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California Medicine May 1972An animal model for acute multiple sclerosis (ms) is experimental allergic encephalomyelitis (eae). eae is produced by intradermal injection of a protein component of... (Review)
Review
An animal model for acute multiple sclerosis (ms) is experimental allergic encephalomyelitis (eae). eae is produced by intradermal injection of a protein component of central nervous system (cns) myelin. Ultrastructural studies of eae and of a peripheral nerve analog, experimental allergic neuritis (ean), have revealed an orderly sequence of cellular events leading to the destruction and removal of myelin with sparing of axons (primary demyelination). Acute ms has not been studied electron microscopically, but the ultrastructural similarities between ean and a case of acute Landry-Guillain-Barré syndrome, a primary demyelinating disease of the peripheral nervous system, suggest that a similar sequence of events might be found in acute ms. While the pathological findings support a cellmediated or delayed hypersensitivity response, there is also evidence for the pathogenetic role of circulating antibodies. Among such evidence is included the finding that sera from animals with eae and humans with acute ms rapidly produce a reversible block of complex (polysynaptic) electrical activity when applied to cns tissue cultures, which suggests a possible mechanism for transient symptoms in ms. Epidemiological and other studies link ms with a viral cause, although no direct evidence that ms is caused by a virus exists. Viral and immunological mechanisms are not mutually exclusive in considering pathogenetic possibilities for ms, for it can be postulated that a viral infection of the central nervous system acts as a triggering agent for a series of immune responses, including production of a bioelectric blocking antibody and demyelination mediated by sensitized cells, the combination of which ultimately produces the total clinical picture of ms.
Topics: Animals; Antibodies; Disease Models, Animal; Encephalomyelitis, Autoimmune, Experimental; Humans; Immunity, Cellular; Multiple Sclerosis; Myelin Sheath; Neuritis; Neuromuscular Junction; Polyradiculopathy; Virus Diseases
PubMed: 4567443
DOI: No ID Found -
Case Reports in Neurology 2021Epstein-Barr virus (EBV) has been associated with a plethora of neurological manifestations including polyneuropathy and polyradiculopathy. A 27-year-old man with a...
Epstein-Barr virus (EBV) has been associated with a plethora of neurological manifestations including polyneuropathy and polyradiculopathy. A 27-year-old man with a recent upper respiratory system infection presented with difficulty in walking. His neurological examination revealed reduced muscle strength in both proximal and distal lower limb muscles without sensory and autonomic signs. Needle electromyography showed abnormal spontaneous activity and reduced recruitment of motor units in muscles innervated by multiple lumbo-sacral roots. Cerebrospinal examination showed increased protein levels with normal cell counts. While spinal MRI was normal, whole-body CT and PET examination showed disseminated lymph node enlargement. Anti-EBV viral capsid antigen and anti-nuclear antigen IgG but not IgM was positive, whereas EBV PCR was negative in blood. Analysis of inguinal lymph node biopsy showed reactive lymphoid hyperplasia and EBV DNA. Leucine-rich glioma-inactivated protein 1 (LGI1) antibody was found in serum but not in CSF. All clinical, imaging, and electrophysiological findings improved following steroid and intravenous immunoglobulin treatment. These findings suggested the acute involvement of lumbo-sacral spinal roots and/or motor neurons. Purely motor polyradiculopathy has been reported in both EBV-positive and LGI1 antibody-positive patients, and EBV infection is known to precede different autoimmune manifestations. Whether EBV infection may trigger LGI1 autoimmunity and cause involvement of spinal motor roots and/or motor neurons needs to be further studied.
PubMed: 34720961
DOI: 10.1159/000518196 -
The British Journal of General Practice... Feb 2014
Topics: Early Diagnosis; Humans; Intervertebral Disc Displacement; Jurisprudence; Magnetic Resonance Imaging; Physical Examination; Polyradiculopathy; Postoperative Complications; Practice Guidelines as Topic; Primary Health Care; Radiography; Time Factors
PubMed: 24567588
DOI: 10.3399/bjgp14X676988 -
Journal of the West African College of... 2018Upper lumbar disc prolapse (ULDP) is a rare and a unique clinical entity which has a potentially devastating clinical outcome. It may manifest with low back or anterior...
Upper lumbar disc prolapse (ULDP) is a rare and a unique clinical entity which has a potentially devastating clinical outcome. It may manifest with low back or anterior thigh pain, polyradiculopathies (from spinal cord or cauda equina compression) and/or degenerative kyphoscoliosis. Its diagnosis is often difficult and may be missed because of the lack of specific root signs. Magnetic resonance imaging is the diagnostic modality of choice. Anterior, posterior and endoscopic approaches have been explored in the treatment of this pathology. However, the treatment outcome for ULDP has been found to be poorer compared to that of similar herniations at lower spinal levels. We report this case to draw attention to the need for a high index of suspicion to make the correct diagnosis.
PubMed: 32551321
DOI: No ID Found -
Cureus Sep 2022We present a case of a 42-year-old female living with poorly controlled diabetes who presented with a nine-month evolution of ataxic gait, reduced motor and sensitive...
We present a case of a 42-year-old female living with poorly controlled diabetes who presented with a nine-month evolution of ataxic gait, reduced motor and sensitive function of lower and upper limbs, and postural anesthesia of fingers, feet, and toes. Deep tendon reflexes were abolished in the lower limbs and markedly diminished in the upper limbs. Cerebrospinal fluid (CSF) analysis showed a high protein level, and both imaging and serologic studies were normal. Although she had a previous electrophysiologic study showing distal symmetric polyneuropathy (DSPN) with an axonal lesion, nerve conduction studies were repeated, and a diagnosis of chronic inflammatory demyelinating polyneuroradiculopathy (CIDP) was made. According to the state of the art, intravenous immunoglobulin (IVIg) was started. The patient's Inflammatory Neuropathy Cause and Treatment (INCAT) score and Medical Research Council (MRC) Sum Score both improved after two cycles. Unfortunately, symptoms quickly recurred, and corticosteroids were introduced to try to delay symptom recurrence, although it worsened diabetes control. Later, IVIg was stopped due to nephrotic syndrome, and immunosuppression was initiated. CIDP is a potentially treatable disease, but the diagnosis must be made as soon as possible to start therapy and reduce sequelae. Neuropathy in patients living with diabetes is common, but patients must be monitored closely to enable a correct diagnosis and adequate treatment.
PubMed: 36304380
DOI: 10.7759/cureus.29390 -
British Medical Journal Feb 1970
Review
Topics: Arsenic Poisoning; Deficiency Diseases; Humans; Isoniazid; Lead Poisoning; Metabolic Diseases; Neoplasms; Nitrofurantoin; Peripheral Nervous System Diseases; Polyradiculopathy
PubMed: 4313649
DOI: 10.1136/bmj.1.5692.349 -
British Medical Journal Sep 1967
Topics: Cerebellopontine Angle; Diagnosis, Differential; Electric Stimulation Therapy; Facial Paralysis; Humans; Multiple Sclerosis; Polyradiculopathy; Prognosis
PubMed: 5298536
DOI: 10.1136/bmj.3.5569.815 -
Neurology Dec 2021Multiple studies highlighting the diagnostic utility of neurofascin-155 (NF155)-immunoglobulin G4 (IgG4) in chronic demyelinating inflammatory polyradiculoneuropathy...
BACKGROUND AND OBJECTIVE
Multiple studies highlighting the diagnostic utility of neurofascin-155 (NF155)-immunoglobulin G4 (IgG4) in chronic demyelinating inflammatory polyradiculoneuropathy (CIDP) have been published. However, few studies comprehensively address the long-term outcomes or clinical utility of NF155-immunoglobulin M (IgM) or NF155-immunoglobulin G (IgG) in the absence of NF155-IgG4. We evaluated phenotypic and histopathologic specificity and differences in outcomes between these NF155 antibody isotypes or IgG subclasses. We also compare NF155-IgG4-seropositive cases to other seropositive demyelinating neuropathies.
METHODS
Neuropathy patient sera at Mayo Clinic were tested for NF155-IgG4, NF155-IgG, and NF155-IgM autoantibodies. Demographic and clinical data of all seropositive cases were reviewed.
RESULTS
We identified 32 NF155 cases (25 NF155-IgG-positive [20 NF155-IgG4-positive], 7 NF155-IgM-seropositive). NF155-IgG4-seropositive patients clinically presented with distal more than proximal muscle weakness, positive sensory symptoms (prickling, asymmetric paresthesia, neuropathic pain), and gait ataxia. Cranial nerve involvement (11/20 [55%]) and papilledema (4/12 [33%]) occurred in many. Electrodiagnostic testing (EDX) demonstrated demyelinating polyradiculoneuropathy (19/20 [95%]). Autonomic involvement occurred in 45% (n = 9, median composite autonomic scoring scale score 3.5, range 1-7). Nerve biopsies from the NF155-IgG4 patients (n = 11) demonstrated grouped segmental demyelination (50%), myelin reduplication (45%), and paranodal swellings (50%). Most patients needed second- and third-line immunosuppression but had favorable long-term outcomes (n = 18). Among 14 patients with serial EDX over 2 years, all except one demonstrated improvement after treatment. NF155-IgG-positive, NF155-IgG4-negative (NF155-IgG-positive) and NF155-IgM-positive patients were phenotypically different from NF155-IgG4-seropositive patients. Sensory ataxia, neuropathic pain, cerebellar dysfunction, and root/plexus MRI abnormalities were significantly more common in NF155-IgG4-positive compared to myelin-associated glycoprotein (MAG)-IgM neuropathy. Chronic immune sensory polyradiculopathy (CISP)/CISP-plus phenotype was more common among contactin-1 neuropathies compared to NF155-IgG4-positive cases. NF155-IgG4-positive cases responded favorably to immunotherapy compared to MAG-IgM-seropositive cases with distal acquired demyelinating symmetric neuropathy ( < 0.001) and had better long-term clinical outcomes compared to contactin-1 IgG ( = 0.04).
DISCUSSION
We report long-term follow-up and clinical outcome of NF155-IgG4 cases. NF155-IgG4 but not IgM or IgG cases have unique clinical-electrodiagnostic signature. We demonstrate NF155-IgG4-positive patients, unlike classical CIDP with neuropathic pain and dysautonomia common at presentation. Long-term outcomes were favorable.
CLASSIFICATION OF EVIDENCE
This study provides Class III evidence that NF155-IgG4-seropositive patients, compared to patients with typical CIDP, present with distal more than proximal muscle weakness, positive sensory symptoms, and gait ataxia.
Topics: Autoantibodies; Cell Adhesion Molecules; Contactin 1; Humans; Immunoglobulin M; Nerve Growth Factors; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
PubMed: 34635556
DOI: 10.1212/WNL.0000000000012932 -
Nature Reviews. Urology Feb 2015During the past century, diverse studies have focused on the development of surgical strategies to restore function of a decentralized bladder after spinal cord or... (Review)
Review
During the past century, diverse studies have focused on the development of surgical strategies to restore function of a decentralized bladder after spinal cord or spinal root injury via repair of the original roots or by transferring new axonal sources. The techniques included end-to-end sacral root repairs, transfer of roots from other spinal segments to sacral roots, transfer of intercostal nerves to sacral roots, transfer of various somatic nerves to the pelvic or pudendal nerve, direct reinnervation of the detrusor muscle, or creation of an artificial reflex pathway between the skin and the bladder via the central nervous system. All of these surgical techniques have demonstrated specific strengths and limitations. The findings made to date already indicate appropriate patient populations for each procedure, but a comprehensive assessment of the effectiveness of each technique to restore urinary function after bladder decentralization is required to guide future research and potential clinical application.
Topics: Humans; Neurosurgical Procedures; Peripheral Nerves; Polyradiculopathy; Plastic Surgery Procedures; Spinal Cord Injuries; Spinal Nerve Roots; Urinary Bladder; Urinary Bladder, Neurogenic
PubMed: 25666987
DOI: 10.1038/nrurol.2015.4 -
Emerging Infectious Diseases Feb 2011Information about the spectrum of disease caused by hepatitis E virus (HEV) genotype 3 is emerging. During 2004-2009, at 2 hospitals in the United Kingdom and France,... (Review)
Review
Information about the spectrum of disease caused by hepatitis E virus (HEV) genotype 3 is emerging. During 2004-2009, at 2 hospitals in the United Kingdom and France, among 126 patients with locally acquired acute and chronic HEV genotype 3 infection, neurologic complications developed in 7 (5.5%): inflammatory polyradiculopathy (n = 3), Guillain-Barre syndrome (n = 1), bilateral brachial neuritis (n = 1), encephalitis (n = 1), and ataxia/proximal myopathy (n = 1). Three cases occurred in nonimmunocompromised patients with acute HEV infection, and 4 were in immunocompromised patients with chronic HEV infection. HEV RNA was detected in cerebrospinal fluid of all 4 patients with chronic HEV infection but not in that of 2 patients with acute HEV infection. Neurologic outcomes were complete resolution (n = 3), improvement with residual neurologic deficit (n = 3), and no improvement (n = 1). Neurologic disorders are an emerging extrahepatic manifestation of HEV infection.
Topics: Acute Disease; Adult; Chronic Disease; Female; France; Guillain-Barre Syndrome; Hepatitis E; Hepatitis E virus; Humans; Male; Middle Aged; Nervous System Diseases; United Kingdom
PubMed: 21291585
DOI: 10.3201/eid1702.100856