Comparison of clinical characteristics between neuromyelitis optica spectrum disorders with and without spinal cord atrophy

Background

Spinal cord lesions is one of the predominant characteristics in patients with neuromyelitis optica spectrum disorders (NMOSD). Interestingly, mounting evidence indicates that spinal cord atrophy (SCA) is one of common clinical features in multiple sclerosis (MS) patients, and correlates closely with the neurological disability. However, Clinical studies related to the SCA aspects of NMOSD are still scarce.

Methods

We retrospectively analyzed 185 patients with NMOSD, including 23 patients with SCA and 162 patients without SCA. Data were collected regarding clinical characteristics, laboratory tests, and magnetic resonance imaging findings.

Results

12.4% of patients had SCA in NMOSD. Patients with SCA had a longer disease duration and higher EDSS at clinical onset and last visit. More importantly, SCA patients were more prone to reach disability milestones (EDSS?≥?6.0). Bowel or bladder dysfunction, movement disorders, and sensory disturbances symptoms were more common in patients with SCA. ESR and CRP were significantly higher in patients with SCA than those without SCA. Patients with SCA were more frequently complicated with cervical cord lesions. However, the ARR, progression index, seropositive rate of NMO-IgG and OCB were similar in the two groups. Futhermore, LETM did not differ significantly between patients with SCA and without SCA in NMOSD patients.

Conclusions

Patients with SCA might have longer disease duration, more severe clinical disability, and more frequently complicated with cervical spinal cord lesions. SCA might be predictive of the more severe neurologic dysfunction and worse prognosis in NMOSD. Inflammation contributes to the development of SCA in NMOSD.

     

Human immunodeficiency virus seroconversion presenting with acute inflammatory demyelinating polyneuropathy: a case report

Introduction

Electrocardiogram (ECG) abnormalities in patients with blunt chest trauma are diverse and non-specific, but may be indicative of potentially life-threatening conditions.

Case presentation

We report a rare case of pneumopericardium with extreme ECG abnormalities after blunt chest trauma in a 22-year-old male. The diagnosis was confirmed using computed tomography (CT) scanning. The case is discussed, together with its differential diagnosis and the aetiology of pneumopericardium and tension pneumopericardium.

Conclusion

Pneumopericardium should be distinguished from other pathologies such as myocardial contusion and myocardial infarction because of the possible development of tension pneumopericardium. Early CT scanning is important in the evaluation of blunt chest trauma.     

Acute inflammatory demyelinating polyneuropathy after treatment with pegylated interferon alfa-2a in a patient with chronic hepatitis C virus infection: a case report

ABSTRACT: INTRODUCTION: The combination of polyethylene glycol (PEG)ylated interferon (pegylated interferon) and ribavirin has been shown to be an effective treatment for chronic hepatitis C virus. In general, common side effects related to this combination therapy are mild and are well tolerated. However, peripheral neuropathy including demyelinating polyneuropathy related to PEG-interferon alpha2a (pegylated interferon alfa-2a) is extremely rare. In the literature, only one case of acute inflammatory demyelinating polyneuropathy related to PEG-interferon alpha2a has been published previously. CASE PRESENTATION: To the best of our knowledge we present only the second case of acute inflammatory demyelinating polyneuropathy related to PEG-interferon alpha2a, occurring in a 63-year-old Caucasian man. He developed tingling, numbness, and weakness of his upper and lower extremities with acute neurological deficits after five weeks of a combination therapy with PEG-interferon alpha2a and ribavirin for chronic hepatitis C virus infection. His clinical course, neurological findings, and his electromyogram results were all consistent with acute inflammatory demyelinating polyneuropathy. Our patient recovered completely after interferon was stopped and symptomatic treatment and a further electromyogram showed a disappearance of neuropathy. Four weeks later, PEG-interferon alpha2a was reintroduced with a gradually increasing dose without any reappearance of neurological symptoms allowing hepatitis C seroconversion. CONCLUSIONS: Recognition of this rare yet possible presentation is important for early and accurate diagnosis and treatment. This case report also suggests that the reintroduction of PEGylated interferon in patients who had presented with acute inflammatory demyelinating polyneuropathy related to interferon alpha may be safe, but this must be confirmed by further studies.     

C2 spinal cord stimulation induces dynorphin release from rat T4 spinal cord: potential modulation of myocardial ischemia-sensitive neurons

During myocardial ischemia, the cranial cervical spinal cord (C1-C2) modulates the central processing of the cardiac nociceptive signal. This study was done to determine 1) whether C2 SCS-induced release of an analgesic neuropeptide in the dorsal horn of the thoracic (T4) spinal cord; 2) if one of the sources of this analgesic peptide was cervical propriospinal neurons, and 3) if chemical inactivation of C2 neurons altered local T4 substance P (SP) release during concurrent C2 SCS and cardiac ischemia. Ischemia was induced by intermittent occlusion of the left anterior descending coronary artery (CoAO) in urethane-anesthetized Sprague-Dawley rats. Release of dynorphin A (1-13), (DYN) and SP was determined using antibody-coated microprobes inserted into T4. SCS alone induced DYN release from laminae I-V in T4, and this release was maintained during CoAO. C2 injection of the excitotoxin, ibotenic acid, prior to SCS, inhibited T4 DYN release during SCS and ischemia; it also reversed the inhibition of SP release from T4 dorsal laminae during C2 SCS and CoAO. Injection of the kappa-opioid antagonist, nor-binaltorphimine, into T4 also allowed an increased SP release during SCS and CoAO. CoAO increased the number of Fos-positive neurons in T4 dorsal horns but not in the intermediolateral columns (IML), while SCS (either alone or during CoAO) minimized this dorsal horn response to CoAO alone, while inducing T4 IML neuronal recruitment. These results suggest that activation of cervical propriospinal pathways induces DYN release in the thoracic spinal cord, thereby modulating nociceptive signals from the ischemic heart.     

Somatosensory cerebral potentials evoke by stimulation of the lumbo-sacral spinal cord in normal subjects and in patients with conus medullaris and cauda equina lesions

Somatosensory cerebral evoked potentials were recorded by intrathecal stimulation of the lumbo-sacral cord and roots in 16 normal subjects and patients having cauda/conus injury (group A, 15 cases), compressive lesions of cauda equina (group B, 13 cases) and lesions of both types covering the lumbar cord (group C, 24 cases). The shape of the intrathecally evoked cerebral potential (IECP) was basically the same as that obtained by posterior tibial nerve stimulation from 12 normal subjects except that the early components were 10–15 msec shorter in latency in the former potential, with an average of 12 msec.IECPs were easily recorded in groups A and B, but a significant delay was found in both groups, especially group A. It was difficult to obtain the IECP in group C, When it could be recorded the latency increase was apparent. These findings were explained on the basis of degeneration of the ascending spinal nerve fibers proximal to the lesion site.     

Cervicothoracic spinal cord and pontomedullary injury secondary to high-voltage electrocution: a case report

ABSTRACT: INTRODUCTION: High-voltage electrical injuries are uncommonly reported and may predispose to both immediate and delayed neurologic complications. CASE PRESENTATION: We report the case of a 43-year-old Caucasian man who experienced a high-voltage electrocution injury resulting in ischemic myelopathy and secondary paraparesis. CONCLUSION: High-voltage electrocution injuries are a serious problem with potential for both immediate and delayed neurologic sequelae. The existing literature regarding effective treatment of neurologic complications is limited. Long-term follow-up and multidisciplinary management of these patients is required.     

Modulation of cardiac ischemia-sensitive afferent neuron signaling by preemptive C2 spinal cord stimulation: effect on substance P release from rat spinal cord

The upper cervical spinal region functions as an intraspinal controller of thoracic spinal reflexes and contributes to neuronal regulation of the ischemic myocardium. Our objective was to determine whether stimulation of the C2 cervical spinal cord (SCS) of rats modified the input signal at the thoracic spinal cord when cardiac ischemia-sensitive (sympathetic) afferents were activated by transient occlusion of the left anterior descending coronary artery (CoAO). Changes in c-Fos expression were used as an index of neuronal activation within the spinal cord and brain stem. The pattern of substance P (SP) release, a putative nociceptive transmitter, was measured using antibody-coated microprobes. Two SCS protocols were used: reactive SCS, applied concurrently with intermittent CoAO and preemptive, sustained SCS starting 15 min before and continuing during the repeated intermittent CoAO. CoAO increased SP release from laminae I and II in the T4 spinal cord above resting levels. Intermittent SCS with CoAO resulted in greater levels of SP release from deeper laminae IV-VII in T4 than CoAO alone. In contrast, SP release from laminae I and II was inhibited when CoAO was applied during preemptive, sustained SCS. Preemptive SCS likewise reduced c-Fos expression in the T4 spinal cord (laminae I-V) and nucleus tractus solitarius but increased expression in the intermediolateral cell column of T4 compared with CoAO alone. These results suggest that preemptive SCS from the high cervical region modulates sensory afferent signaling from the ischemic myocardium.     

Prostaglandin D2 in rat brain,spinal cord and pituitary: Basal level and regional distribution

A highly sensitive and specific radioimmunoassay for prostaglandin D₂ has been developed and used to determine the basal level and regional distribution of this prostaglandin in rat brain, spinal cord and pituitary. The assay can detect as little as 20 pg of prostaglandin D₂, and the antiserum used shows 20% cross-reactivity to prostaglandin D₁, 0.1% cross-reactivity to 13,14-dihydro-15-ketoprostaglandin D₂ and even lower cross-reactivity to other prostaglandins. Prostaglandin D₂-like immunoreactivity was extracted with ethanol from the rat tissues. The immunoreactivity comigrated with authentic prostaglandin D₂ on silica gel thin layer chromatography, showed the dilution curve parallel to that of the authentic compound, and decreased in amounts by the pretreatment of animals with indomethacin, suggesting that it was prostaglandin D₂ itself. To avoid a postmortem formation of prostaglandins, we sacrificed animals by microwave irradiation at 4.5 kW for 1.2 sec under which conditions both prostaglandin D synthetase and prostaglandin D dehydrogenase were completely inactivated. The amount of prostaglandin D₂ in whole brain measured under these conditions was 3.42±0.59 ng (mean+S.E.M.), and those of prostaglandin E₂ and F_2α measured by the respective radioimmunoassays were 1.32±0.24 and 0.96±0.20 ng, respectively. Prostaglandin D₂ was widely distributed in rat brain, spinal cord and pituitary. The highest concentrations were seen in pineal gland and neurointermediate pituitary followed by anterior pituitary. Lower but significant concentrations were observed in other parts of brain, among which hypothalamus and septum showed the relatively high concentrations.     

Patient with pontine warning syndrome and bilateral posterior internuclear ophthalmoplegia: case report

Background

Capsular warning syndrome was first described in 1993, featured with repetitive episodes of motor and/or sensory dysfunction without cortical signs. Recently, it has been demonstrated that clinically typical capsular warning syndrome can be associated with pontine infarct and the term �pontine warning syndrome� was coined.

Case Presentation

A 54-year-old woman with a history of hypertension was seen with profound left-sided hemiplegia. She had had 3 episodes of left-sided weakness before complete hemiplegia. Her speech was slurred. Left central facial palsy and hemiglossoplegia were presented. Her left plantar response was extensor and bilateral posterior internuclear ophthalmoplegia was seen on neurologic examination. Biochemical tests revealed hyperglycemia and dyslipidemia on the next day. MRI demonstrated an acute right paramedian pontine infarct. The patient was commenced on oral clopidogrel, atorvastatin and acarbose. After 23 days of hospitalization, she was discharged with severe left hemiplegia.

Conclusions

1) Pontine warning syndrome may be underestimated and understudied. 2) Posterior internuclear ophthalmoplegia is a rare clinical sign in cerebrovascular diseases, while it can help to locate a brainstem lesion rather than an internal capsular one. 3) Blood pressure lowing administration may be improper for patients with pontine warning syndrome.

     

Lung mechanics in individuals with spinal cord injury: effects of injury level and posture.

Individuals with spinal cord injury (SCI) exhibit reduced lung volumes and flow rates as a result of respiratory muscle weakness. These features have not, however, been investigated in relation to the combined effects of injury level and posture. Changes in forced vital capacity (FVC), forced expiratory volume in 1 s (FEV(1)), FEV(1)/FVC, forced expiratory flow at 50% vital capacity (FEF(50)), inspiratory capacity (IC), and expiratory reserve volume (ERV) were assessed by injury level in the seated and supine positions in 74 individuals with SCI. The main findings were 1) FVC, FEV(1), and IC increased with descending SCI level down to T(10), below which they tended to level off; 2) supine values of FVC and FEV(1) tended to be larger in the supine compared with the seated posture down to injury level T(1), caudad to which they were less than when seated; 3) IC increased proportionately more down to injury level L(1), below which it declined slightly and plateaued; 4) ERV was measurable even at high cervical injuries, was generally smaller in the supine position, reached peak values in both positions at T(10) injury level, and then rapidly declined at lower levels; 5) when subjects were separated according to current, former, and never smokers, only formerly smoking paraplegic individuals demonstrated spirometric values significantly less than paraplegic individuals who never smoked. Changes in spirometric measurements in SCI are dependent on injury level and posture. These findings support the concept that the increase in vital capacity in supine position is related to the effect of gravity on abdominal contents and increase in IC.     

A cervical ligamentum flavum cyst in an 82-year-old woman presenting with spinal cord compression: a case report and review of the literature

Introduction

Legionnaires' disease is recognized as a multi-systemic illness. Afflicted patients may have pulmonary, renal, gastrointestinal tract and central nervous system complications. However, renal insufficiency is uncommon. The spectrum of renal involvement may range from a mild and transient elevation of serum creatinine levels to anuric renal failure requiring dialysis and may be linked to several causes. In our present case report, we would like to draw attention to the importance of the pathological documentation of acute renal failure by reporting a case of a patient with acute tubulointerstitial nephritis complicating Legionnaires' disease.

Case presentation

A 55-year-old Caucasian man was admitted to our hospital for community-acquired pneumonia complicated by acute renal failure. Legionella pneumophila serogroup type 1 was diagnosed. Although the patient's respiratory illness responded to intravenous erythromycin and ofloxacin therapy, his renal failure worsened, he became anuric, and hemodialysis was started. A renal biopsy was performed, which revealed severe tubulointerstitial nephritis. After initiation of steroid therapy, his renal function improved dramatically.

Conclusions

This case highlights the importance of kidney biopsies in cases where acute renal failure is a complicating factor in Legionnaires' disease. If the presence of acute tubulointerstitial nephritis can be confirmed, it will likely respond favorably to steroidal treatment and thus irreversible renal damage and chronic renal failure will be avoided.     

Lymphocytes from SJL/J mice immunized with spinal cord respond selectively to a peptide of proteolipid protein and transfer relapsing demyelinating experimental autoimmune encephalomyelitis.

Relapsing experimental autoimmune encephalomyelitis (R-EAE) can be induced in SJL/J mice by immunization with spinal cord homogenate and adjuvant. The specific Ag(s) responsible for acute disease and subsequent relapses in this model is unknown. Myelin basic protein (BP), an encephalitogenic peptide of BP (BP 87-99), and proteolipid protein (PLP) can each induce R-EAE in SJL/J mice, and a peptide of PLP (PLP 139-151) has been reported to induce acute EAE. To determine the encephalitogens in cord-immunized mice with R-EAE, the in vitro proliferative responses of lymph node cells (LNC) and central nervous system mononuclear cells to BP, BP peptides, and PLP peptides were examined during acute EAE and during relapses. LNC responded only to PLP peptides 139-151 and 141-151 and did not respond to BP or its peptides during acute or chronic disease. Central nervous system mononuclear cells also preferentially responded to PLP 139-151 and 141-151 during acute and relapsing disease. A PLP 139-151 peptide-specific Th cell line was selected from LNC of cord-immunized donors. Five million peptide-specific line cells transferred severe relapsing demyelinating EAE to naive recipients. We conclude that PLP peptide 139-151 is the major encephalitogen for R-EAE in cord-immunized SJL/J mice. We demonstrate for the first time that Th cells specific for this peptide are sufficient to transfer relapsing demyelinating EAE. The predominance of a PLP immune response rather than a BP response in SJL/J mice suggests that genetic background may determine the predominant myelin Ag response in human demyelinating diseases such as multiple sclerosis.     

Collision tumor with inflammatory breast carcinoma and malignant phyllodes tumor: a case report and literature review

Background

Pelvic reconstruction after hemipelvectomy can greatly improve the weight-bearing stability of the supporting skeleton and improve patients’ quality of life. Although an autograft can be used to reconstruct pelvic defects, the most suitable choice of autograft, i.e., the use of either femur or tibia, has not been determined. We aimed to analyze the mechanical stresses of a pelvic ring reconstructed using femur or tibia after hemipelvectomy using finite element (FE) analysis.

Methods

FE models of normal and reconstructed pelvis were established based on computed tomography images, and the stress distributions were analyzed under physiological loading from 0 to 500 N in both intact and restored pelvic models using femur or tibia.

Results

The vertical displacement of the intact pelvis was less than that of reconstructed pelvis, but there was no significant difference between the two reconstructed models. In FE analysis, the stress distribution of the intact pelvic model was bilaterally symmetric and the maximum stresses were located at the sacroiliac joint, arcuate line, ischiatic ramus, and ischial tuberosity. The maximum stress in each part of the reconstructed pelvis greatly exceeded that of the intact model. The maximum von Mises stress of the femur was 13.9 MPa, and that of the tibia was 6.41 MPa. However, the stress distribution was different in the two types of reconstructed pelvises. The tibial reconstruction model induced concentrated stress on the tibia shaft making it more vulnerable to fracture. The maximum stress on the femur was concentrated on the connections between the femur and the screws.

Conclusions

From a biomechanical point of view, the reconstruction of hemipelvic defects with femur is a better choice.     

Vemurafenib and concomitant stereotactic radiation for the treatment of melanoma with spinal metastases: A case report

A 56-year-old man with BRAFV600E melanoma and spinal metastases treated with vemurafenib and stereotactic radiation showed a partial response without neurological, skin or mucosal toxicity, 8 months after completion of this combination. This case suggests that stereotactic radiation spares normal tissues and might be safer than conventional fractionated radiation with vemurafenib.     

Cerebral toxoplasmosis with multiple hemorrhage lesions in an HIV infected patient: A case report and literature review

A 47-year-old man was admitted to a hospital for disturbance of consciousness. He was diagnosed with multiple hemorrhagic brain abscesses in bilateral hemispheres with human immunodeficiency virus (HIV) infection, and was transferred to our hospital for further examination and treatment. On admission, although he could respond to pain stimuli, he could not talk or communicate. His laboratory data on admission revealed CD4-positive T cell count of 67 cells/μL, and HIV1-RNA viral load of 5.6 × 10⁵ copies/mL. Both the serum IgG Toxoplasma gondii antibody and the cerebrospinal fluid polymerase chain reaction for Toxoplasma gondii DNA were positive. He was diagnosed with cerebral toxoplasmosis and HIV infection. His level of consciousness worsened, and the number of hemorrhagic lesions had increased in both hemispheres and the left thalamus on the computed tomography scan following two weeks of antitoxoplasma therapy. These newly discovered hemorrhagic lesions revealed in the CT had been found as the high intensity signal regions of initial fluid-attenuated inversion recovery magnetic resonance imaging. After five weeks of treatment, the hemorrhagic lesions gradually improved along with the patient's consciousness. Antiretroviral therapy was initiated six weeks following antitoxoplama therapy with reassurance that immune reconstitution inflammatory syndrome did not occur. After approximately four months of antitoxoplasma therapy, the patient was discharged into a group home with residual left hemiparesis on maintenance antitoxoplasma and antiretroviral therapy. Clinicians should recognize the delay of clinical and radiological improvement for hemorrhagic cerebral toxoplasmosis and patiently continue the antitoxoplasma therapy.     

Granulomatous skin lesions complicating Varicella infection in a patient with Rothmund-Thomson syndrome and immune deficiency: case report

Acro-cardio-facial syndrome (ACFS) is a rare genetic disorder characterized by split-hand/split-foot malformation (SHFM), facial anomalies, cleft lip/palate, congenital heart defect (CHD), genital anomalies, and mental retardation. Up to now, 9 patients have been described, and most of the reported cases were not surviving the first days or months of age. The spectrum of defects occurring in ACFS is wide, and both interindividual variability and clinical differences among sibs have been reported. The diagnosis is based on clinical criteria, since the genetic mechanism underlying ACFS is still unknown. The differential diagnosis includes other disorders with ectrodactyly, and clefting conditions associated with genital anomalies and heart defects. An autosomal recessive pattern of inheritance has been suggested, based on parental consanguinity and disease's recurrence in sibs in some families. The more appropriate recurrence risk of transmitting the disease for the parents of an affected child seems to be up to one in four. Management of affected patients includes treatment of cardiac, respiratory, and feeding problems by neonatal pediatricians and other specialists. Prognosis of ACFS is poor.