Mononeuritis multiplex is a disease of the nervous system that affects two random body areas with severe pain and damage to motor, and sensory function. It most commonly occurs due to disruption of blood supply to the peripheral nervous system, although various etiologies are thought to underlie the disease. Affected areas depend on the causative etiologies.
Presentation
History is critical in determining the underlying causative factors responsible for the disease. Associated pain is of aching quality and is felt deep in the body. It starts in areas of the low back and then radiates unilaterally to the knee and the thigh. Lancinating pain may occur, particularly at night. Patients with diabetes mellitus generally present in a different manner. They report an acute episode of severe pain in the thigh on one side only. This is usually followed by wasting of the muscles of the anterior thigh and associated weakness.
In addition to pain, patients frequently complain of several symptoms of neurological nature such as tingling, dysesthesia (pain of burning quality), numbness, paresthesias (abnormal sensations) and sometimes frank paralysis.
On physical exam, deep tendon reflexes are generally preserved and accompanied by excellent muscle strength, with the exception of localized regions where the damage is more severe. Other findings on the physical exam may point to the involvement of the following nerve structures: femoral nerve, axillary nerve, radial nerve, median nerve, ulnar nerve, sciatic nerve, common peroneal nerve, peroneal nerve and sciatic nerve. The latter is most frequently affected [3]. In addition, mononeuritis multiplex is linked with autonomic dysfunction.
Entire Body System
- Inflammation
Also found in: Wikipedia. mononeuritis [mon″o-noo͡-ri´tis] inflammation of a single nerve. mononeuritis mul´tiplex simultaneous inflammation of several nerves remote from one another. [medical-dictionary.thefreedictionary.com]
QUESTION Pancreatitis is inflammation of an organ in the abdomen called the pancreas. See Answer [medicinenet.com]
Sural nerve biopsies showed multifocal axonal loss in all patients, with epineurial perivascular inflammation affecting small calibre vessels in three. Three patients improved with immunotherapy. [ncbi.nlm.nih.gov]
- Congestive Heart Failure
Heart Failure 422 Case 67 An Elderly Woman with a Progressive Neuropathy 429 Case 68 A Young Woman with Difficulty Swallowing 433 Case 69 A Young Woman with Intermittent Weakness and a Positive Family History of Similar Problems 436 Case 70 A Woman with [books.google.com]
He deteriorated with subsequent pneumonia, congestive heart failure and death four months after the initial neurologic presentation. [bcm.edu]
- Short Stature
Stature Small Jaw Muscle Hypertrophy and Stiffness 609 Case 99 An Elderly Woman with Muscle Spasms 612 Case 100 A Woman with Proximal Muscle Weakness and Neuromuscular Irritability 616 Case 101 A Woman with Focal Spontaneous Muscle Movements 620 Index [books.google.com]
- Sepsis
The intestinal lesions are known to break through the wall of the bowels (perforation), thus leading to a potentially life threatening complication (sepsis). At Stage 2, the disease is usually fatal within 2 or 3 years. [secure.ssa.gov]
Respiratoric
- Pneumonia
Published online: February 06, 2008 Issue release date: 1989 Number of Print Pages: 3 Number of Figures: 0 Number of Tables: 0 ISSN: 0014-3022 (Print) eISSN: 1421-9913 (Online) For additional information: https://www.karger.com/ENE Abstract Mycoplasma pneumoniae [karger.com]
Pneumocystis jiroveci pneumonia in a patient with Wegener's granulomatosis treated efficiently with caspofungin. Mycoses 2008;51 Suppl 1:65-7. 13. Dadoniene J, Pileckyte M, Baranauskaite A, Kirdaite G. [lungindia.com]
Keywords Covid-19, SARS-CoV2, Mononeuritis Multiplex Case Description We present the case of a 63-year-old female patient from Ipiales (Nariño, Colombia) with COVID-19 Pneumonia (positive PCR test on April 9, 2020, repeated on April 29, 2020, confirming [clinmedjournals.org]
He deteriorated with subsequent pneumonia, congestive heart failure and death four months after the initial neurologic presentation. [bcm.edu]
Gastrointestinal
- Nausea
The infection leads to nausea, vomiting, and diarrhea. A perianal rash (larva currens) may occur in the chronic stage. [aafp.org]
nausea, bone marrow suppression Oxycarbazepine 600-2400 mg Fatigue, nausea, dizziness, leukopenia Lamotrigine 50-500 mg Serious rash, dizziness, nausea, sedation Topiramate 25-400 mg Sedation, weight loss, nephrolithiasis, myopia, angle closure glaucoma [clevelandclinicmeded.com]
She also described neck stiffness along with her chief complaints but denied any nausea, vomiting, and changes in gait or memory. [cureus.com]
- Vomiting
The infection leads to nausea, vomiting, and diarrhea. A perianal rash (larva currens) may occur in the chronic stage. [aafp.org]
She also described neck stiffness along with her chief complaints but denied any nausea, vomiting, and changes in gait or memory. [cureus.com]
[citation needed] The signs and symptoms of autonomic neuropathy include the following: Urinary bladder conditions: bladder incontinence or urine retention Gastrointestinal tract: dysphagia, abdominal pain, nausea, vomiting, malabsorption, fecal incontinence [en.wikipedia.org]
Cardiovascular
- Hypertension
He was not a known hypertensive or diabetic. On examination, he had bilateral pedal edema and non-tender hepatomegaly extending 2 cm from the costal margin. There were no hypoesthetic patches. [neurologyindia.com]
Her past medical history was significant for hypertension, dyslipidemia, and surgically treated ovarian cancer Review of systems was positive for decreased appetite and postmenopausal bleeding. [shmabstracts.com]
Case Report A 71-year-old man with previous medical histories of hypertension, hemorrhoids, and glaucoma first presented with weight loss, hair loss, abdominal colic, altered taste, and diarrhea for a 4-month duration. [journal.frontiersin.org]
Significant pathological history: Hypertension and hypothyroidism. During her ICU hospitalization, she had kidney failure that required several dialysis sessions. Simultaneously, she showed persistent febrile syndrome and a neurological deficit. [clinmedjournals.org]
Musculoskeletal
- Arthritis
Arthritis Rheum. 1978; 21: 820-826 Scopus (29) PubMed Crossref Google Scholar Carson DA Lawrance S Catalano M Vaughan J Abraham G Radioimmunoassay of IgG and IgM rheumatoid arthritis reacting with human IgG. [amjmed.com]
Rheumatoid vasculitis is an example of the latter type of arteritis associated with high-titer 19S IgM rheumatoid factor typically occurring in patients with long-standing erosive rheumatoid arthritis. [ncbi.nlm.nih.gov]
The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988 ; 31 : 315 –24. 5 Maini RN, Breedveld FC, Kalden JR et al. [rheumatology.oxfordjournals.org]
J Rheum Dis -0001; (): Circulating leptin and its correlation with rheumatoid arthritis activity: a meta-analysis Young Ho Lee, M.D., Ph.D, Gwan Gyu Song, M.D., Ph.D. J Rheum Dis -0001; (): [jrd.or.kr]
- Osteoporosis
However, patients given ≥1 vs 0 AT had significantly higher Vasculitis Damage Indexes (VDI): 2.93±2.09 vs 1.96±1.40 (P<0.001), reflecting more frequent osteoporosis (33 vs 18%, P=0.013) or peripheral neuropathy (60 vs 38%, P=0.004). Figure 1. [acrabstracts.org]
Neurologic
- Peripheral Neuropathy
Peripheral neuropathy in Tangier disease. Brain. 1983; 106: 911-928 Albers J.W. Porphyric neuropathy. in: Mendell J.R. Kissel J.T. Cornblath D.R. Diagnosis and management of peripheral nerve disorders. [nmd-journal.com]
Home | CCC | Peripheral neuropathy DDx by, last update March 28, 2019 Overview Diseases that affect the the peripheral nerves, either motor or sensory. [lifeinthefastlane.com]
Mononeuritis multiplex is an extremely uncommon form of acquired peripheral neuropathy in childhood. We discuss the differential diagnosis and review previous descriptions of this rare condition. [ncbi.nlm.nih.gov]
Features of Textbook of Peripheral Neuropathy Include : ̈ Practical yet comprehensiveóan accessible ìgo-toî reference for clinicians ̈ Covers all clinically relevant peripheral neuropathies ̈ Clinical Pearls and Key Points are set off from the text for [books.google.ro]
- Foot Drop
Mononeuritis multiplex is a peripheral sensorimotor neuropathy usually producing foot or wrist drop, commonly associated with necrotizing arteritis of small and medium-sized arteries. [ncbi.nlm.nih.gov]
For example, sudden loss of the ability to lift the foot normally while walking (foot drop) can be caused by mononeuritis multiplex, when it is accompanied by loss of nerve function elsewhere in the body. [medicinenet.com]
Case 58A A Woman with Leg Numbness Pain Weakness and Slow Nerve Conduction Velocities 372 Case 58B A Boy with Leg Weakness 375 Case 59 A Man with Hand Weakness and Numbness After Skiing 383 Case 60 A Man with Recurrent Foot Drop 386 Case 61 An Elderly [books.google.com]
- Cranial Neuropathy
Generally, neuropathies can cause: Pain A tingling sensation Numbness Skin that feels sensitive to the touch Weak or paralyzed muscles Types of cranial neuropathies When a nerve located in the skull is damaged, it's called a cranial neuropathy. [columbianeurology.org]
More to come on cranial neuropathies. This is gonna take awhile! LOL [butudontlooksick.wordpress.com]
A case of neuro-Behcet's syndrome with recurrent multiple cranial neuropathy (in Japanese) Clin Neurol 1975, 15, 99–103. Google Scholar Fujiwara, N., Ishikawa. Sural nerves in Behcet's disease. [link.springer.com]
Vasculitis affecting the peripheral nerves commonly presents as subacute, progressive, asymmetric sensori-motor polyneuropathy or Mononeuritis multiplex, and sometimes as mono-neuropathy, pure sensoryneuropathy, cranial nerves neuropathy, or autonomic [elynsgroup.com]
Familial granulomatous synovitis, uveitis, and cranial neuropathies. Am J Med. 1985; 78: 801-804 Heininger U. Ries M. Christ P. Harms D. Simultaneous palsy of facial and vestibular nerve in a child with Lyme borreliosis. [nmd-journal.com]
- Meningism
We present an unusual case of a patient with scrub typhus who developed acalculous cholecystitis, aseptic meningitis and mononeuritis multiplex. The patient was successfully treated with oral minocycline. [ncbi.nlm.nih.gov]
[…] and mononeuritis multiplex, Page 1 of 1 < Previous page | Next page > /docserver/preview/fulltext/jmm/61/2/291_jmm034678-1.gif We present an unusual case of a patient with scrub typhus who developed acalculous cholecystitis, aseptic meningitis and mononeuritis [jmm.microbiologyresearch.org]
: The triad of neurologic manifestations of Lyme disease; meningitis, cranial neuritis and radiculo-neuritis. Neurology 35:47–53, 1985. PubMed Google Scholar [12] Simeoni J., Conci P., Kob K. [link.springer.com]
[…] infiltration Cytology: Abnormal in 80% of patients with ≥ 3 exams Lactic dehydrogenase β 2 -microglobulin Meningeal biopsy may be useful if other tests are negative Peripheral nervous system syndromes General: May be symmetric or asymmetric Mononeuropathies [neuromuscular.wustl.edu]
- Polyradiculoneuropathy
CMT) H Hypothyroidism Predominately motor peripheral neuropathy Guillain-Barré syndrome, chronic inflammatory polyradiculoneuropathy Hereditary motor and sensory neuropathy Diabetes mellitus Others-e.g. acute intermittent porphyria, lead poisoning, diphtheria [lifeinthefastlane.com]
HIV-induced neuropathy can occur rapidly, presenting as an AIDP (acute inflammatory demyelinating polyradiculoneuropathy) syndrome, usually near the time of seroconversion. [bcm.edu]
This pattern of involvement is termed a polyradiculoneuropathy. [clevelandclinicmeded.com]
- DOI - PubMed EFNS Task Force European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy (2010) Report of a joint task force of the European Federation [pubmed.ncbi.nlm.nih.gov]
These neurological events can occur at any point after the initiation of immunotherapy and involve a wide variety of neuropathies, neuromuscular disorders, and demyelinating polyradiculoneuropathies. [jmedicalcasereports.biomedcentral.com]
Workup
History, physical exam and overall clinical picture are critical in suggesting the presence of the disease. Laboratory tests are subsequently performed to determine the underlying etiologic cause. It is not necessary to perform imaging tests to establish the diagnosis.
Workup generally includes a complete blood count, c-reactive protein levels and erythrocyte sedimentation rate (ESR) to assess any possible systemic inflammatory disease, parvovirus B-19 serology, Borrelia burgdorferi serology when suspicion in Lyme disease is high, human immunodeficiency virus (HIV) serology in patients at risk, hepatitis serology, herpes viral serology and an autoimmune profile.
Some conditions that can result in mononeuritis multiplex can only be diagnosed with a nerve biopsy. Histopathological analysis will show infiltration of inflammatory cells within the perivascular compartment, damage to axonal structures, presence of multinucleated giant inflammatory cells and axonal loss [10]. Mononeuritis multiplex that is particularly associated with HIV manifests with necrotizing vasculitis in epineural arteries.
Microbiology
- Borrelia Burgdorferi
Abstract Lyme borreliosis is an infectious disease caused by the spirochete Borrelia burgdorferi. Neurological complications are frequently reported but the pathogenesis remains largely unknown. [ncbi.nlm.nih.gov]
Workup generally includes a complete blood count, c-reactive protein levels and erythrocyte sedimentation rate (ESR) to assess any possible systemic inflammatory disease, parvovirus B-19 serology, Borrelia burgdorferi serology when suspicion in Lyme disease [symptoma.com]
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 [slideshare.net]
Treatment
Treatment commences after the specific etiology underlying mononeuritis multiplex is diagnosed. Options considerably vary depending on the disease. Vasculitis, in particular, needs to be promptly treated to avoid serious complications that can lead to death [11] [12]. The aim of the treatment, aside from resolving the original condition that led to the development of the symptoms, is to provide supportive care for sufficient independent functioning and make sure symptoms are controlled. For example, in diabetes mellitus, it is necessary to keep blood sugar levels under close observation.
Supportive treatment can be administered in various forms. Most commonly, it consists of vocational and occupational therapy, in addition to providing devices that may help the patient in achieving mobility such as braces, splints, and wheelchairs. Physical therapy in the form of physical exercises can also improve overall muscular strength.
Overall safety management is also critical, as patients are more prone to falls and injuries due to the damage of motor and sensory function. Some safety measures that need to be followed are the following: use of railing whenever they are present, good lighting to avoid unnecessary accidents and adequate testing of the temperature of water before showering. It is also necessary to remove unnecessary physical obstacles such as loose rugs and to avoid wearing shoes with open toes or high heels.
Some patients may suffer from damage to sensations. In these cases, it is very important to regularly monitor the development of injuries or bruises, especially in the feet or areas affected by the disease. Without adequate examination, the areas may become infected without the awareness of the patient, because of deficient pain sensation.
On the other hand, some areas are particularly prone to damage due to excessive pressure such as the elbows and the knees. It is critical for the patient to avoid putting pressure on sensitive areas. Patients should be advised not to cross their legs as this increases pressure over the knees or lean on their elbows.
Pharmacotherapy plays an important part in the management of the disease. Lancinating pain may be treated with several anticonvulsants such as phenytoin, carbamazepine, pregabalin, and gabapentin. Antidepressants can also decrease pain, especially amitriptyline, duloxetine, and nortriptyline. Pharmacotherapy is not restricted to prescription drugs. Over the counter medications can be useful, particularly acetaminophen and non-steroidal anti-inflammatory agents such ibuprofen. Nonetheless, medications should be kept to a minimum in order to decrease side effects.
Patients are advised to improve their positioning to remove pressure over any area with damaged nerves. This helps in reducing pain. Unfortunately, autonomic dysfunction does not respond well to treatment.
Prognosis
Mononeuritis multiplex can completely resolve when the disease is diagnosed and treated promptly, although multiple recurrences can occur. Nonetheless, patients should expect months or sometimes years for resolution of their symptoms.
Overall morbidity depends on many factors and may range from absence of any disability to severe dysfunction. Mononeuritis multiplex can also be accompanied by a number of complications in the form of muscular atrophy, deformity of anatomical structures, recurrent injuries that the patient may not be aware of, organ dysfunction (particularly organs under autonomic control such as the heart, bladder and stomach), and psychological damage which can result in reduced confidence and social interaction. The latter can also occur because of excessive pain and motor dysfunction. Finally, impotence may develop in male patients.
Etiology
Mononeuritis multiplex results from damage to the axons, and ultimately resulting in deficits in the transmission of the electrical messages between nerve cells. Oxygen deficiency because of a decrease in blood flow as well as damage to the walls of blood vessels subsequent to inflammation are responsible for the development of the condition and target especially small arteries within the nerve structures.
Mononeuritis multiplex is associated with various processes and conditions. Infections are particularly important in the etiology of the disease. Several infectious disorders are known to be involved and they include hepatitis B and C, acute infection with hepatitis A, infections with herpes simplex virus, HIV infections with or without AIDS, Lyme disease, leprosy and acute infection with parvovirus B-19 [2]. Rheumatological diseases also play a prominent role in the pathology of the disease. Known associated rheumatological conditions are scleroderma, Sjogren syndrome, Wegener granulomatosis, Behcet disease, Henoch-Schönlein purpura, giant cell arteritis, rheumatoid arthritis, polyarteritis nodosa and systemic lupus erythematosus [3].
Other conditions that may be associated with mononeuritis multiplex are diabetes mellitus, neurosarcoidosis, amyloidosis, small cell lung cancer, graft versus host disease, tumor invasion of nerve structures (especially with B-cell leukemia, carcinoid tumor and lymphomas), cryoglobulinemia, hypereosinophilia, Churg-Strauss syndrome, idiopathic thrombocytopenic purpura, atopy-related peripheral neuritis, amphetamine angiitis and gasoline sniffing [4].
Patients with mononeuritis multiplex are usually susceptible to multiple recurrences, with worsening progression over time. Around 33% of all cases have unknown etiology [5].
Epidemiology
Mononeuritis multiplex is a rare disorder, although overall incidence and prevalence remain unknown. The most common causes of mononeuritis multiplex involve damage to blood vessels supplying neural structures, with the aged being the most affected. The mean age of onset of vasculitic neuropathy is between 51 and 67 years [6].
Reported prevalence in conjunction with the most important causative factors is the following [7]: 60% in polyarteritis nodosa, 8% in untreated hepatitis C (although this is likely to be over-estimated), 8% in Sjogren's syndrome, 40 to 50% in rheumatoid arthritis vasculitis, 5% to 20% in rheumatoid arthritis, 13% in Wegener's granulomatosis, 25% in Churg-Strauss syndrome, 65% in vasculitic neuropathies, 1% in systemic lupus erythematosus (SLE), 9% in patients with SLE but with evidence of vasculitis and a small association with Henoch Schönlein Purpura and temporal arteritis.
Pathophysiology
The pathophysiological processes underlying mononeuritis multiplex remain unknown. It is thought, however, that activated white blood cells and endothelial cells are central to the pathogenesis of the disease, in addition to the altered function of adhesion molecules. The common form of mononeuritis multiplex involves necrosis and inflammation of the small arteries supplying the nerve fibers. This eventually leads to random areas of ischemia. In addition, inflammation involving the full thickness of the vessel well in combination with fibrinoid necrosis can cause ischemia and injury to the nerve cells [8].
Other disease processes may also result in the development of the condition. They include the formation of immune complexes, especially in the context of polyarteritis nodosa and essential mixed cryoglobulinemia. This mechanism is also thought to be involved in polyarteritis nodosa associated with hepatitis B and cryoglobulinemia related to hepatitis C infection [9]. Furthermore, an exaggerated immune response may result from hapten formations. This mechanism is thought to play a particularly important role in vasculitis resulting from drug hypersensitivity. Finally, ANCA (antineutrophil cytoplasmic antibodies) production and exaggerated activation of T lymphocytes are thought to mediate the vasculitis in Wegener's granulomatosis and Churg-Strauss syndrome. ANCA are also likely to mediate damage in microscopic polyarteritis [9].
Prevention
There are no preventive measures that can be used for mononeuritis multiplex.
Summary
Mononeuritis multiplex presents with a constellation of symptoms that mostly target the peripheral nervous system. It results from a large array of medical conditions that ultimately affect the functioning of the nerves. The most common cause is a disruption of the vascular blood supply to the peripheral nerve structures, with the development of random areas of necrosis along the nerve fiber. Diabetes is one of the main culprits, in addition to infections, vasculitis and other rheumatological diseases [1]. Patients commonly present with severe episodes of localized and acute pain, with lancination. There may also be associated motor dysfunction, with weakness and atrophy. Workup is aimed at elucidating the cause and mostly consists of an array of laboratory tests. Imaging is not necessary to diagnose the disease, but in some cases, biopsy and pathological analysis are required. Treatment is aimed at controlling symptoms and resolving the underlying condition. Pharmacotherapy is particularly important in controlling the pain. Furthermore, patients also require social and psychological support. Prognosis varies considerably depending on the underlying etiology. Most patients can expect to recover, although multiple recurrences are very common.
Patient Information
Mononeuritis multiplex is a medical condition that manifests with damage to nerve structures in the body. Several underlying diseases can lead to the development of the condition, although deficient blood supply to peripheral nerve structure is the most common cause. This usually results from high levels of sugars in the blood, in addition to many infectious and inflammatory diseases. Patients usually present with sharp or deep pain occurring in sudden episodes, and may be associated with muscular weakness localized to particular muscles in the human body. Workup is aimed at elucidating underlying causes and generally consists of a broad range of blood tests. Imaging is not useful for diagnosing the disease, but sometimes the physician may take a sample of tissue for pathological analysis. This may reveal the cause in select conditions. Treatment is broad and is directed at the presenting symptoms, in addition to underlying causes. Patients can benefit from a range of medications that can help them with their pain. Most patient will do well, although outcomes vary a lot, depending on the disease.
References
- Kelkar P, Parry GJ. Mononeuritis multiplex in diabetes mellitus: evidence for underlying immune pathogenesis. J Neurol Neurosurg Psychiatry. 2003 Jun; 74(6):803-6.
- Elamin M, Alderazi Y, Mullins G, et al. Perineuritis in acute lyme neuroborreliosis. Muscle Nerve. 2009 Jun; 39(6):851-4.
- Koo J. The psychosocial impact of acne: patient's perceptions. J Am Acad Dermatol. 1995; 32:S26-S30.
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- Mauermann ML, Ryan ML, Moon JS, et al. Case of mononeuritis multiplex onset with rituximab therapy for Waldenstrom's macroglobulinemia. J Neurol Sci. 2007 Sep 15; 260(1-2):240-3.
- Jansen T, Plewig G. Acne fulminans. Int J Dermatol. 1998; 37:254-257.
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- Wilkins JW, Voorhees JJ. Prevalence of nodulocystic acne in white and Negro males. Arch Dermatol. 1970; 102:631-634.
- Bennett DL, Groves M, Blake J, et al. The use of nerve and muscle biopsy in the diagnosis of vasculitis: a 5 year retrospective study. J Neurol Neurosurg Psychiatry. 2008 Dec; 79(12):1376-81.
- Gorson KC. Vasculitic neuropathies: an update. Neurologist. 2007 Jan; 13(1):12-9.
- Garzoni L, Vanoni F, Rizzi M, et al. Nervous system dysfunction in Henoch-Schonlein syndrome: systematic review of the literature. Rheumatology (Oxford). 2009 Dec; 48(12):1524-9.