A Case of Fever and a Wrist Drop

2018 ◽  
pp. 69-74
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Nervous System ◽  
Gastrointestinal Tract ◽  
Peripheral Neuropathy ◽  
Peripheral Nerve ◽  
Polyarteritis Nodosa ◽  
Joint Pain ◽  
Immunosuppressive Agents ◽  
Nerve Biopsy ◽  
Definitive Diagnosis ◽  
Wrist Drop

Polyarteritis nodosa (PAN) is a systemic illness that most often involves the skin, kidneys, and gastrointestinal tract, in addition to the peripheral nervous system. It most often affects middle-aged individuals, men more than women. The particular type of peripheral neuropathy is often mononeuropathy or mononeuritis multiplex—a condition in which there is discrete involvement of multiple individual nerves. Definitive diagnosis of the vascular nature of the neuropathy requires peripheral nerve biopsy, which will demonstrate inflammation throughout the walls of small and medium-sized arteries. The CNS is also frequently involved in PAN. Constitutional symptoms, including fever, fatigue, and diffuse muscle and joint pain, are common and raise the suspicion of a systemic illness. Treatment generally involves the use of corticosteroids and immunosuppressive agents such as cyclophosphamide or azathioprine.

scholarly journals Peripheral nerve biopsy: Is it still important for the early diagnosis of neural leprosy?

2021 ◽  
Author(s):  
Isabella Sabião Borges ◽  
João Victor Aguiar Moreira ◽  
Thales Junqueira Oliveira ◽  
Maria Fernanda Prado Rosa ◽  
Gabriel Nunes Melo Assunção ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Early Diagnosis ◽  
Peripheral Nerve ◽  
Clinical Evidence ◽  
Early Recognition ◽  
Diagnostic Errors ◽  
Nerve Biopsy ◽  
Skin Lesions ◽  
National Reference Center ◽  
Neural Impairment

Background: The early recognition of neural impairment in leprosy represents a challenge in clinical practice and peripheral nerve biopsy may be required for diagnostic. Objective: Characterize the epidemiological, clinical, electroneuromyographic, laboratory and histopathological aspects of patients undergoing peripheral nerve biopsy during investigation of primary neural leprosy. Methods: 104 patients with peripheral neuropathy, referred to a national reference center leprosy, were biopsied. All patients had clinical evidence of peripheral neuropathy associated with the absence of skin lesions and were being investigated. Results: Of 104 biopsied, leprosy was confirmed in 89.4%. 66 were classified as primary neural leprosy and 27 as neural relapse or reinfection. All cases confirmed presented asymmetric neural impairment with predominance of sensory symptoms (88.2%), followed by muscular weakness and/or amyotrophy in 44.1% and pain in 34.4%. Neural thickening of one or more nerves was observed in 78.5% of the patients. The biopsied nerves were: ulnar (67.8%), superficial fibular (21.5%), sural (8.6%), radial (1.1%) and deep fibular (1.1%). 29% presented histopathological abnormalities and 4.4% acid fast bacilli. Nerve and superjacent skin qPCR were positive in 49.5% and 24.8% of cases, respectively. The patients with multiple mononeuropathy presented higher frequency of neural thickening (p<0.0001) and histopathological abnormalities (p=0.0077), but lower rates of positivity of ELISA anti-PGL-I (p=0.0100), qPCR in the peripheral blood (p=0.0157), and in the slit skin smear (p=0.0032). Conclusions: Peripheral nerve biopsy is an important tool in the investigation of primary neural cases, contributing to the early diagnosis and reducing diagnostic errors and the need for empirical treatment.

Peripheral Nerve Amyloidosis in Sural Nerve Biopsies

2000 ◽  
Vol 124 (1) ◽  
pp. 114-118
Author(s):  
Bijal Rajani ◽  
Vakesh Rajani ◽  
Richard A. Prayson
Keyword(s):  
Peripheral Neuropathy ◽  
Peripheral Nerve ◽  
Sural Nerve ◽  
Axonal Degeneration ◽  
Nerve Biopsy ◽  
Axonal Loss ◽  
Autonomic Symptoms ◽  
Pathologic Features ◽  
Ultrastructural Appearance ◽  
Muscle Biopsies

Abstract Objective.—Amyloidosis is a well-recognized but uncommon cause of peripheral neuropathy. Our objectives were to determine the overall prevalence of peripheral nerve amyloidosis in sural nerve biopsies and to evaluate the clinical and pathologic features of these lesions. Methods.—All available histologic and ultrastructural materials on biopsy tissue from 13 cases of peripheral nerve amyloidosis were examined. Muscle biopsies performed at the same time as the nerve biopsy were reviewed when available. Clinical data were collected on all patients. Results.—The prevalence of amyloidosis in sural nerve biopsies at our institution was 13 (1.2%) of 1098 cases over a 15.8-year period. These patients ranged in age from 41 to 82 years (median, 61 years) at initial presentation and included 10 men and 3 women. Presenting neuropathy symptoms were sensory in 6 of the 13 patients, motor in 2 cases, and mixed in 5 cases. Cardiac, renal, or gastrointestinal involvement was present in 7 of 13 cases. Two patients had myeloma and 7 had systemic autonomic symptoms. Two patients had probable familial amyloid polyneuropathy, and 1 patient demonstrated an alanine 60 point mutation. Amyloid, identified as amorphous eosinophilic extracellular deposits demonstrating apple green birefringence on Congo red stain or recognized by its characteristic fibrillar ultrastructure by electron microscopy, was identified in the endoneurium in 12 nerves, perineurium in 2 nerves, and epineurium in 9 nerves. Chronic inflammation was identified in 5 nerves. Axonal loss was recorded as mild (&lt;25%) in 1 nerve, moderate (25% to 75%) in 8 nerves, and severe (&gt;75%) in 4 nerves. Axonal degeneration predominated over demyelination in 8 of 10 cases that could be evaluated. Concomitant muscle biopsies contained amyloid deposits in 8 of 9 cases. Conclusions.—Amyloidosis is a rare (1.2% in our series) cause of peripheral neuropathy with a distinct microscopic and ultrastructural appearance. Just over half the patients in our study had visceral organ involvement and systemic autonomic symptoms. The peripheral neuropathy was associated with axonal degeneration and a moderate to severe axonal loss in the majority of cases. Amyloid deposition was present in 8 out of 9 muscle biopsies performed at the same time.

Confusion, Muscle Cramps, and Weight Loss

2018 ◽  
pp. 127-132
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Weight Loss ◽  
Nervous System ◽  
Neuropathic Pain ◽  
Peripheral Nerve ◽  
Immunosuppressive Agents ◽  
Muscle Cramps ◽  
Morvan’S Syndrome ◽  
Peripheral Nerve Hyperexcitability ◽  
Core Symptoms ◽  
Cerebellar Symptoms

Morvan’s syndrome is a rare autoimmune antibody syndrome causing central, peripheral, and autonomic manifestations. It has been most consistently associated with Caspr2 antibodies, although LGI1 antibodies have also been found in some patients. The Caspr2 protein is located throughout the central and peripheral nervous system. This may account for the diversity of presentations that have been reported in patients with Caspr2 antibodies. The seven core symptoms of Caspr2 antibodies include cognitive dysfunction/seizures, cerebellar symptoms, peripheral nerve hyperexcitability, autonomic dysfunction, insomnia, neuropathic pain, and weight loss. Treatment of Morvan’s syndrome and other Caspr-associated antibody syndromes is largely empirical. Typically, patients are given steroids, intravenous immunoglobulin, and if necessary, other immunosuppressive agents. Prognosis for remission is very good, but relapses may occur.

scholarly journals Association of Peripheral Neuropathy in Hepatitis C Infection with and without Cryoglobulineamia

2021 ◽  
Vol 15 (8) ◽  
pp. 2487-2490
Author(s):  
Asmat Ullah ◽  
Mirwais Kakar ◽  
Bilal Ahmed ◽  
Sadia Jabbar ◽  
Inayat Ullah
Keyword(s):  
Hepatitis C ◽  
Peripheral Neuropathy ◽  
Peripheral Nerve ◽  
Nerve Biopsy ◽  
Cross Sectional Study ◽  
Sural Nerve Biopsy ◽  
Cranial Neuropathy ◽  
Hepatitis C Infection ◽  
Cross Sectional ◽  
Peripheral Nerve Involvement

Objective: The purpose of this study was to find out the association of peripheral neuropathy in hepatitis C infection with and without cryoglobulineamia. Study Design: Cross sectional study Place and Duration: Conducted in Liver Transplant Unit, Gambat Institute of Medical Sciences, Gambat Khairpur Mirs, Sindh for the duration of six months from November 2020 to April 2021. Methods: Total 50 patients who had hepatitis C infection and peripheral neuropathy were included in this study. Patients were aged between 18- 60 years. Detailed demographics of patients including age, sex and body mass index were recorded after taking informed written consent. When symptoms and evidence of peripheral sensory or motor involvement were evident, clinical neuropathy was diagnosed. Sural nerve biopsy was done on patients and the biopsy specimen was evaluated morphologically and morphometrically. Multiple neuropathy, cranial neuropathy, and polyneuropathy are all terms used to describe peripheral nerve involvement. Our research focused on the motor conduction of the median, ulnar, and common peroneal nerves, measuring MCV, CMAP amplitude, and distal latency (DL) in both patients with and without cryoglobulinaemia for each nerve. The SPSS 20.0 version was used to analyze the data. Results: Mean age of the patients was 46.23±9.87 years with mean BMI 29.16±11.27 kg/m2. There were 30 (60%) females and 20 (40%) were males. We found that 35 (70%) patients had CG involvement with peripheral neuropathy and 15 (30%) cases were without CG. Prevalence of polyneuropathy was higher 19 (54.3%) in CG patients as compared to non CG 2 (13.3%). Mononeuropathy or multiple neuropathy was higher in HCV CG patients 13 (37.1%) as compared to HCV non CG patients 4 (26.7%). 25 patients underwent nerve biopsy (20 CG patients and 5 non CG). Prevalence of epineurial vasculitis and fascicular loss of axons was higher in non CG patients while demyelination + axonal degeneration were prevalent in CG patients. MCV of the deep peroneal nerve in patients with CG+ was low as compared to CG. Even though no statistically significant differences were detected, the other neurophysiological measures pointed to a more extensive and severe involvement of peripheral nerve in CG+ patients. Conclusion: We concluded in this study that the association of peripheral neuropathy in HCV patients with cryoglobulinaemia was greater as compared to non-CG HCV patients. It appears that both CG+ and CG patients suffer from peripheral nerve injury via a vasculitic mechanism, as evidenced by clinical and morphological observations. Serum CG levels indicate a more severe and broad neuropathic involvement, however research suggests that cryoglobulins are not the only element in the vasculitic process. Keywords: Cryoglobulinemia, Peripheral Neuropathy, HCV

scholarly journals Neurotoxic Agents and Peripheral Neuropathy

2021 ◽  
Author(s):  
Neslihan Eskut ◽  
Asli Koskderelioglu
Keyword(s):  
Heavy Metals ◽  
Nervous System ◽  
Peripheral Neuropathy ◽  
Peripheral Nerve ◽  
Peripheral Nervous System ◽  
Treatment Options ◽  
Nerve Fibers ◽  
Pattern Of Injury ◽  
Extrapyramidal Signs ◽  
Neurobehavioral Symptoms

Neurotoxicity may develop with exposure to various substances such as antibiotics, chemotherapeutics, heavy metals, and solvents. Some plants and fungi are also known to be neurotoxic. Neurotoxicity can develop acutely within hours, or it can develop as a result of exposure for years. Neurotoxicity can be presented with central or peripheral nervous system findings such as neurobehavioral symptoms, extrapyramidal signs, peripheral neuropathy. Peripheral nerve fibers are affected in different ways by neurotoxicant injury. The pattern of injury depends on the target structure involved. The focus of this chapter includes signs, symptoms, pathophysiology, and treatment options of neurotoxicity.

scholarly journals Persistent Neuropathy in Lepromatous Leprosy Patients

2021 ◽  
Author(s):  
Patricia Penna ◽  
Robson Vital ◽  
IZABELA PITTA ◽  
Mariana Hacker ◽  
Ana Salles ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Peripheral Nerve ◽  
Nerve Biopsy ◽  
Lepromatous Leprosy ◽  
Nerve Damage ◽  
Sensory Nerves ◽  
High Dose ◽  
Peripheral Nerve Damage ◽  
End Of Treatment ◽  
Leprosy Patients

Abstract Lepromatous leprosy (LL) patients have evidence of extensive peripheral nerve damage as soon as a diagnosis is made, but most of them have few or no symptoms related to peripheral neuropathy. Usually, they do not have the cardinal signal of leprosy neuritis. However, disability caused by peripheral nerve injuries has consequences throughout the entire life of these patients and the pathophysiological mechanisms of nerve damage are still poorly understood. The objective of this study was to evaluate the outcome of peripheral neuropathy in a group of LL patients in an attempt to understand the mechanisms of nerve damage. We evaluated medical records of 14 LL patients that had undergone a neurological evaluation at the beginning of Leprosy treatment then worsened at least 4 years after the end of treatment and underwent nerve biopsy. The symptoms at the beginning of treatment were compared with those at the time of the biopsy. Pain was a symptom in only one patient at the beginning and was a complaint in 9 patients by the time of biopsy. Neurological examination showed that the majority of patients already had alterations in medium and large caliber fibers at the beginning of the treatment, and pain increased by the time of biopsy, while neurological symptoms and signs deteriorated independently of the use of prednisone or thalidomide. Nerve Conduction Studies demonstrated that sensory nerves were the most affected. LL patients can develop a silent progressive degenerative peripheral neuropathy, which continues to develop despite high dose long term corticoid therapy.

scholarly journals Reducing monocarboxylate transporter MCT1 worsens experimental diabetic peripheral neuropathy

2020 ◽  
Author(s):  
Mithilesh Kumar Jha ◽  
Xanthe Heifetz Ament ◽  
Fang Yang ◽  
Ying Liu ◽  
Michael J. Polydefkis ◽  
...  
Keyword(s):  
Nervous System ◽  
Peripheral Neuropathy ◽  
Peripheral Nerve ◽  
Diabetic Peripheral Neuropathy ◽  
Peripheral Nerve Regeneration ◽  
Metabolic Energy ◽  
Monocarboxylate Transporter ◽  
Diabetic Patients ◽  
Wild Type ◽  
Monocarboxylate Transporter 1

AbstractDiabetic peripheral neuropathy (DPN) is one of the most common complications in diabetic patients. Though the exact mechanism for DPN is unknown, it clearly involves metabolic dysfunction and energy failure in multiple cells within the peripheral nervous system (PNS). Lactate is an alternate source of metabolic energy that is increasingly recognized for its role in supporting neurons. The primary transporter for lactate in the nervous system, monocarboxylate transporter-1 (MCT1), has been shown to be critical for peripheral nerve regeneration and metabolic support to neurons/axons. In this study, MCT1 was reduced in both sciatic nerve and dorsal root ganglia in wild-type mice treated with streptozotocin (STZ), a common model of type-1 diabetes. Heterozygous MCT1 null mice treated with STZ developed a more severe DPN compared to wild-type mice, as measured by greater axonal demyelination, decreased peripheral nerve function, and increased numbness to innocuous low-threshold mechanical stimulation. Given that MCT1 inhibitors are being developed as both immunosuppressive and chemotherapeutic medications, our results suggest that clinical development in patients with diabetes should proceed with caution. Collectively, our findings uncover an important role for MCT1 in DPN and provide a potential lead toward developing novel treatments for this currently untreatable disease.

Polyarteritis Nodosa

2013 ◽  
Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Gastrointestinal Tract ◽  
Peripheral Nervous System ◽  
Diagnostic Criteria ◽  
Polyarteritis Nodosa ◽  
Treatment Recommendations ◽  
General Treatment ◽  
Neurological Manifestations

Polyarteritis nodosa (PAN) is a systemic illness that most often involves the peripheral nervous system, skin, kidneys, and gastrointestinal tract. In this chapter, we review the diagnostic criteria for PAN, its most common neurological manifestations affecting the peripheral nervous system as well as central nervous system presentations, and discuss general treatment recommendations.

scholarly journals Immunoglobulin G4-related disease presenting with peripheral neuropathy: a case report

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jeong Bin Bong ◽  
Dong Kun Lee ◽  
Min A Lee ◽  
Byoung Wook Hwang ◽  
Hyun Goo Kang
Keyword(s):  
Nervous System ◽  
Peripheral Neuropathy ◽  
Peripheral Nerve ◽  
Plasma Cells ◽  
High Serum ◽  
High Dose ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Immune Mediated ◽  
Serum Igg4

Abstract Background Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition characterized by high serum IgG4 concentrations and tissue infiltration by IgG4-positive plasma cells. Reports have demonstrated that IgG4-RD affects various organs, including the pancreas, kidney, lung, thyroid, and lacrimal and salivary glands. In the nervous system, hypertrophic pachymeningitis and hypophysitis are mainly related to IgG4-RD; however, the peripheral neuropathy involvement is unusual. Case presentation We report on a 69-year-old woman with multiple mononeuropathy, weight loss and kidney mass in the setting of IgG4-RD. Biopsies of the kidney mass showed lymphoplasmacytic sclerosing inflammation with numerous IgG4-positive plasma cells. IgG4 and IgG4/IgG ratios in the blood were elevated. The patient was treated with high dose methylprednisolone with improvement in her neuropathy. Conclusions IgG4-RD is a relatively recently reported systemic fibrous inflammatory disease caused by the infiltration of IgG4-positive plasma cells in various organs. In the nervous system, symptomatic peripheral nerve invasion is very rare. However, as demonstrated in our case, IgG4-RD may present with primarily peripheral nerve disease.

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