syst. 2019 Feb 12. [Medline]. Said G, Lacroix-Ciaudo C, Fujimura H, et al.[emedicine.medscape.com]
  • PAN is mostly a monophasic disease and the prognosis depends on the involved organ systems. If the heart or the central nervous system is involved, prognosis is poor.[ncbi.nlm.nih.gov]

Etiology

  • When the etiology involves inflammation, the term mononeuritis multiplex is used. In most cases, the individual neuropathies are of named nerves (i.e., median, ulnar, peroneal, etc.) as opposed to small nerve twigs.[casemed.case.edu]
  • […] such as the following (see Causes, Presentation, Workup, and Treatment): Vasculitis [4, 5, 6, 7] Amyloidosis [8] Direct tumor involvement - Lymphoma, leukemia [9, 10] Rheumatoid arthritis [12, 13] Paraneoplastic syndromes [16, 17] Pathophysiology and Etiology[emedicine.medscape.com]
  • The etiology has remained elusive although an association of IIH with numerous conditions has been reported: Endocrine abnormalities that have been reported include menstrual irregularities [1], adrenal insufficiency [2], hypoparathyroidism [3], and hypothyroidism[kundoc.com]

Epidemiology

  • Epidemiological, clinical and laboratory profiles of cutaneous polyarteritis nodosa patients: Report of 22 cases and literature review. Autoimmun Rev. 2016 Jun. 15 (6):558-63. [Medline]. Lee P, Bruni J, Sukenik S.[emedicine.medscape.com]
  • Clinical and epidemiologic aspects.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • Pathogenesis Inflammation of the walls of nutrient and epineural arteries is the main pathophysiological feature in vasculitic neuropathy.[ncbi.nlm.nih.gov]
  • […] systemic disorders such as the following (see Causes, Presentation, Workup, and Treatment): Vasculitis [4, 5, 6, 7] Amyloidosis [8] Direct tumor involvement - Lymphoma, leukemia [9, 10] Rheumatoid arthritis [12, 13] Paraneoplastic syndromes [16, 17] Pathophysiology[emedicine.medscape.com]

Prevention

  • Preventive measures depend on the specific disorder. For example, with diabetes, eating healthy foods and keeping a tight control of blood sugar may help prevent multiple mononeuropathy from developing.[medlineplus.gov]
  • Mononeuritis multiplex from vasculitis requires urgent treatment to prevent further progression and disability.[casemed.case.edu]

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