Primary Epstein-Barr virus infection in immunocompetent patients with acute transverse myelitis and a combination of polyradiculitis and anterior horn syndrome as neurological manifestations.

Neurological manifestations of a primary Epstein-Barr virus (EBV) infection are rare. We describe a case with acute transverse myelitis and another case with a combination of polyradiculitis and anterior horn syndrome as manifestations of a primary EBV infection.The first case is a 50-year-old immunocompetent male diagnosed with acute transverse myelitis, 2 weeks after he was clinically diagnosed with infectious mononucleosis. The second case is an 18-year-old immunocompetent male diagnosed with a combination of polyradiculitis and anterior horn syndrome while he had infectious mononucleosis. The first patient was treated with methylprednisolone. After 1 year, he was able to stop performing clean intermittent self-catheterisation. The second patient completely recovered within 6 weeks without treatment.Primary EBV infection should be considered in immunocompetent patients presenting with acute transverse myelitis and a combination of polyradiculitis and anterior horn syndrome. Antiviral treatment and steroids are controversial, and the prognosis of neurological sequelae is largely unknown.

Neuroprotective Effects of Valproic Acid in a Rat Model of Cauda Equina Injury.

BACKGROUND: Histone deacetylase inhibitors, including valproic acid (VPA), are promising therapeutic interventions in neurological disorders and play an important role in synaptic activity and neuronal function. METHODS: A total of 30 rats were randomly allocated to 3 groups: sham, control, and VPA. The rats in the VPA and control groups received laminectomy at the L4 level of the vertebrae and silicone gel implantation into the epidural spaces L5 and L6. Rats in the sham group only received laminectomy at the L4 level of vertebrae without any implantation. VPA (300 mg/kg in saline) was administered 2 hours before the surgery. After the surgery, the VPA group received further VPA injections at 300 mg/kg twice a day for 1 week. The same volume of saline was injected in the control group. Neurobehavioral tests using the Basso, Beattie, Bresnahan scale and the oblique board test were performed for 1 week starting at 2 hours before surgery up to day 7 after surgery. At day 7 after surgery, tissues from the compressed cauda equina (L5-L6) were subjected to hematoxylin and eosin, luxol fast blue, or immunofluorescence staining, whereas the terminal deoxynucleotidyl transferase-mediated biotinylated UTP nick-end label assay staining was performed on the tissue from the dorsal root ganglions and the lumbar segment of the spinal cord proximal to the compressed cauda equina (L5-L6). RESULTS: The behavioral results suggested a significant improvement in the lower limb motor function in the VPA group compared with controls (P < 0.05). Furthermore, histologic assessment revealed a significant reduction in nerve fibers showing Wallerian degeneration and demyelinating lesions in the VPA group, in addition to an increased myelination compared with the control group (P < 0.05). The terminal deoxynucleotidyl transferase-mediated biotinylated UTP nick-end label assay staining revealed a significant decrease in the number of apoptotic neurons in the spinal cord anterior horn and dorsal root ganglions in the VPA group compared with controls (P < 0.05). CONCLUSIONS: Our data demonstrated that VPA could alleviate cauda equina injury, reduce apoptotic cells, and improve motor recovery, suggesting a neuroprotective effect in acute cauda equina syndrome.

[Intravascular Large B Cell Lymphoma with Cauda Equina Syndrome: A Case Report and Review of the Literature].

A 62-year-old man complained of gait disturbance, bladder and bowel dysfunction and paresthesia of both legs one month before admission. His symptoms were suggestive of cauda equina syndrome. After admission, he developed rapid progressive numbness and weakness of both legs and a disturbance of consciousness. A random skin biopsy was performed and a histological diagnosis of intravascular large B cell lymphoma (IVLBCL) was reached. His symptoms were improved after rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) therapy.

Protective effect of ginsenoside Rg1 on lidocaine-induced apoptosis.

Lidocaine, as an anesthetic substance, is often used for surface and spinal anesthesia. However, studies have shown that lidocaine may induce transient neurological symptoms and cauda equina syndrome. In the present study the effects of the ginsenoside Rg1 (Rg1) on lidocaine­induced apoptosis were assessed in Jurkat cells using flow cytometry and terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL). The data showed that incubation with Rg1 provides protection against lidocaine­induced apoptosis in cultured Jurkat cells. In order to investigate the effect of Rg1 on the apoptosis pathway, caspase 3 gene expression was determined. The results suggested that the protective effect of Rg1 on lidocaine­induced apoptosis is mediated by altering the level of B­cell lymphoma­2 (BCL­2) family proteins and downregulating caspase­3 expression. In conclusion, the present study demonstrated that incubation with Rg1 provides protection against lidocaine­induced apoptosis in cultured Jurkat cells. In addition, the study demonstrated that Rg1 is a notable antiapoptotic molecule that is capable of blocking the caspase­dependent signaling cascade in Jurkat cells, and that the protective effect of Rg1 on lidocaine­induced apoptosis is mediated by altering levels of BCL­2 family proteins and downregulating caspase­3 expression. The present study provides the basis for understanding and evaluating the effect of Rg1 in the in vivo treatment of lidocaine-induced transient neurological symptoms and cauda equina syndrome by lidocaine.

Successful treatment of infliximab-associated immune-mediated sensory polyradiculopathy with intravenous immunoglobulin.

Infliximab, a tumor necrosis factor-alpha antagonist, is used to treat many inflammatory diseases. Various forms of demyelinating neuropathies have been reported as neurological complications associated with infliximab use. There have been few reports of pure sensory neuropathy associated with infliximab. We report the clinical, electrophysiological, and pathological findings of a patient with subacute sensory polyradiculopathy 1 month after infliximab therapy for psoriasis vulgaris. Immune-mediated pathogenesis was suggested by positive anti-ganglioside antibodies and rapid response to intravenous immunoglobulin. This is the first reported case of sensory polyradiculopathy with positive anti-ganglioside antibodies following infliximab therapy. Our findings suggest the clinical importance of immunological investigations and treatment in demyelinating neuropathies following infliximab therapy.

Serotonergic 5-HT(1A) receptor agonist (8-OH-DPAT) ameliorates impaired micturition reflexes in a chronic ventral root avulsion model of incomplete cauda equina/conus medullaris injury.

Trauma to the thoracolumbar spine commonly results in injuries to the cauda equina and the lumbosacral portion of the spinal cord. Both complete and partial injury syndromes may follow. Here, we tested the hypothesis that serotonergic modulation may improve voiding function after an incomplete cauda equina/conus medullaris injury. For this purpose, we used a unilateral L5-S2 ventral root avulsion (VRA) injury model in the rat to mimic a partial lesion to the cauda equina and conus medullaris. Compared to a sham-operated series, comprehensive urodynamic studies demonstrated a markedly reduced voiding efficiency at 12 weeks after the VRA injury. Detailed cystometrogram studies showed injury-induced decreased peak bladder pressures indicative of reduced contractile properties. Concurrent external urethral sphincter (EUS) electromyography demonstrated shortened burst and prolonged silent periods associated with the elimination phase. Next, a 5-HT(1A) receptor agonist, 8-hydroxy-2-(di-n-propylamino)-tetralin (8-OH-DPAT), was administered intravenously at 12 weeks after the unilateral L5-S2 VRA injury. Both voiding efficiency and maximum intravesical pressure were significantly improved by 8-OH-DPAT (0.3-1.0 mg/kg). 8-OH-DPAT also enhanced the amplitude of EUS tonic and bursting activity as well as duration of EUS bursting and silent period during EUS bursting. The results indicate that 8-OH-DPAT improves voiding efficiency and enhances EUS bursting in rats with unilateral VRA injury. We conclude that serotonergic modulation of the 5-HT(1A) receptor may represent a new strategy to improve lower urinary tract function after incomplete cauda equina/conus medullaris injuries in experimental studies.

A case of sensory ataxia as the presenting manifestation of neurosarcoidosis.

Sarcoidosis rarely selectively affects the cauda equina with characteristic motor and sensory impairments.Using imaging, we report a case of cauda equina polyradiculopathy presenting with progressive sensory ataxia without clinical or electrophysiological evidence of motor involvement. Neurosarcoidosis was diagnosed pathologically by proximal dorsal root biopsy after systemic investigations for inflammatory, infectious, and neoplastic etiologies were found to be negative. There was clinical and radiographic improvement with corticosteroids. In addition, we review previously reported cases of cauda equina sarcoidosis.

Acute spinal cord compression caused by disseminated Nocardia infection involving the conus medullaris.

Distinguishing between an infective and malignant process provides a diagnostic challenge for clinicians. This case highlights an example of an acute spinal cord compression that could fall into either of these two categories. The diagnosis in this case of disseminated Nocardiosis is an extremely rare cause of acute spinal cord compression and to our knowledge intrinsic conus medullaris infection from Nocardia has not previously been reported in the literature. Nocardia cyriacigeorgica is an emerging strain of Nocardia species recently identified which was previously categorised as Nocardia asteroides type VI infection. The challenge of eliciting the diagnosis and the need to have an index of suspicion of Nocardia as a possible aetiology agent is shown in the report. The case shows this is especially important in evaluation of a multi-system infection in an immunosuppressed individual. The case described highlights an interesting diagnostic case with the resultant causative organism an emerging strain of Nocardia species with no previous reported cases of conus medullaris involvement.

Dramatic efficacy of infliximab in cauda equina syndrome complicating ankylosing spondylitis.

Cauda equina syndrome is an uncommon complication of ankylosing spondylitis (AS) characterized by the slow and insidious development of severe neurologic impairment. Imaging studies usually show a wide lumbar canal with dural ectasia. No medical or surgical treatment has been proven effective. We managed the care of a 66-year-old man who had longstanding AS and clinical features of cauda equina syndrome, including anal incontinence and buttock hypoesthesia. Magnetic resonance imaging demonstrated no cause for these symptoms other than AS. The patient was treated with infliximab, a monoclonal antibody to tumor necrosis factor alpha that is used for the treatment of active AS. After 3 infliximab infusions, sphincter control and sensation were normal. The treatment was continued, and he was still doing well 1 year later. This is the first report of an effective treatment for cauda equina syndrome complicating AS. Our case report strongly supports an inflammatory mechanism to this condition.

The efficacy of prostaglandin E1 derivative in patients with lumbar spinal stenosis.

STUDY DESIGN: Randomized controlled trial. OBJECTIVE: To examine the effect of limaprost, an oral prostaglandin (PG) E1 derivative, on health-related quality of life (HRQOL) in patients with symptomatic lumbar spinal stenosis (LSS), compared to etodolac, a NSAID. SUMMARY OF BACKGROUND DATA: Limaprost, an oral PGE1 derivative, was developed in Japan to treat numerous ischemic symptoms of thromboangiitis obliterans (TAO) and LSS. Previous studies have demonstrated the effectiveness of limaprost in the symptoms in patients with LSS. However, the evidence for effect on patient-reported outcomes, such as patient's HRQOL or satisfaction, is limited. METHODS: This study was conducted at 4 study sites in Japan. Briefly, inclusion criteria were: age between 50 and 85 years; presence of both neurogenic intermittent claudication (NIC) and cauda equina symptoms (at least presence of bilateral numbness in the lower limbs); and MRI-confirmed central stenosis with acquired degenerative LSS. Limaprost (15 microg/d) or etodolac (400 mg/d) was administered for 8 weeks. The primary outcome was Short Form (SF)-36, and the secondary outcomes were the verbal rating scale of low back pain and leg numbness, walking distance, subjective improvement, and satisfaction. RESULTS: A total of 79 participants were randomized (limaprost:etodolac = 39:40). Thirteen participants withdrew from the study (limaprost:etodolac = 5:8) and 66 completed the study (limaprost:etodolac = 34:32). Comparisons showed that limaprost resulted in significantly greater improvements in the SF-36 subscales of physical functioning, role physical, bodily pain, vitality, and mental health. Limaprost was also significantly better than etodolac for leg numbness, NIC distance, and subjective improvement and satisfaction. In the subgroup analysis stratified by symptom severity, limaprost seemed more effective for milder symptoms. No serious adverse effects were reported in either treatment group. CONCLUSION: In this study, limaprost was found to be efficacious on most outcome measures, such as HRQOL, symptoms and subjective satisfaction, in LSS patents with cauda equina symptoms.

Significant clinical improvement in radiation-induced lumbosacral polyradiculopathy by a treatment combining pentoxifylline, tocopherol, and clodronate (Pentoclo).

Radiation-induced (RI) peripheral neuropathy is a rare and severe delayed complication of radiotherapy that is spontaneously irreversible, with no standard of treatment. We previously developed a successful antioxidant treatment in RI fibrosis and necrosis. Two patients with progressive worsening RI lumbosacral polyradiculopathy experienced over several years a significant clinical improvement in their neurological sensorimotor symptoms with long-term pentoxifylline-tocopherol-clodronate treatment, and good safety.

Cauda equina syndrome due to leptomeningeal carcinomatosis of the ovary.

BACKGROUND: Serous cystadenocarcinoma is the most common malignant ovarian tumor. 85% are associated with extraovarian spread at the time of diagnosis. Cauda equina syndrome due to leptomeningeal ovarian serous cystadenocarcinomatosis is rare. CASE: A 66-year-old female with stage IV ovarian papillary serous cystadenocarcinoma presented with perianal numbness and sphincter dysfunction. On exam she had decreased anal tone with saddle anesthesia. Her MRI did not demonstrate any leptomeningeal involvement. CSF showed malignant cells consistent with metastatic ovarian adenocarcinoma. She received intrathecal methotrexate, capecitabine and bevacizumab. She expired 8 months later. CONCLUSION: Ovarian cancer metastasizng to the cauda equina should be highly suspected based on the clinical presentation alone, even with unremarkable imaging studies. CSF cytology should be checked in cases presenting with cauda equina syndrome.

Malignant lymphoma originating in the cauda equina mimicking the inflammatory polyradiculoneuropathy.

A 67-year-old woman was diagnosed with inflammatory polyradiculoneuropathy. The intravenously administered immunoglobulin (IVIG) treatment that she received several times over a 3-year period relieved her clinical symptoms of muscle weakness and sensory disturbances, but these symptoms had worsened thereafter despite further IVIG treatment. MRI detected a solid tumor involving the cauda equina and pathological examinations confirmed this to be malignant lymphoma. The clinical and radiological findings for the malignant lymphoma of the cauda equina in this patient were quite similar to those for the inflammatory polyradiculoneuropathy.

Cauda-conus syndrome resulting from neurocysticercosis.

A 60-year-old male, presented with insidious onset, gradually progressive, burning paresthesia over the saddle area, sphincteric disturbance, impotence and paraparesis. Investigations revealed a ring-enhancing lesion in the conus medullaris suggestive of neurocysticercosis. This was supported by quantitative enzyme-linked immunosorbant assay from purified cell fraction of taenia solium cysticerci. On treatment with steroids he showed marked improvement.

[Extrapulmonary complications of Mycoplasma pneumoniae infections]. / Ekstrapulmonale komplikationer ved mycoplasma pneumoniae-infektioner.

INTRODUCTION: Mycoplasma pneumoniae is a common cause of atypical pneumonia in children and young adults. The infection is generally mild and only a very few patients are admitted to hospital. However, extrapulmonary complications are well recognised--mostly as manifestations from the central nervous system (CNS). MATERIAL AND METHODS: We describe 21 patients with M. pneumoniae infection seen at Rigshospitalet, Copenhagen, from 1994 to 2000. RESULTS: The patients had fever, headache, myalgia, and cough. Biochemically, they were characterised by leucocytosis, an increased level of C-reactive protein, and infiltrations on the chest x-ray. A total of seven patients developed extrapulmonary complications to the infection in the form of encephalitis (3), polyradiculitis (1), transversel myelitis (1), erythema multiforme (3), cardial arrhythmia (1), and haemolytic anaemia (2). Three patients had more than one complication at the same time. DISCUSSION: The incidence of patients with complications to M. pneumoniae infection was higher than that reported in the literature, probably because these patients are typically submitted to the Department of Infectious Diseases, Rigshospitalet. The pathogenesis of extrapulmonary complications to M. pneumoniae infection is unknown.

Hydroxyurea therapy in paraparesis and cauda equina syndrome due to extramedullary haematopoiesis in thalassaemia: improvement of clinical and haematological parameters.

Patients with beta-globin disorders show amelioration of clinical condition by sustained synthesis of fetal haemoglobin in adult life. We report data on a patient with beta(o)-thalassaemia genotype and thalassaemia intermedia clinical phenotype. He received therapy with hydroxyurea (20 mg/kg/d) because of the presence of extramedullary masses causing paraparesis, neurogenic bladder and impotence. During therapy, the patient showed an improved clinical picture and a significant increase in total Hb (from 71.8 to 103.2 g/L) and a gamma/alpha globin synthetic ratio (from 0.39 to 0.68). The myelosuppressive effect of hydroxyurea was revealed by a decrease in CFU-GEMM, BFU-E, and CFU-GM. Therefore hydroxyurea can be effective in the treatment of patients with extramedullary haematopoiesis (EMH) who are not transfusion-dependent and cannot be treated with radiotherapy.