Presentation
An Unusual Case of Henoch Schönlein Purpura Presenting as Mononeuritis Multiplex Download PDF Published Date: August 25, 2016 An Unusual Case of Henoch Schönlein Purpura Presenting as Mononeuritis Multiplex Deepak Goyal 1*, Neelam Mohan 1, and Jyoti Sehgal [elynsgroup.com]
Cryoglobulinemic vasculitis Cryoglobulinemic vasculitis presents with rash over lower limbs, arthralgia, Raynaud’s phenomenon and neuropathy Polyarteritis nodosa Polyarteritis nodosa presents with fever, myalgia, arthralgia, multisystem involvement and [pgblazer.com]
Peripheral neuropathy (especially mononeuritis multiplex) can be present at 10. Mononeuritis and mononeuritis multiplex can involve pain, palsy and radiculopathy. [medical-dictionary.thefreedictionary.com]
Reports of initial presentation with MM are rare [ 3 ],[ 8 ],[ 9 ]. [bmcneurol.biomedcentral.com]
A 40-year-old woman presented with progressive lower leg pain and spontaneous toe movement. The EMG showed a posterior tibial nerve mononeuropathy and continuous myokymic discharges in posterior tibial-innervated muscles. [ncbi.nlm.nih.gov]
Entire Body System
- Episodic Weakness
Episodic Weakness 464 Case 73B An African American Man with Hyperthyroidism and Acute Paralysis 470 Case 74 A Boy with Large Muscles Leg Pain and Elevated Serum Creatine Kinase 472 Case 75 A Woman with Proximal Muscle Weakness Calf Hypertrophy Heel Contractures [books.google.com]
- Infectious Mononucleosis
Methods: Case report Results: We are reporting a case of a 35-year-old man who presented with mononeuritis multiplex following an infectious mononucleosis associated with a mixed cryoglobulinemia. [cambridge.org]
Respiratoric
- Sputum
Sputum for AFB was negative on two occasions. Chest X-ray PA view revealed consolidation and nodular shadows in both lower zones [Figure 2]. [lungindia.com]
Skin
- Hyperpigmentation
He had developed spontaneously resolving hyperpigmentation over forehead, back, hands, and legs and erythematous rashes over the trunk 3 months earlier followed by gradual onset of numbness and weakness in both legs and difficulty in buttoning. [annalsofian.org]
Neurologic
- Thalamic Pain
Get a broader perspective on your field from new chapters on Labral Tears of the Shoulder and Hip, Pubalgia, Chondral Injuries, Central Post-Stroke Pain (Thalamic Pain Syndrome), Chemotherapy-induced Peripheral Neuropathy, Radiation Fibrosis Syndrome, [books.google.de]
- Myokymia
Toe movements and myokymia were unaffected by the proximal transection of the lesion but ceased abruptly when the distal end of the fusiform "tumor" was resected, suggesting that spontaneous electrical foci may have been located along the nerve lesion [ncbi.nlm.nih.gov]
Workup
What is the medical workup for peripheral neuropathy? Every workup for peripheral neuropathy initially consists of a careful medical history and comprehensive physical examination. [scottsdaleneuropathyinstitute.com]
Laboratory workup was normal and immunological tests were negative. [neurologyindia.com]
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]
Investigations Blood workup revealed normocytic anemia, thrombocytosis and increased erythrocyte sedimentation rate (ESR) (110 mm/h), C-reactive protein (CRP) (33 mg/L) and angiotensin converting enzyme (ACE) (74 units/L). [bmcneurol.biomedcentral.com]
Treatment
and then every month while treatment continues After the end of treatment U/A should be checked at least every 6 months. [online.epocrates.com]
These include: a wheelchair braces supports corrective shoes Physical Therapy Your doctor may recommend physical therapy as part of your treatment plan. [healthline.com]
We also noted marked improvement in pain in these patients with treatment. One patient failed to respond to treatment initially, but had eventual improvement over a two year period. [jnnp.bmj.com]
See Table III for specific treatment requirements based on etiology of mononeuritis multiplex. Table III. [clinicaladvisor.com]
Practical, clinically relevant material facilitates the diagnosis and treatment of musculoskeletal, pain, and chronic disabling conditions. [books.google.de]
Prognosis
Prognosis The prognosis of patients with mononeuritis multiplex (MNM) depends on the underlying etiology. [online.epocrates.com]
Probability of survival without prescription of new add-on therapies of 193 patients with EGPA, PAN or MPA without baseline poor-prognosis factors (FFS=0). P determined with log-rank test. [acrabstracts.org]
These neuropathies were severe and relapsing, in contrast to a usually benign prognosis. [jrheum.org]
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]
[…] macroglobulinemia (WM)/Lymphoplasmocytic lymphoma (LPL) Mantle Cell Hodgkin (10%) Epidemiology: Younger patients Clinical Spread: Less likely to be extra-nodal than NHL Associated polyneuropathies Demyelinating Drug-induced ( Checkpoint inhibitors ) Prognosis [neuromuscular.wustl.edu]
Etiology
Assess for etiology A thorough history should be undertaken to assess for the cause of MNM. [clinicaladvisor.com]
Overall mortality in MNM is not known but is likely to depend on the underlying etiology. [online.epocrates.com]
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]
Diagnostic tests, usually beginning electrodiagnostic testing, are indicated in patients with atypical clinical features, unknown etiology, and/or severe or rapidly progressive symptoms. [amboss.com]
With a systematic approach to the evaluation of these disorders, approximately 75% of patients have a specific etiologic diagnosis. [clevelandclinicmeded.com]
Epidemiology
Mononeuritis Multiplex Lymphoma 5 Lymphoma Features Epidemiology: Most common in industrialized countries Lymphoma Types Non-Hodgkin type (NHL) (90%) Defined by lymphocyte characteristics B-cells (85%): Associated polyneuropathies Aggressive Type: Diffuse [neuromuscular.wustl.edu]
[…] neuropathies such as migraine, encephalopathy, chorea, brain stem dysfunction, myelopathy, mononeuritis multiplex, Guillain-Barre-like syndrome, and antiganglioside-positive neuropathy with antibodies, strong associations remain largely unconfirmed in epidemiologic [diki.pl]
Leprosy now: epidemiology, progress, challenges, and research gaps. Lancet Infect Dis, 11 (2011), pp. 464-470 [6] W. Winnie, J. Srinivasan. Leprosy and the peripheral nervous system: basic and clinical aspects. [elsevier.es]
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]
Pathophysiology
Pathophysiologically there are tau-positive inclusions. There is no cure. [mrcpandme.blogspot.com]
Their presentation varies depending on the underlying pathophysiology. The speed of progression of the polyneuropathy coupled with its nature (axonal or demyelinating) can help identify its aetiology. [rcemlearning.co.uk]
A larger study is required to explore the pathophysiology, natural history, and possible treatment options for patients with mononeuritis multiplex in diabetes. [jnnp.bmj.com]
Back to Top Pathophysiology and Natural History Despite the diverse array of medical disorders that cause peripheral neuropathies, peripheral nerves exhibit only a few distinct pathologic reactions to an insult or disease: wallerian degeneration, axonal [clevelandclinicmeded.com]
[…] 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
Knowledge of the pathogenesis, genetics, and molecular biology of neuromuscular disorders is essential both in developing and applying new therapies and preventive measures, and in formulating genetic and prognostic advice. [books.google.com]
Prevention of complications Education, regular foot inspection, chiropody, soft shoes, and orthotics are important to avoid foot ulcers in patients with distal polyneuropathy. [rcemlearning.co.uk]