Mononeuritis Multiplex

Mononeuritis Multiplex

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Mononeuritis multiplex is a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas. Multiple nerves in random areas of the body can be affected. As the condition worsens, it becomes less multifocal and more symmetrical. Mononeuropathy multiplex syndromes can be distributed bilaterally, distally, and proximally throughout the body. (See Presentation.) [1, 2, 3]

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 (see Causes, Presentation, Workup, and Treatment):

Diabetes mellitus [1, 3]

Vasculitis [4, 5, 6, 7]

Amyloidosis [8]

Direct tumor involvement – Lymphoma, leukemia [9, 10]

Polyarteritis nodosa [11]

Rheumatoid arthritis [12, 13]

Systemic lupus erythematosus [14, 15]

Paraneoplastic syndromes [16, 17]

The damage to the nerves involves destruction of the axon (ie, the part of the nerve cell that is analogous to the copper part of a wire) and therefore interferes with nerve conduction. 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.

Mononeuritis multiplex can be associated with many different conditions, as described below.

Mononeuritis multiplex can be associated with the following infections:

Lyme disease [18]

Leprosy [19, 20, 21]  – An Indian study, by Jaiswal et al, found mononeuritis multiplex to be the most common clinical presentation in a cohort of 18 patients with Hansen neuritis, with nerve conduction studies revealing the condition’s presence in 13 (72.2%) cases; severe sensory axonopathy was more prevalent than severe motor axonopathy [22]

Acute viral hepatitis A [23]

Hepatitis B [24]

Hepatitis C [25, 26]

Acute parvovirus B-19 infection [27]

Herpes simplex virus infection [28]

AIDS and HIV infection [29, 30]

Mononeuritis multiplex can be associated with the following rheumatologic disorders:

Wegener granulomatosis [31, 32]

Henoch-Schönlein syndrome [6, 33]

Sjögren syndrome [34, 35]

Behçet’s disease [36, 37]

Temporal (giant cell) arteritis [38]

Systemic lupus erythematosus [14, 15]

Rheumatoid arthritis [12, 13]

Polyarteritis nodosa – A retrospective study by Criado et al of 22 cases of cutaneous polyarteritis nodosa found that a quarter of the patients had mononeuritis multiplex [11, 39]

Scleroderma [40]

Mononeuritis multiplex can be associated with the following chronic conditions:

Diabetes mellitus [1, 3]

Amyloidosis [8]

Neurosarcoidosis [41, 42]

Celiac disease [43]

Mononeuritis multiplex can be associated with the following cancer-related conditions:

Chronic graft versus host disease (GVHD) [44]

Direct tumor invasion with intraneural spread – Lymphoma, [10] B-cell leukemia, [9] carcinoid tumor [45]

Paraneoplastic – Small cell lung cancer [16, 17]

Mononeuritis multiplex can be associated with the following hematologic conditions:

Churg-Strauss syndrome [46, 47]

Hypereosinophilia

Cryoglobulinemia [48]  – A single-center, retrospective study by Feldman et al found that of 131 patients with possible or definite cryoglobulinemia-associated neurologic symptoms, 16 (12.2%) had mononeuritis multiplex [49]

Hypereosinophilia [50]

Atopy-related peripheral neuritis (see below) [51]

Idiopathic thrombocytopenic purpura [52, 53]

Mononeuritis multiplex can be associated with the following miscellaneous conditions:

Amphetamine angiitis [54]

Gasoline sniffing [55]

In addition, mononeuritis multiplex can be associated with the genetic disorder Tangier disease [56, 57] and with multiple compression neuropathies.

Moreover, in a study of 157 patients with eosinophilic granulomatosis with polyangiitis, Cottin et al found mononeuritis multiplex to be associated with cases that included systemic vasculitis and the presence of antineutrophil cytoplasmic antibodies. [58]

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. [59]

In a cross-sectional, Japan-wide survey, Isobe et al found that patients with atopy-related peripheral neuritis (n = 133) tended to develop mononeuritis multiplex, with their lower limbs predominantly affected. The investigators also determined that most patients with atopy-related peripheral neuritis were female and that individuals with the disease tended to have a higher eosinophil count than did patients in the study with atopic myelitis. [51]

The actual incidence of mononeuritis multiplex in the United States and rest of the world 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.

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.

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. 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

A Danish study by Al-Zuhairy et al found that compared with healthy controls, patients who had suffered from multifocal motor neuropathy, a form of mononeuritis multiplex, for a median period of 24 years had a 9% decrease in the Rasch-built Overall Disability Scale for Multifocal Motor Neuropathy, a three-fold increase in the Neuropathy Impairment Score, a 29% reduction in isokinetic strength, a 56% decrease in grip strength, a 13% prolongation of the Timed 25-Foot Walk, and a 20% impairment measured via the EQ-5D-DL index value. [60]

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.

Persons with decreased sensation should be instructed to frequently check their feet or other affected areas for bruises, cuts, wounds, or other injuries. In addition, patients who are insensate or incapacitated should be instructed to avoid prolonged pressure on various points on the body (eg, knees, elbows, sacrum) so as to discourage the development of pressure sores or ulcers.

Safety awareness instruction is important for these patients because of their impaired sensation and decreased ability to compensate for limitations. The patient should be instructed to ensure that there is always adequate lighting, to test the water temperature before bathing or immersing body parts, and to wear protective shoes (no open toes or high heels).

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Divakara Kedlaya, MBBS Medical Director, Physical Medicine and Rehabilitation and Pain Management, St Mary Corwin Medical Center

Divakara Kedlaya, MBBS is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Dean H Hommer, MD Chief, Department of Pain Management, Brooke Army Medical Center

Dean H Hommer, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Pain Medicine, American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American College of Healthcare Executives, American College of Sports Medicine, American Institute of Ultrasound in Medicine, American Society of Interventional Pain Physicians, American Society of Regional Anesthesia and Pain Medicine

Disclosure: Nothing to disclose.

Paul V Brooks, MD Medical Director, Department of Physical Medicine and Rehabilitation, Lexington Clinic, PSC; Assistant Professor, Department of Orthopedics, Division of Sports Medicine, Assistant Professor, Department of Surgery, University of Kentucky College of Medicine

Paul V Brooks, MD is a member of the following medical societies: American Academy of Pain Medicine, American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Association of University Professors, American College of Sports Medicine, American Medical Association, American Pain Society, American Spinal Injury Association, Association for Academic Psychiatry, and Brain Injury Association of America

Disclosure: Nothing to disclose.

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