-
International Journal of Surgery Case... May 2022Peripheral nervous system involvement is very common in Sjogren's syndrome (SS); however, polyradiculopathy has been reported rarely in association with SS, and...
INTRODUCTION AND IMPORTANCE
Peripheral nervous system involvement is very common in Sjogren's syndrome (SS); however, polyradiculopathy has been reported rarely in association with SS, and predominantly chronic forms have been described. Here, we reported a case from our Neurosurgery Department in Intan Medika KIM Hospital, Bangka Island, Pangkalpinang, Indonesia; as Academic Health System of Universitas Padjadjaran.
CASE PRESENTATION
A 32-year-old woman, diagnosed with Sjogren's syndrome that was characterized by anti-nuclear, anti-Ro, anti-La and anti dsDNA-antibodies positives since 3 years ago; consulted to our department for a chronic low back with a radicular pain in both lower limbs from the gluteal area to both feet together with numbness, hyperstesis and allodynia. The pain was evaluated by the visual analogue scale (VAS) score of 8; we then performed cervico-lumbal computed tomography (CT) scan that demonstrated multiple protruded discs of the cervical- and lumbar-spine.
CLINICAL DISCUSSION
Pain was treated with lumbar interlaminar epidural steroid injections as a safe technique that allows relieving patient symptoms; after 10 min, the patient experienced an improvement in her pain with reduced scores to 0-1 in VAS, as well as a significant improvement on her quality of life later on.
CONCLUSION
The use of lumbar interlaminar epidural steroid injections for an alternative therapeutic for neuropathic pain in SS gives a satisfactory result in terms of improvement of pain as well as a significant improvement on patients' quality of life.
PubMed: 35439726
DOI: 10.1016/j.ijscr.2022.107053 -
BMC Neurology Mar 2022The long-term use of an oral corticosteroid suppresses immunity. Here, we describe a case involving a patient with weakness in the bilateral lower extremities due to...
BACKGROUND
The long-term use of an oral corticosteroid suppresses immunity. Here, we describe a case involving a patient with weakness in the bilateral lower extremities due to cytomegalovirus (CMV) lumbosacral polyradiculitis.
CASE PRESENTATION
A 64-year-old man visited a university hospital for symmetric motor weakness in both lower extremities (Medical Research Council grade: 2). Symptoms started 1 month before and gradually aggravated. The patient had been taking oral prednisolone for 10 years in order to control pain in multiple joints due to seronegative rheumatoid arthritis. He also had neuropathic pain on the entire right lower extremity and voiding difficulty. Gadolinium-enhanced magnetic resonance imaging revealed enhancement along the entire lumbosacral nerve roots. In the cerebrospinal fluid analysis (CSF), elevated white blood cell (WBC) count (19 cells/μL) and protein level (142.5 mg/dL) were observed. CMV detection by polymerase chain reaction (PCR) was positive. We diagnosed the patient as having lumbosacral polyradiculitis due to CMV. Ganciclovir (250 mg twice daily) was administered intravenously. Two months after initiating Ganciclovir, in the CSF analysis, CM detection by PCR was negative, and no WBC was found.
CONCLUSION
We reported a patient who had symmetric motor weakness in the bilateral lower extremities induced by CMV lumbosacral polyradiculitis. Its occurrence seems to be related to immunosuppresion due to the long-term use of an oral corticosteroid. When a patient who is taking an oral corticosteroid shows motor weakness in the bilateral lower extremities, CMV lumbosacral polyradiculitis is one of the possible disorders to be differentiated.
Topics: Adrenal Cortex Hormones; Cytomegalovirus; Cytomegalovirus Infections; Ganciclovir; Humans; Male; Middle Aged; Polyradiculopathy
PubMed: 35287603
DOI: 10.1186/s12883-022-02623-3 -
Neurology India 2022Malignant atrophic papulosis (MAP), or systemic Degos disease, is an obliterative vasculopathy of unknown origin, characterized by erythematous papules found on the... (Review)
Review
Malignant atrophic papulosis (MAP), or systemic Degos disease, is an obliterative vasculopathy of unknown origin, characterized by erythematous papules found on the skin, central nervous system (Neuro-MAP) and gastrointestinal tract. Neurological involvement occurs in approximately 20% of systemic cases, is progressive and largely fatal. It can be described in two forms: 1) the parenchymal presenting with meningoencephalitis and meningomyelitis and 2) the neurovascular presenting with large cerebral infarcts, intracranial and subarachnoid hemorrhage, subdural hematoma and venous sinus thrombosis. Predilection to subdural hematoma or hygroma is characteristic for neurological involvement in MAP in comparison to other vasculpathies and vasculitides. Peripheral nervous system manifestations are less common and include polyradiculopathy, neuropathy, and myopathy. CSF analysis usually shows mild to moderate pleocytosis, increased protein content, and normal glucose. Brain MRI may reveal cortical, subcortical and deep white matter ischemic lesions with possible nodular, leptomeningeal, dural, or ependymal enhancement. Spinal cord MRI may reveal patchy lesions from the periphery to the center or cord atrophy in progressive course. Neurological involvement in MAP has a grave prognosis. The interval from onset of papulosis to death averages two years in patients with neurological involvement. There is no confirmed treatment for MAP but there are promising reports with eculizumab and treprostinil.
Topics: Atrophy; Hematoma, Subdural; Humans; Malignant Atrophic Papulosis; Prognosis; Skin
PubMed: 35263846
DOI: 10.4103/0028-3886.338719 -
International Orthopaedics Jun 2022Post-void residual (PVR) scans of less than 200 ml are increasingly being used to rule out the likelihood of cauda equina syndrome (CES) and to delay emergency MRI... (Review)
Review
OBJECTIVE
Post-void residual (PVR) scans of less than 200 ml are increasingly being used to rule out the likelihood of cauda equina syndrome (CES) and to delay emergency MRI scanning in suspected cases. This study was done to review a series of 50 MRI confirmed cases of CES and to test the hypothesis that a PVR of less than 200 ml was unlikely to be present.
METHODS
Fifty consecutive medicolegal cases involving CES were audited. Records were reviewed to see if PVR scans were done. MRI scans were reviewed, clinical and radiological diagnosis reviewed, and treatment recorded.
RESULTS
Out of 50 CES cases, 26 had had PVR scans. In 14/26 (54%) the PVR scan was ≤ 200 ml. In one case, the CES diagnosis was in question leaving 13/26 (50%) cases where there was a clear clinical and MRI diagnosis of CES despite the PVR being ≤ 200 ml. All 13 were classified as incomplete cauda equina syndrome (CESI) and all proceeded to emergency decompression.
CONCLUSIONS
This study is the first in the literature to demonstrate that there is a significant group of CES patients who require emergency decompression but have PVRs ≤ 200 ml. The results demonstrate the existence of a significant group of CESI patients whose bladder function may be deteriorating, but they have not yet reached the point where the PVR is over 200 ml. Given the accepted understanding that CESI is best treated with emergency decompression, such patients are likely to have worse outcomes if MRI scanning and therefore surgery is delayed. We recommend the following: PVR is recommended as an assessment tool in suspected CES. A PVR of ≤ 200 reduces the likelihood of having CES but does not exclude it; clinical suspicion of CES should always lead to an MRI scan. Further investigation of PVR as a prognostic tool is recommended.
Topics: Cauda Equina Syndrome; Disease Progression; Humans; Magnetic Resonance Imaging; Polyradiculopathy; Retrospective Studies; Urinary Bladder
PubMed: 35182176
DOI: 10.1007/s00264-022-05341-0 -
Clinical Case Reports Jan 2022Due to SARS-COV-2 (COVID-19) pandemic and its catastrophic impact on society, the FDA granted emergency use authorization for some vaccines. Possible rare side effects...
Due to SARS-COV-2 (COVID-19) pandemic and its catastrophic impact on society, the FDA granted emergency use authorization for some vaccines. Possible rare side effects could not have been observed in this relatively short period. We are reporting an elderly lady with multiple comorbidities who presented with progressive lower limb weakness that started seven days after receiving the first dose of the COVID-19 vaccine. The electrodiagnostic study showed demyelinating polyneuropathy with secondary axonal degeneration consistent with Guillain-Barré syndrome. We ruled out other possible causes for GBS, suggesting a postvaccine nature for her presentation. The patient received intravenous immunoglobulin (IVIG) for five days and gradually improved, which supports our initial diagnosis.
PubMed: 35079381
DOI: 10.1002/ccr3.5237 -
International Orthopaedics Feb 2022International uniformity of definition and classification are crucial for diagnosis and management of cauda equina syndrome (CES). They are also useful for clinicians... (Review)
Review
PURPOSE
International uniformity of definition and classification are crucial for diagnosis and management of cauda equina syndrome (CES). They are also useful for clinicians when discussing CES with patients and relatives, and for medicolegal purposes.
METHODS
We reviewed published literature using PubMed on definition and classification of cauda equina syndrome since 2000 (21 years). Using the search terms 'cauda equina' and 'definition' or 'classification', we found and reviewed 212 papers.
RESULTS
There were 17 different definitions of CES used in the literature. There were three well-defined methods of classification of CES. The two-stage system of incomplete CES (CESI) versus CES with retention (CESR) is the most commonly used classification, and has prognostic value although the details of this continue to be debated.
CONCLUSION
We used the existing literature to propose a clear definition of CES. We also drew on peer-reviewed published literature that has helped to amplify and expand the CESI/CESR dichotomy, adding categories that are both less severe than CESI, and more severe than CESR, and we propose clear definitions in a table form to assist current and future discussion and management of CES.
Topics: Cauda Equina Syndrome; Humans; Polyradiculopathy; Prognosis
PubMed: 34862914
DOI: 10.1007/s00264-021-05273-1 -
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 -
American Journal of Neurodegenerative... 2021Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic autoimmune demyelinating peripheral neuropathy that leads to symmetrical muscular weakness,...
INTRODUCTION
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic autoimmune demyelinating peripheral neuropathy that leads to symmetrical muscular weakness, sensory deficit, hyporeflexia, chronic fatigue, and impaired quality of life (QoL). The current study aims to investigate the effects of gabapentin versus pregabalin on pain, sleep disturbances, and QoL in CIDP patients.
METHODS
This clinical trial was conducted on 40 patients diagnosed with CIDP randomly allocated to treatment with 100-500 mg gabapentin (n=20) or 50-300 mg pregabalin (n=20) both co-medicated with 37.5 mg venlafaxine. The dose of gabapentin/pregabalin was adjusted based on the patient's tolerability/response to the treatment. Visual analogue scale (VAS), Pittsburg Sleep Quality Questionnaire and Short Form Health Survey (SF-36) were filled at baseline, within three, six, nine and 12 months after the interventions to assess pain severity, sleep quality and QoL, respectively. The Iranian Registry of Clinical Trials (IRCT) code: IRCT20200217046523N16, https://fa.irct.ir/search/result?query=IRCT20200217046523N16.
RESULTS
Gabapentin revealed a dose-dependent efficacy in pain severity (-value =0.004, r=0.287), sleep quality (-value <0.001, r=0.387) and QoL (-value =0.001, r=-0.378), but pregabalin (-value >0.05). Co-medication of gabapentin plus venlafaxine could significantly improve sleep quality (-value =0.009) and QoL (-value =0.004), but pain severity (-value =0.796). Pregabalin plus venlafaxine showed statistically significant improvement in pain (-value =0.046), sleep quality (-value <0.001) and QoL (-value <0.001). The comparison of the two medications revealed the superiority of pregabalin in pain relief (-value >0.001) and QoL (-value =0.03) to pregabalin.
CONCLUSION
Based on this study, the co-medication of pregabalin and venlafaxine led to remarkable superior outcomes compared to venlafaxine plus gabapentin in the management of pain, sleep quality, and QoL due to CIDP.
PubMed: 34712518
DOI: No ID Found -
Cureus Sep 2021Idiopathic nutritional deficiencies are often overlooked in patients with no history of malabsorption. However, it may lead to severe neurologic dysfunction that can...
Idiopathic nutritional deficiencies are often overlooked in patients with no history of malabsorption. However, it may lead to severe neurologic dysfunction that can sometimes be irreversible. We present a case in which early recognition of copper deficiency has led to a better outcome for the patient, who presented with acute myeloneuropathy. A 45-year-old male with no significant history of malnutrition or malabsorption presented with complaints of acute encephalopathy, bilateral wrist drop, bilateral tingling and weakness in his hands as well as urinary incontinence. Workup upon arrival was nonrevealing, the patient was treated initially as presumed AIDP (acute inflammatory demyelinating polyradiculopathy), and he underwent plasmapheresis with no response. Since the patient did not respond to plasmapheresis and he had a significantly low folate levels with initial labs. Further nutritional workup was done, which revealed low copper (levels of 0.45), vitamins A, E, and B1. The patient was also tested for celiac which was negative, underwent upper endoscopy and colonoscopy which were both not significant. Decision was made to treat patient early with IV copper infusion as symptoms were deemed most likely due to copper deficiency. The patient received a total of 4 IV doses, after which the patient had a significant clinical response after infusion therapy and repeat copper levels revealed an increase as well (levels of 0.71). Prior to discharge, the patient had significant improvement in wrist drop as well as symptoms of tingling and numbness. Despite being a trace element, copper deficiency can cause significant neurologic impairment. Furthermore, early recognition has proved to be imperative in neurologic recovery and supplementation has proven to be successful in improving patient's quality of life.
PubMed: 34692339
DOI: 10.7759/cureus.18130 -
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