Prion-related peripheral neuropathy in sporadic Creutzfeldt-Jakob disease

2018 ◽  
Vol 90 (4) ◽  
pp. 424-427 ◽  
Author(s):  
Simone Baiardi ◽  
Veronica Redaelli ◽  
Paolo Ripellino ◽  
Marcello Rossi ◽  
Alessia Franceschini ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Western Blotting ◽  
Clinical Symptoms ◽  
Nerve Biopsy ◽  
Plaque Type ◽  
Clinical Onset ◽  
Phenotypic Spectrum ◽  
Neurophysiological Data ◽  
Jakob Disease ◽  
Strain Dependent

ObjectiveTo assess whether the involvement of the peripheral nervous system (PNS) belongs to the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease (sCJD).MethodsWe examined medical records of 117 sCJDVV2 (ataxic type), 65 sCJDMV2K (kuru-plaque type) and 121 sCJDMM(V)1 (myoclonic type) subjects for clinical symptoms, objective signs and neurophysiological data. We reviewed two diagnostic nerve biopsies and looked for abnormal prion protein (PrPSc) by western blotting and real-time quaking-induced conversion (RT-QuIC) in postmortem PNS samples from 14 subjects.ResultsSeventy-five (41.2%) VV2-MV2K patients, but only 11 (9.1%) MM(V)1, had symptoms or signs suggestive of PNS involvement occurring at onset in 18 cases (17 VV2-MV2K, 9.3%; and 1 MM(V)1, 0.8%) and isolated in 6. Nerve biopsy showed a mixed predominantly axonal and demyelinating neuropathy in two sCJDMV2K. Electromyography showed signs of neuropathy in half of the examined VV2-MV2K patients. Prion RT-QuIC was positive in all CJD PNS samples, whereas western blotting detected PrPSc in the sciatic nerve in one VV2 and one MV2K.ConclusionsPeripheral neuropathy, likely related to PrPSc deposition, belongs to the phenotypic spectrum of sCJDMV2K and VV2 and may mark the clinical onset. The significantly lower prevalence of PNS involvement in typical sCJDMM(V)1 suggests that the PNS tropism of sCJD prions is strain dependent.

scholarly journals Acupuncture in diabetic peripheral neuropathy—protocol for the randomized, multicenter ACUDPN trial

Trials ◽  
2021 ◽  
Vol 22 (1) ◽  
Author(s):  
J. Dietzel ◽  
S. Hörder ◽  
I. V. Habermann ◽  
G. Meyer-Hamme ◽  
K. Hahn ◽  
...  
Keyword(s):  
Neuropathic Pain ◽  
Peripheral Neuropathy ◽  
Nerve Conduction ◽  
Diabetic Peripheral Neuropathy ◽  
Clinical Symptoms ◽  
Multicenter Trial ◽  
Routine Care ◽  
Open Label ◽  
Diabetes Type Ii ◽  
Parallel Group

Abstract Background Acupuncture is used to treat patients with diabetic peripheral neuropathy; however, the evidence is unclear. We present the design and methodology of the ACUDPN (ACUpuncture in Diabetic Peripheral Neuropathy) trial, which investigates the effectiveness of acupuncture for the treatment of diabetic peripheral neuropathy (DPN) symptoms. The aim of this study is to investigate whether acupuncture is effective for the treatment of DPN symptoms. Methods This study is a two-armed, randomized, controlled, parallel group, open-label, confirmatory, multicenter trial (8-week intervention period plus 16 weeks of follow-up). Physicians in outpatient units in Germany who specialize in acupuncture treatment will treat 110 diabetes type II patients with clinical symptoms of peripheral neuropathy in the feet and legs with signs of neuropathy according to nerve conduction testing. The patients will be randomized in a 1:1 ratio to one of the following two groups: (a) semi-standardized acupuncture plus routine care or (b) routine care alone. Acupuncture will consist of 12 treatments per patient over 8 weeks. The primary outcome will be the overall DPN-related complaints in the extremities after 8 weeks as measured by the Visual Analog Scale (VAS). Further outcome measures will include DPN-related pain, the Neuropathic Pain Symptom Inventory (NPSI), Diabetic Peripheral Neuropathic Pain Impact (DPNPI) scores, and nerve conduction parameters of the sural nerve at weeks 8, 16, and 24. Discussion The results of this trial will be available in 2021 and will help clarify whether acupuncture can be considered effective for the treatment of DPN with regard to the subdimensions of the neuropathic clinical picture. Trial registration ClinicalTrials.gov NCT03755960. Registered on 11 August 2018.

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.

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 Iatrogenic Creutzfeldt-Jakob disease following human growth hormone therapy: case report

2002 ◽  
Vol 60 (2B) ◽  
pp. 458-461 ◽  
Author(s):  
Luís Otávio Sales Ferreira Caboclo ◽  
Nancy Huang ◽  
Guilherme Alves Lepski ◽  
José Antônio Livramento ◽  
Carlos Alberto Buchpiguel ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Case Report ◽  
Signal Intensity ◽  
Clinical Symptoms ◽  
Growth Hormone Therapy ◽  
Human Growth ◽  
Autopsy Material ◽  
Pituitary Glands ◽  
Jakob Disease ◽  
Mri Sequences

We report the case of a 41-year-old man with iatrogenic Creutzfeldt-Jakob disease (CJD) acquired after the use of growth hormone (GH) obtained from a number of pituitary glands sourced from autopsy material. The incubation period of the disease (from the midpoint of treatment to the onset of clinical symptoms) was rather long (28 years). Besides the remarkable cerebellar and mental signs, the patient exhibited sleep disturbance (excessive somnolence) from the onset of the symptoms, with striking alteration of the sleep architecture documented by polysomnography. 14-3-3 protein was detected in the CSF, and MRI revealed increased signal intensity bilaterally in the striatum, being most evident in diffusion-weighted (DW-MRI) sequences. This is the second case of iatrogenic CJD associated with the use of GH reported in Brazil.

Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease

The Lancet ◽  
1998 ◽  
Vol 352 (9129) ◽  
pp. 703-704 ◽  
Author(s):  
David A Hilton ◽  
Edward Fathers ◽  
Philip Edwards ◽  
James W Ironside ◽  
John Zajicek
Keyword(s):  
Clinical Onset ◽  
Jakob Disease

Wide phenotypic spectrum of axonal peripheral neuropathy patients with KIF5A mutations

2017 ◽  
Vol 381 ◽  
pp. 380
Author(s):  
D.E. Nam ◽  
J.H. Woo ◽  
M.J. Kim ◽  
S.Y. Shin ◽  
B.O. Choi ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Phenotypic Spectrum

scholarly journals Low diagnostic yield of sural nerve biopsy in patients with peripheral neuropathy and primary amyloidosis

1993 ◽  
Vol 120 (1) ◽  
pp. 60-63 ◽  
Author(s):  
Zachary Simmons ◽  
Mila Blaivas ◽  
Arnold J. Aguilera ◽  
Eva L. Feldman ◽  
Mark B. Bromberg ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Sural Nerve ◽  
Diagnostic Yield ◽  
Nerve Biopsy ◽  
Sural Nerve Biopsy ◽  
Primary Amyloidosis

scholarly journals Factors determining the potential for onward transmission of variant Creutzfeldt–Jakob disease via surgical instruments

2006 ◽  
Vol 3 (11) ◽  
pp. 757-766 ◽  
Author(s):  
Tini Garske ◽  
Hester J.T Ward ◽  
Paul Clarke ◽  
Robert G Will ◽  
Azra C Ghani
Keyword(s):  
Surgical Procedures ◽  
Clinical Symptoms ◽  
Surgical Instruments ◽  
Model Parameters ◽  
Mathematical Framework ◽  
Unknown Parameters ◽  
Medium Risk ◽  
Jakob Disease ◽  
The Uk ◽  
Single Instrument

While the number of variant Creutzfeldt–Jakob disease (vCJD) cases continues to decline, concern has been raised that transmission could occur directly from one person to another through routes including the transfer of blood and shared use of surgical instruments. Here we firstly present data on the surgical procedures undertaken on vCJD patients prior to onset of clinical symptoms, which supports the hypothesis that cases via this route are possible. We then apply a mathematical framework to assess the potential for self-sustaining epidemics via surgical procedures. Data from hospital episode statistics on the rates of high- and medium-risk procedures in the UK were used to estimate model parameters, and sensitivity to other unknown parameters about surgically transmitted vCJD was assessed. Our results demonstrate that a key uncertainty determining the scale of an epidemic and whether it is self-sustaining is the number of times a single instrument is re-used, alongside the infectivity of contaminated instruments and the effectiveness of cleaning. A survey into the frequency of re-use of surgical instruments would help reduce these uncertainties.

scholarly journals Distinctive properties of plaque-type dura mater graft-associated Creutzfeldt–Jakob disease in cell-protein misfolding cyclic amplification

2016 ◽  
Vol 96 (5) ◽  
pp. 581-587 ◽  
Author(s):  
Atsuko Takeuchi ◽  
Atsushi Kobayashi ◽  
Piero Parchi ◽  
Masahito Yamada ◽  
Masanori Morita ◽  
...  
Keyword(s):  
Dura Mater ◽  
Protein Misfolding ◽  
Cell Protein ◽  
Protein Misfolding Cyclic Amplification ◽  
Plaque Type ◽  
Jakob Disease ◽  
Dura Mater Graft

scholarly journals Acupuncture in Diabetic Peripheral Neuropathy - Protocol for the Randomized, Multicenter ACUDPN Trial.

2021 ◽  
Author(s):  
Joanna Dietzel ◽  
Sebastian Hörder ◽  
Isabel Habermann ◽  
Stephanie Roll ◽  
Miriam Ortiz ◽  
...  
Keyword(s):  
Neuropathic Pain ◽  
Peripheral Neuropathy ◽  
Nerve Conduction ◽  
Diabetic Peripheral Neuropathy ◽  
Clinical Symptoms ◽  
Multicenter Trial ◽  
Routine Care ◽  
Open Label ◽  
Diabetes Type Ii ◽  
Parallel Group

Abstract Background: Acupuncture is used to treat patients with diabetic peripheral neuropathy; however, the evidence is unclear. We present the design and methodology of the ACUDPN (ACUpuncture in Diabetic Peripheral Neuropathy) trial, which investigates the effectiveness of acupuncture for the treatment of diabetic peripheral neuropathy (DPN) symptoms. The aim of this study is to investigate whether acupuncture is effective for the treatment of DPN symptoms.Methods: This study is a two-armed, randomized, controlled, parallel group, open-label, confirmatory, multicenter trial (8-week intervention period plus 16 weeks of follow-up). Physicians in outpatient units in Germany who specialize in acupuncture treatment will treat 110 diabetes type II patients with clinical symptoms of peripheral neuropathy in the feet and legs with signs of neuropathy according to nerve conduction testing. The patients will be randomized in a 1:1 ratio to one of the following two groups: (a) semi-standardized acupuncture plus routine care or (b) routine care alone. Acupuncture will consist of 12 treatments per patient over 8 weeks. The primary outcome will be the overall DPN-related complaints in the extremities after 8 weeks as measured by the Visual Analog Scale (VAS). Further outcome measures will include DPN-related pain, the Neuropathic Pain Symptom Inventory (NPSI), Diabetic Peripheral Neuropathic Pain Impact (DPNPI) scores and nerve conduction parameters of the sural nerve at weeks 8, 16 and 24.Discussion: The results of this trial will be available in 2021 and will help clarify whether acupuncture can be considered effective for the treatment of DPN with regard to the subdimensions of the neuropathic clinical picture.Trial registration: ClinicalTrials.gov: NCT03755960. Registered on November 19, 2018, https://clinicaltrials.gov/ct2/show/study/NCT03755960.

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