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 ◽  
...  

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.

2020 ◽  
Vol 114 (11) ◽  
pp. 792-797
Author(s):  
Diogo Fernandes dos Santos ◽  
Douglas Eulálio Antunes ◽  
Bruno Carvalho Dornelas ◽  
Bruno Araujo da Cunha ◽  
Thales Junqueira Oliveira ◽  
...  

Abstract Background The early recognition of neural impairment in leprosy, especially in primary neural forms, represents a challenge in clinical practice and a peripheral nerve biopsy may be required for diagnostic confirmation. This study aims to characterize the epidemiological, clinical, electroneuromyographic, laboratory and histopathological aspects of patients undergoing peripheral nerve biopsy during investigation of primary neural cases in leprosy. Methods A total of 104 patients with peripheral neuropathy who were referred to a national reference centre for leprosy were biopsied from 2014 to 2018. All cases underwent clinical, laboratory, histopathological and electroneuromyographic evaluations. Results Of 104 biopsied patients, leprosy was confirmed in 89.4% (93/104). The biopsied nerves were the ulnar (67.8% [63/93]), superficial fibular (21.5% [20/93]), sural (8.6% [8/93]), radial (1.1% [1/93]) and deep fibular (1.1% [1/93]). Twenty-nine percent (27/93) presented histopathological abnormalities and 4.4% (4/93) presented acid-fast bacilli. Nerve and superjacent skin quantitative polymerase chain reaction were positive in 49.5% (46/93) and 24.8% (23/93) of cases, respectively. Patients with multiple mononeuropathy had a higher frequency of histopathological abnormalities (p=0.0077). Conclusions This study reinforces peripheral nerve biopsy's role as an important tool in the investigation of primary neural cases, contributing to the early diagnosis and also reducing diagnostic errors and the need for empirical treatment.


2021 ◽  
Author(s):  
Alencar Pereira dos Santos ◽  
Diogo Fernandes dos Santos ◽  
Isabela Maria Bernardes Goulart ◽  
Thales Junqueira Oliveira ◽  
Isabella Sabião Borges ◽  
...  

Background: The long incubation period of leprosy, its insidious signs and symptoms produce difficulties in its diagnosis and correct clinical classification. The early recognition of neural impairment in leprosy, especially in household contacts with subclinical infection and in the primary neural form, in which the classic clinical and laboratory findings of the disease are, by definition, absent, represents a major challenge in clinical practice. Objectives: Characterize the clinical, molecular, serological and neurophysiological aspects in the early diagnosis of leprosy neuropathy, in household contacts with subclinical infection (positive ELISA anti-PGL1 serology. Design and setting: Longitudinal study carried out at the Clinics Hospital - Federal Univeristy of Uberlândia, a center specialized in Leprosy/Sanitary Dermatology. Methods: 361 seropositive household contacts (CDSP), defined as subclinical infection, were recruited, followed up at a national referral center for leprosy in Brazil, from 2014 to 2016. All individuals underwent a clinical, laboratory and neurophysiological evaluation. Results: 361 CDSP were evaluated. The qPCR analysis was positive in 35.5% (128/361) in the dermal shaving and in 25.8% (85/361) in the skin biopsy of the CDSP. In the electroneuromyographic evaluation, 23.5% (93/361) of the CDSP showed signs of neural involvement, with an average of 2.1 nerves compromised by CDSP. 62.3% (53/93) presented a pattern of mononeuropathy in ENMG. Conclusions: Annual monitoring of CDSP, a prevalence of peripheral neural impairment assessed by ENMG, favoring early treatment.


2018 ◽  
pp. 69-74
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand

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.


2000 ◽  
Vol 124 (1) ◽  
pp. 114-118
Author(s):  
Bijal Rajani ◽  
Vakesh Rajani ◽  
Richard A. Prayson

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.


2021 ◽  
Vol 15 (8) ◽  
pp. 2487-2490
Author(s):  
Asmat Ullah ◽  
Mirwais Kakar ◽  
Bilal Ahmed ◽  
Sadia Jabbar ◽  
Inayat Ullah

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


2021 ◽  
Author(s):  
Patricia Penna ◽  
Robson Vital ◽  
IZABELA PITTA ◽  
Mariana Hacker ◽  
Ana Salles ◽  
...  

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.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jana Müller ◽  
Charlotte Kreutz ◽  
Steffen Ringhof ◽  
Maximilian Koeppel ◽  
Nikolaus Kleindienst ◽  
...  

AbstractImpaired postural control is often observed in response to neurotoxic chemotherapy. However, potential explanatory factors other than chemotherapy-induced peripheral neuropathy (CIPN) have not been adequately considered to date due to primarily cross-sectional study designs. Our objective was to comprehensively analyze postural control during and after neurotoxic chemotherapy, and to identify potential CIPN-independent predictors for its impairment. Postural control and CIPN symptoms (EORTC QLQ-CIPN20) were longitudinally assessed before, during and three weeks after neurotoxic chemotherapy, and in three and six months follow-up examinations (N = 54). The influence of peripheral nerve function as determined by nerve conduction studies (NCS: compound motor action potentials (CMAP) and sensory action potentials (SNAP)), physical activity, and muscle strength on the change in postural control during and after chemotherapy was analyzed by multiple linear regression adjusted for age and body mass index. Postural control, CIPN signs/symptoms, and CMAP/SNAP amplitudes significantly deteriorated during chemotherapy (p < .01). During follow-up, patients recovered from postural instabilities (p < .01), whereas CIPN signs/symptoms and pathologic NCS findings persisted compared to baseline (p < .001). The regression model showed that low CMAP and high SNAP amplitudes at baseline predicted impairment of postural control during but not after chemotherapy. Hence, pre-therapeutically disturbed somatosensory inputs may induce adaptive processes that have compensatory effects and allow recovery of postural control while CIPN signs/symptoms and pathologic peripheral nerve function persist. Baseline NCS findings in cancer patients who receive neurotoxic chemotherapy thus might assist in delineating individual CIPN risk profiles more precisely to which specific exercise intervention programs could be tailor-made.


Author(s):  
Kelvin Miu

Laryngeal cancer is a common head and neck cancer and typically presents with voice hoarseness in patients older than 60 years. Early recognition of signs and symptoms of laryngeal cancer can lead to early diagnosis and treatment, therefore improving patient outcomes. This article aims to provide an overview of the anatomy of the larynx, presentation and management of laryngeal cancer, and common follow-up problems.


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