Cervical Meninges Research Articles

Algal Meningoencephalitis due toProtothecaspp. in a Dog

A 6-year-old Boxer was examined because of progressive neurologic signs, with severe hindlimb ataxia and head tilt on presentation. There was no history of diarrhea or vomiting. MRI of the brain revealed multifocal ill-defined T1-enhancing lesions affecting the cerebrum, brainstem, and cervical meninges, without associated mass effect. Meningoencephalitis was considered the most likely diagnosis. Multiple algae were observed on the cytology of the CSF and were most consistent withProtothecaspp. Antiprotozoal treatment was denied by the owners, and 5 weeks after diagnosis, the dog was euthanized due to progression of the neurologic deficits, and a necropsy was performed. Histological changes in the brain were compatible with severe multifocal protothecal meningoencephalitis. The specificProtothecaspecies was not identified. The gastrointestinal tract was unremarkable on histology. According to this report,Protothecaspp. should be included in the differentials for neurological deficits even in the absence of gastrointestinal signs.

Pathogenetic factors for excessive IgA production: Th2-dominated immune response in canine steroid-responsive meningitis-arteritis

Canine steroid-responsive meningitis-arteritis (SRMA) is a systemic inflammatory disease with a predominant manifestation within the cervical meninges, increased immunoglobulin A (IgA) levels in serum and cerebrospinal fluid (CSF), and a shift of the B:T cell ratio towards a higher percentage of B cells. A Th2-dominated immune response associated with SRMA was therefore hypothesised. Pellets of peripheral blood mononuclear cells (PBMNCs) and CSF white blood cells (CSF WBCs) from dogs in the acute phase of SRMA (n=16) and under glucocorticoid treatment for SRMA (n=16) were investigated for interleukin (IL)-2, interferon (IFN)-γ, IL-4, IL-5 and IL-10 mRNA expression by means of reverse-transcriptase real-time polymerase chain reaction. Results were compared with those of dogs with other inflammatory (n=9) and neoplastic disorders (n=10) of the central nervous system. A tendency towards low levels of Th1 response related cytokines (IL-2, IFN-γ) and high IL-4 expression was observed indicating a Th2-skewed immune response. The pronounced IL-4 production may be an important pathogenetic factor for excessive IgA production in the acute phase of SRMA and for those cases under glucocorticoid treatment.

Intrathecal administration of recombinant human N-acetylgalactosamine 4-sulfatase to a MPS VI patient with pachymeningitis cervicalis

In mucopolysaccharidosis VI, or Maroteaux–Lamy syndrome, deficiency of N-acetylgalactosamine 4-sulfatase leads to storage of glycosaminoglycans (GAGs) and MPS VI patients often develop spinal cord compression during the course of the disease due to GAG storage within the cervical meninges, requiring neurosurgical intervention, as intravenous (IV) enzyme replacement therapy (ERT) is not expected to cross the blood–brain barrier. We report the use of intrathecal (IT) recombinant human N-acetylgalactosamine 4-sulfatase (arylsulfatase B, or ASB) in a MPS VI child with spinal cord compression whose parents initially refused the surgical treatment. Assessments were performed at baseline, with clinical, neurological and biochemical evaluations, urodynamic studies and MRI of the CNS. Changes on these parameters were evaluated after IT infusions of ASB administered monthly via lumbar puncture (LP) in a IV ERT naive patient. To our knowledge, this was the first MPS VI patient who received IT ERT. Despite significant urodynamic improvement and some neurological amelioration, the patient developed worsening of walking capacity. After IV ERT was started, the patient presented with a generalized hypotonia and a life-saving surgical fixation of the neck was then performed. The results observed on this MPS VI patient suggest that instability of the cervical vertebrae could be unmasked by IV ERT as joint storage is reduced, and the decrease in neck stiffness and stability could confound the expected improvement of SCC manifestations following IT ERT. The study of further patients, if possible in a clinical trial setting, is needed to evaluate the potential of a non-surgical IT ERT treatment of SCC for MPS VI.

Intrathecal enzyme replacement therapy in a patient with mucopolysaccharidosis type I and symptomatic spinal cord compression

In mucopolysaccharidosis I, deficiency of alpha-L-iduronidase can cause spinal cord compression (SCC) due to storage of glycosaminoglycans (GAGs) within the cervical meninges. As intravenous enzyme replacement therapy (ERT) is not likely to provide enzyme across the blood-brain barrier, standard treatment for this complication is usually surgical, which has a high morbidity and mortality risk. We report on the use of intrathecal (IT) laronidase in a MPS I patient with SCC who refused the surgical treatment. Assessments were performed at baseline, with clinical and biochemical evaluations, 4-extremity somatosensory evoked potentials, 12 min walk test and MRI studies of the CNS. Changes on these parameters were evaluated after 4 IT infusions of laronidase administered monthly via lumbar puncture. To our knowledge, this was the first MPS patient who received IT ERT. No major adverse events were observed. There were no clinically significant changes in serum chemistries. CSF GAG results revealed pretreatment values slightly above normal standards: 13.3 mg/L (NV < 12 mg/L) which after IT laronidase infusions were within normal levels (10.3 mg/L). 12MWT presented a 14% improvement, with better performance on stability and gait control. Maximum voluntary ventilation showed 55.6% improvement considering the percentage of predicted (26.7% at baseline compared to 41.9%); Maximum Inspiration Pressure improved 36.6% of predicted (26.8% at baseline to 36.7%); Pulmonary diffusion improved 17.6% of predicted %. In conclusion, although the improvement observed in this case with IT laronidase should be confirmed in further patients, this procedure seems to be a safe treatment for SCC in MPS I.

Selective CD11a upregulation on neutrophils in the acute phase of steroid-responsive meningitis-arteritis in dogs

Steroid-responsive meningitis-arteritis (SRMA) is a systemic inflammatory disease of juvenile to young adult dogs with a relapsing course and most prominent manifestation in the cervical meninges. The most important laboratory finding is a marked neutrophilic pleocytosis. Integrin (CD11a, b, c) expression on polymorphonuclear cells (PMNs) was quantified by immunophenotyping and subsequent flow cytometric measurements. Values were determined for peripheral blood in the acute phase of SRMA ( n = 14) as well as during glucocorticosteroid treatment ( n = 16). Results were compared to those from dogs with other neurological diseases ( n = 49) and healthy individuals ( n = 7). Integrin expression was also investigated on PMNs deriving from cerebrospinal fluid (CSF) of dogs in the acute phase of SRMA ( n = 14). In a second part of the study PMNs of healthy dogs were incubated with sera of dogs in the acute phase of SRMA ( n = 12). The influence on integrin expression was studied and results were compared to those after incubation with pooled sera of dogs suffering from idiopathic epilepsy ( n = 3). PMNs in peripheral blood of dogs in the acute phase of SRMA showed higher values of CD11a expression when compared to dogs under treatment and to control groups, whereas CD11b and c expression was comparable among the different groups. In the acute phase of SRMA CD11b expression on PMNs in CSF was increased in comparison to that in peripheral blood. Incubation with SRMA sera caused a stronger upregulation of CD11a than did pooled epilepsy sera in 9/12 cases whereas an upregulation of CD11b and c was observed in single cases only. High CD11a expression on PMNs in peripheral blood appears to be an important factor in the pathogenesis of SRMA. This integrin is known to be essential for adhesion of PMNs within the neutrophil recruitment cascade and therefore might mediate the enhanced invasion of neutrophils into the subarachnoidal space eventually leading to meningitis and clinical signs. Since sera of dogs suffering from SRMA selectively induce an upregulation of CD11a it can be suspected that this fluid contains one or multiple factors being responsible for this.

Disproportionally strong increase of B cells in inflammatory cerebrospinal fluid of dogs with Steroid-responsive Meningitis-Arteritis

Steroid-responsive Meningitis-Arteritis (SRMA) is a systemic inflammatory disease of juvenile to young adult dogs with a relapsing course and most prominent manifestation in the cervical meninges. Immunophenotyping and flow cytometric measurement of lymphocytes in peripheral blood (PB) and CSF was performed in the acute phase of SRMA ( n = 12) and during glucocorticosteroid treatment ( n = 10). Values were compared to those from dogs with other neurologic diseases ( n = 63) and healthy individuals ( n = 7). Dogs with SRMA had high CD4:CD8α ratios in PB and low T:B cell ratios in PB and CSF suggesting that a T H2-mediated immune response occurs. The T:B cell ratio in CSF was markedly lower than that in PB indicating that either a selective recruitment of B cells or, alternatively, their strong intrathecal proliferation takes place. SRMA appears to be a valuable animal model for the investigation of compartmentalization of immune responses and for studies on differences in local central nervous system and systemic immune responses.

Meningoencefalomielitis como manifestación inicial de un tumor cerebral que imita una encefalitis por virus del herpes simple: descripción de un caso

Meningoencephalomyelitis can be due to a number of treatable causes. A tumoural aetiology is rare and they are generally malignant tumours with an ominous prognosis. We report a case of meningoencephalomyelitis that initially presented as encephalitis due to herpes simplex virus (HSV) and which was finally seen to be an anaplastic oligoastrocytoma. We describe the case of a 68-year-old male with a history of just strong low back pain during the previous month, who was admitted to hospital because of progressive clinical symptoms involving cognitive impairment and myoclonias. The polymerase chain reaction for the cerebrospinal fluid was positive for HSV and magnetic resonance imaging revealed diffuse compromise of right temporal lobe, the spinal cord and the cervical meninges. The progressive deterioration of the patient despite treatment with acyclovir, anti-tuberculosis agents and with corticoids made it necessary to perform a biopsy study of the cervical meninges, the results of which suggested non-specific macromonocytic meningoencephalitis. The patient's condition continued to deteriorate until he died. The post-mortem examination revealed a grade III oligoastrocytoma in both temporal lobes, which had extended into the adjacent subarachnoid space and the cerebral and cervical leptomeninges. Non-specific symptoms of low back pain can conceal a brain tumour. Attention is drawn to how infrequently it manifests clinically and in imaging studies as meningoencephalomyelitis due to direct tumoural invasion. This should be considered as a possibility when faced with a slowly progressing clinical picture that, despite the initial suspicion of encephalitis due to HSV, does not respond to the usual treatment.

A Case of Fibrosarcoma of the Cervical Meninges

A Case of Fibrosarcoma of the Cervical Meninges

The role of birth in the pathogenesis of meningeal haemorrhage and congestion in newborn lambs.

SUMMARY: In a controlled experiment, term lambs born of caesarean sacrifice, normal or artificially prolonged birth were examined at autopsy to determine the role of vaginal birth in the pathogenesis of presumed birth injury to the fetal CNS characterised by subdural and extradural haemorrhage, subarachnoid haemorrhage and congestion, and blood‐stained cerebrospinal fluid in and around the cranial and spinal meninges of lambs dying during or within 7 days of birth. Additional material, comprising near‐term fetuses, was examined at an abattoir to determine the prevalence of meningeal lesions in unborn fetuses.Mean durations of parturition in the caesarean‐born controls, natural and artifically‐delayed vaginal deliveries were 0 min., 56 min., and 185 min. respectively. The experimental treatments influenced the frequency of cranial lesions only. Single lambs were more prone to injury than twin lambs. The incidence of affected lambs in delayed births was 8.9 times higher than that in caesarean births and 1.7 times higher than that in normal births. Within the delayed group the incidence of affected single lambs in the subgroups was similar despite wide differences in the nature of parturition. It was concluded that the induced lesions resembled those associated with the perinatal mortality of livestock and infants and that pathogenetic variables included interactions between the duration and the vigour of the birth process. Repeated stretching and relaxation of the rostral fetal cranio‐vertebral skeleton during Stage 2 labour may be involved in pathogenesis. At that stage of delivery, the fetus is relatively fixed because the chest and shoulders have firmly engaged the pelvic inlet, while the head and neck are exposed to the lower pressure of the vagina and atmosphere. Intrafetal pressure is hydrostatically continuous with intrauterine pressure which is higher than atmospheric pressure. Rises in intrauterine pressure due to labour are probably transmitted hydraululically to the fetal cranio‐vertebral canal leading to stretching of the head and neck. Because the fetal CNS is firmly anchored to the cranio‐vertebral skeleton such movement may set up the shearing action necessary to rupture small vessels in the cranial and cervical meninges.

Multiple melanotic meningiomas of the cervical cord. Case report.

✓ The clinical course and pathological findings are described in a patient who survived 17 years following her first operation for multiple encapsulated extradural and intradural melanotic meningiomas of the cervical meninges. Although the recurrence of these tumors suggested a malignant histological component, the long history of survival, the restricted location, and absence of melanotic tumors elsewhere, and the basic histological background all favored an uncommon variant of meningioma.