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Neurology(R) Neuroimmunology &... Jul 2020To determine serologic characteristics, frequency, phenotype, paraneoplastic associations, and electrodiagnostic and histopathologic features accompanying contactin-1...
OBJECTIVE
To determine serologic characteristics, frequency, phenotype, paraneoplastic associations, and electrodiagnostic and histopathologic features accompanying contactin-1 autoimmunity.
METHODS
Archived sera known to produce synaptic tissue-based immunofluorescence patterns were reevaluated, and contactin-1 specificity was confirmed by recombinant protein assays. Screening of 233 chronic/relapsing demyelinating neuropathies for additional cases was performed.
RESULTS
We identified 10 contactin-1 IgG seropositive cases. Frequency of contactin-1 immunoglobulin (Ig) G among tested Mayo Clinic chronic/relapsing demyelinating neuropathies was 2%. Sensory predominant presentations (n = 9, 90%), neuropathic pain (n = 6, 60%), and subacute progression (n = 5, 50%) were commonly encountered among contactin-1 neuropathies. Two patients had chronic immune sensory polyradiculopathy-like phenotype at presentation. Electrodiagnostic studies were consistent with demyelination (slowed conduction velocities and/or prolonged distal latencies) without conduction block. Markedly elevated CSF protein (median 222 mg/dL, range 69-960 mg/dL), thickening/gadolinium enhancement of nerve roots (4/5), and subperineural edema on nerve biopsy (4/4) were other characteristic features. Three cases were diagnosed with paraneoplastic demyelinating neuropathies (thymoma, n = 1; breast cancer, n = 1; plasmacytoma, n = 1). Four of the 9 patients treated with IV immunoglobulin demonstrated initial clinical improvement, but the favorable response was sustained in only 1 case (median follow-up, 60 months). Sustained clinical stabilization or improvement was observed among 3 of the 6 cases in whom second-line therapies (rituximab, cyclophosphamide, and azathioprine) were used.
CONCLUSION
Contactin-1 IgG has a distinct sensory predominant presentation commonly associated with neuropathic pain, with demyelinating changes on electrophysiologic studies. A paraneoplastic cause should be considered. Testing of contactin-1 IgG among cases with similar presentations may guide immunotherapy selection, especially second-line immunotherapy consideration.
Topics: Adult; Aged; Aged, 80 and over; Autoantibodies; Autoimmune Diseases of the Nervous System; Contactin 1; Electrodiagnosis; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Young Adult
PubMed: 32461352
DOI: 10.1212/NXI.0000000000000771 -
Ochsner Journal 2020Meningitis caused by is associated with devastating clinical outcomes. A considerable number of patients will develop long-term neurologic complications. Hearing loss,...
Meningitis caused by is associated with devastating clinical outcomes. A considerable number of patients will develop long-term neurologic complications. Hearing loss, diffuse brain edema, and hydrocephalus are frequently encountered. Acute spinal cord dysfunction and polyradiculopathy can develop in some patients. A 63-year-old female was admitted to our hospital with sudden-onset bilateral lower extremity weakness. On admission, the patient had evidence of spinal cord dysfunction given the abnormal motor and sensory physical examination findings and the absent sensation with a sensory level at dermatome T4 on neurologic examination. Computed tomography myelography did not show evidence of spinal cord compression or transverse myelitis. Cerebrospinal fluid examination was positive for pneumococcal meningitis. The patient was treated with antibiotics and steroids. Nerve conduction studies demonstrated the absence of response, suggesting damage to the peripheral nerves and polyradiculopathy. The patient was treated with plasmapheresis for possible Guillain-Barré syndrome; however, she did not improve despite appropriate antibiotics, steroids, and plasmapheresis. She developed persistent quadriparesis, sensory impairments in upper and lower extremities, and bowel and bladder sphincter dysfunction. Our case demonstrates the development of spinal cord dysfunction (supported by the sudden onset of paraplegia and the presence of a sensory level) and polyradiculopathy (flaccid paralysis, ascending weakness, and absence of response in neurophysiologic studies suggesting severe damage to the peripheral nerves). The appearance of either complication is unusual, and the simultaneous occurrence of both complications is even more uncommon.
PubMed: 32612480
DOI: 10.31486/toj.18.0153 -
International Journal of Molecular... Nov 2022The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The... (Review)
Review
The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The different entities of the disease include chronic immune sensory polyradiculopathy (CISP) and autoimmune nodopathies. It is debatable whether CIDP occurring in the course of other conditions, i.e., monoclonal IgG or IgA gammopathy, should be treated as a separate disease entity from idiopathic CIDP. This study aims to evaluate the molecular differences of the nodes of Ranvier and the initial axon segment (AIS) and juxtaparanode region (JXP) as the potential cause of phenotypic variation of CIDP while also seeking new pathomechanisms since JXP is sequestered behind the paranode and autoantibodies may not access the site easily. The authors initially present the structure of the different parts of the neuron and its functional significance, then discuss the problem of whether damage to the juxtaparanodal region, Schwann cells and axons could cause CIDP or if these damages should be separated as separate disease entities. In particular, AIS's importance for modulating neural excitability and carrying out transport along the axon is highlighted. The disclosure of specific pathomechanisms, including novel target antigens, in the heterogeneous CIDP syndrome is important for diagnosing and treating these patients.
Topics: Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Axons; Autoantibodies; Syndrome
PubMed: 36362407
DOI: 10.3390/ijms232113621 -
Scientific Reports Nov 2023The clinical course of Lyme neuroborreliosis (LNB) is highly variable. Delayed diagnosis and treatment still remain actual challenges. Moreover, there is a lack of...
The clinical course of Lyme neuroborreliosis (LNB) is highly variable. Delayed diagnosis and treatment still remain actual challenges. Moreover, there is a lack of studies analyzing the factors associated with different LNB syndromes. We aimed to analyze clinical and epidemiological features of LNB in hospitalized adults in eastern Lithuania. A retrospective study was performed for patients presenting in the years 2010-2021. A total of 103 patients were included in the study, 100 with early, and three with late LNB. Patients with early LNB most often presented polyradiculitis [75/100, (75%)], which was also the most common initial neurological syndrome. Peripheral facial palsy was diagnosed in 53/100 (53%) patients, in 16/53 (30.2%) cases both facial nerves were affected. Encephalitis or myelitis was diagnosed in 14% of patients with LNB. A total of 76/103 (73.8%) patients were discharged with residual symptoms or signs. One patient presenting encephalomyelitis died because of bacterial complications. The absence of observed erythema migrans (EM) was the predictor of peripheral facial palsy, while female sex and EM untreated with antibiotics were predictors of isolated polyradiculitis. A fever of ≥ 38 ° °C and pleocytosis of ≥ 300 × 10/l were associated with the development of encephalitis or myelitis in patients with early LNB.
Topics: Humans; Adult; Female; Facial Paralysis; Lyme Neuroborreliosis; Retrospective Studies; Polyradiculopathy; Bell Palsy; Encephalitis; Myelitis; Erythema Chronicum Migrans
PubMed: 37964035
DOI: 10.1038/s41598-023-47312-4 -
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 -
Brain Communications 2020Our study aims to quantitate neuromuscular morbidity from radiotherapy in Hodgkin lymphoma including: (i) frequency and (ii) time of onsets for neurological...
Our study aims to quantitate neuromuscular morbidity from radiotherapy in Hodgkin lymphoma including: (i) frequency and (ii) time of onsets for neurological localizations; (iii) degree of disabilities and (iv) number of clinical visits compared to cardiopulmonary Hodgkin lymphoma-radiation complications. Medical records from Mayo Health systems were retrieved; identifying neuromuscular radiation treated Hodgkin lymphoma-complications from 1 January 1994 to 31 December 2016. Of an estimated 4100 post-radiotherapy Hodgkin lymphoma patients, 4.6% (189) were identified with complications. Mean latency to physician visit for symptoms was 23.7 years (range: 1-50). Most commonly identified complications included: head drop 10% (19) with or without myopathy, myopathy 39% (73), plexopathy 29% (54), myelopathy 27% (51) and polyradiculopathy 13% (24). Other findings included benign and malignant nerve sheath tumours 5% (9), phrenic and long thoracic mononeuropathies 7% (14) and compressive spinal meningioma 2% (4). Patients frequently had multiple coexisting complications (single = 76% [144], double = 17% [33], triple = 4% [8], quadruple = 2% [4]). Cardiac 28% (53) and pulmonary 15% (29) complications were also seen in these patients. History of Hodgkin lymphoma was initially overlooked by neurologists (14.3%, 48/336 clinical notes). Hospital and outpatient visits for complications were frequent: neuromuscular 19% (77/411) versus cardiopulmonary 30% (125/411). Testing was largely exclusionary, except when imaging identified secondary malignancy. Modified Rankin score at diagnosis varied: 0-1 (55.8%), 2-3 (5.8%) and 4-5 (38.3%). Neuromuscular complications among post-radiation Hodgkin lymphoma are diverse, occurring in ∼1 of 20 having markedly delayed onsets often eluding diagnosis. Frequent care visits and major morbidity are common. Survivorship recommendations should recognize the diverse neurological complications.
PubMed: 32954302
DOI: 10.1093/braincomms/fcaa050 -
Translational Neuroscience 2020Guillain-Barré syndrome (GBS) is a worldwide demyelinating polyradiculopathy and polyneuropathy. Currently, there is no specific drug for GBS, and established treatment...
INTRODUCTION
Guillain-Barré syndrome (GBS) is a worldwide demyelinating polyradiculopathy and polyneuropathy. Currently, there is no specific drug for GBS, and established treatment is generally based on immune-modulating treatment with plasma exchange or intravenous immunoglobulin in combination with supportive care. This study aimed to investigate the efficiency of integrated Chinese and Western medicine for acute GBS treatment.
METHODS
We enrolled 73 subjects, and randomly divided them into two groups: 35 cases in the traditional Chinese medicine (TCM) group, and 28 in the Control group. The Control group was treated with the common Western medicine for one month; and the TCM group was administrated with one month of common treatment combined with TCM medication.
RESULTS
Compared to the controls, TCM significantly enhanced the treatment efficiency in symptom expression, including the TCM syndrome score, the activity of daily living score, Hughes functional score and sensory dysfunction assessment. The total effective rate of the TCM group was 94.29%, significantly better than controls (78.59%). Moreover, TCM provide better improvement in motor nerve conduction functions (distal motor latency and motor conduction velocity) and sensory nerve conduction functions (sensory conduction velocity and sensory nerve action potential) in median nerve, ulnar nerve, and common fibular nerve.
CONCLUSION
When combined with TCM administration, the GBS treatment could acquire better outcomes.
PubMed: 32161685
DOI: 10.1515/tnsci-2020-0007 -
BMC Neurology Nov 2023Syphilis is associated with a wide variety of systemic presentations, earning it the moniker "The great mimicker". Neurosyphilis is classically associated with...
BACKGROUND
Syphilis is associated with a wide variety of systemic presentations, earning it the moniker "The great mimicker". Neurosyphilis is classically associated with meningovasculitis in the acute-subacute stage and tabes dorsalis and dementia paralytica in later stages. However, one of the less well described presentations include Guillain-Barre Syndrome. This case presents a patient with an ascending polyneuropathy suspicious for Guillain-Barre Syndrome who also had other atypical findings including a truncal sensory loss, optic disc swelling, and rash ultimately found to have neurosyphilis. Electrodiagnostic testing was consistent with demyelination, supporting a diagnosis of neurosyphilis associated Guillain-Barre Syndrome.
CASE PRESENTATION
A 37-year-old female presented to the emergency department with a weakness and difficulty swallowing. She described a three-month history of symptoms, initially starting with a persistent headache followed by one month of a pruritic rash on her chest, palms, and soles. Two weeks prior to presentation, she developed progressive weakness in her arms, numbness in her arms and chest, and difficulty swallowing. Neurological exam was notable for multiple cranial neuropathies, distal predominant weakness in all extremities, length-dependent sensory loss, and hyporeflexia. Investigation revealed a positive Venereal Disease Research Laboratory in her cerebrospinal fluid without significant pleocytosis, contrast enhancement in cranial nerves V, VII, and VIII on MRI, and a demyelinating polyneuropathy on electrodiagnostic testing. She was diagnosed with Guillain-Barre syndrome, secondary to neurosyphilis. The patient acutely declined and required intubation, and ultimately made a full recovery after treatment with plasmapheresis and penicillin.
CONCLUSIONS
This case describes a clinical entity of syphilitic Guillain-Barre Syndrome and highlights the importance of including syphilis in the differential of any patient presenting with ascending polyradiculopathy, especially given the resurgence of syphilis.
Topics: Humans; Female; Adult; Guillain-Barre Syndrome; Syphilis; Neurosyphilis; Exanthema
PubMed: 38001427
DOI: 10.1186/s12883-023-03471-5 -
Neuropediatrics Feb 2021Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and disabling immunomediated radiculoneuropathy. Its worldwide epidemiology is heterogeneous...
BACKGROUND
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and disabling immunomediated radiculoneuropathy. Its worldwide epidemiology is heterogeneous and, in adults, CIDP prevalence varies from 0.6 to 9 cases per 100,000 population. Juvenile CIDP (jCIDP) is even rarer, with age-specific prevalence rates varying from 0.23 to 1.26 owing to different diagnostic criteria (American Academy of Neurology [AAN] and European Federation of Neurological Societies/Peripheral Nerve Society [EFNS/PNS]), different age grouping or, genuine differences.
OBJECTIVES
We assessed jCIDP incidence and prevalence in Sardinia, an area at very-high risk for autoimmune diseases, using comparable methods.
DESIGN
The study area was the northern Sardinia, insular Italy, with 491,571 inhabitants and a pediatric population (0-18 years) of 79,086 individuals.
RESULTS
On prevalence day (December 31, 2019) the total crude, age-specific prevalence rate were 6.32 per 100,000 according with AAN criteria, 7.58 per 100,000 population with European Neuromuscular Center (ENMC) criteria, and 8.85 per 100,000 population with both 2006 and 2010 EFNS/PNS criteria. Crude mean incidence rate were 0.42 per 100,000 per year with AAN criteria, 0.50 per 100,000 per year with ENMC criteria, and 0.59 per 100,000 per year using 2006 and 2010 EFNS/PNS criteria. Of the eight patients, six had typical CIDP, one had multifocal-acquired demyelinating sensory and motor neuropathy (MADSAM), and one chronic immune sensory polyradiculopathy (CISP). Patient's disability was generally mild. Clinical course was progressive, monophasic, or relapsing.
CONCLUSION
jCIDP prevalence and incidence rates in Sardinia were criteria-dependent, the lowest obtained when using AAN criteria, the highest using the EFNS/PNS. Nonetheless, even with the exclusion of the "possible" category, by using comparable methodology, prevalence rates in Sardinia are considerably higher than the range reported in all previous jCIDP studies.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Incidence; Infant; Italy; Mediterranean Islands; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Practice Guidelines as Topic; Prevalence
PubMed: 33111308
DOI: 10.1055/s-0040-1715626 -
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