Multisystemic leukocytoclastic vasculitis affecting the central nervous system

Leukocytoclastic vasculitis is a self-limiting disease with rare systemic complications in the majority of patients. This report describes a 10-year-old male with leukocytoclastic vasculitis caused by an insect bite who presented with central nervous system, lung, kidney, and gastrointestinal involvement in addition to cutaneous findings. The diagnosis was confirmed by histopathologic examination of cerebral hematoma material. Initially, his symptoms improved with a treatment protocol including steroid, cyclophosphamide, intravenous immunoglobulin, and plasmapheresis, but the patient succumbed because of nosocomial sepsis.     

Small vessel vasculopathies affecting the central nervous system.

Small vessel diseases affecting the central nervous system are common causes of ischemic and hemorrhagic stroke. Diseases affecting the small vessels are due either to abnormalities in the content of the vessel or to abnormalities in the vessel wall. Causes include atherosclerosis, inflammatory vasculitides, and noninflammatory vasculopathies such as postpartum angiopathy, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), Susac syndrome, and Sneddon syndrome.     

仅累及中枢神经系统血管炎相关分析

目的初步探讨仅累及中枢神经系统血管炎的临床特点、检查及治疗方法。方法对诊治的11例仅累及神经系统血管炎的患者进行相关分析。结果患者多年轻化,临床表现多样化,磁共振表现无特征性,免疫系统相关检查阴性,大多对激素治疗有效。结论年轻患者,反复脑血管病,而无脑血管病危险因素,即使免疫相关检查正常,也要高度警惕仅累及中枢神经系统血管炎可能,及早完善DSA,尽早激素干预治疗。     

Demyelinating disease affecting both central and peripheral nervous system

Introduction

Demyelinating disease affecting both the central and the peripheral nervous systems has rarely been reported.

Case report

A 30-year-old man, presented with ataxia and diffuse areflexia due to polyneuropathy fullfilling demyelination criteria. His medical history was notable for central nervous system demyelination compatible with multiple sclerosis. He improved transiently with intravenous immunoglobulin and then stabilized with methotrexate.

Conclusion

This case report distinguishes a new kind of inflammatory disease affecting both central and peripheral nervous system. It seems to be different from multiple sclerosis and chronic immune demyelinating polyneuropathy, because of high hyperproteinorachia and absence of oligoclonal bands in the cerebrospinal fluid.     

Mitochondrial transplantation strategies as potential therapeutics for central nervous system trauma

Mitochondria are essential cellular organelles critical for generating adenosine triphosphate for cellular homeostasis, as well as various mechanisms that can lead to both necrosis and apoptosis. The field of mitochondrial medicine is emerging in which injury/disease states are targeted therapeutically at the level of the mitochondrion, including specific antioxidants, bioenergetic substrate additions, and membrane uncoupling agents. Consequently, novel mitochondrial transplantation strategies represent a potentially multifactorial therapy leading to increased adenosine triphosphate production, decreased oxidative stress, mitochondrial DNA replacement, improved bioenergetics and tissue sparing. Herein, we describe briefly the history of mitochondrial transplantation and the various techniques used for both in vitro and in vivo delivery, the benefits associated with successful transference into both peripheral and central nervous system tissues, along with caveats and pitfalls that hinder the advancements of this novel therapeutic.     

Edematous changes in the central nervous system of zitter rats with genetic spongiform encephalopathy

The pathogenesis of progressive spongy degeneration in zitter rats with genetic spongiform encephalopathy was examined histopathologically and biochemically in the context of edematous change in the central nervous system (CNS). Histopathological studies revealed that vacuolation in the CNS of zitter rats progressed with aging and the severity of spongy degeneration was markedly divergent in different areas in the CNS. Edematous change was confirmed by a consistently higher water content in the brain of zitter rats than in that of normal SD/J rats at all ages. Furthermore, a close relationship between spongy degeneration and edematous change in the CNS was demonstrated by regional measurement of specific gravity (SPGR) of brain tissues and quantification of the spongy degeneration by computer-image analysis. The brain regions with lighter SPGR were more severely affected by spongy degeneration. These results suggest that edematous change is related to the pathogenesis of spongy degeneration in the CNS of the zitter rat.     

Mutations affecting myelination in the central nervous system: research tools in neurobiology

Myelin formation requires the intimate involvement of both neurons and certain types of glial cell. It is thus an extremely difficult process to study. Here N. Baumann describes some elegant experiments in which myelin deficient mutants are used to probe some of the biochemical processes underlying development of the myelin sheath.     

Mitochondrial encephalopathy

Mitochondrial disorders cause a wide spectrum of diseases in children. Their presentation is nonspecific with encephalomyopathy, failure to thrive, seizures, ophthalmoplegia, and sensorineural hearing loss. These disorders are progressive and are aggravated by fever and infections. They can be caused by mutations in nDNA or mtDNA. Diagnosis requires a complex battery of clinical studies coupled with diagnostic findings on muscle biopsy (abnormal structure, histochemistry, or enzyme studies) or DNA testing. Therapy for mitochondrial disorders remains largely ineffective.     

Mitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system

The mnd mouse spontaneously develops slowly evolving motoneuron pathology leading to progressive motor impairment. There is strong evidence that a complex interplay between oxidative stress, mitochondria abnormalities and alteration of glutamate neurotransmission plays an important role in the pathogenesis of motor neuron diseases. Therefore, we investigated the presence of mitochondrial dysfunction in frontal, central (comprising the motor area) and occipital regions of the cerebral cortex and in the spinal cord of 35-week-old mnd mice. Lipid peroxide derivatives reacting with thiobarbituric acid (TBARS) were measured in the cervical, thoracic and lumbar spinal cord. In addition biochemical and behavioural analyses were carried out in mnd mice chronically treated with l-carnitine from the 11th to the 34th week of life (mndT mice). Slight but significant alterations of mitochondrial enzyme activities were seen in the mnd cortical regions. The central area was the most affected and both complex I, IV and citrate synthase were decreased with respect to controls. The rate of oxygen consumption (QO2) was markedly decreased in both the upper (cervical + upper portion of the thoracic region) and lower (lumbar + lower portion of the thoracic region) mnd spinal cord. The level of TBARS showed a rostro-caudal trend to increase, being 30% higher in the lumbar tract of mnd mice in comparison with controls. L-carnitine treatment increased the mitochondrial enzyme activities in cortical regions towards control value and was effective in enhancing QO2 and decreasing TBARS levels in the spinal cord of mndT. Behavioural testing showed that L-carnitine significantly delayed the onset of motor behaviour impairment. This beneficial effect was declining at 35 week of age, when the biochemical measurements were performed.     

Primary Ewing's sarcoma affecting the central nervous system: a review and proposed prognostic considerations

Ewing's sarcoma (ES) is a part of a larger family of round blue cell tumors, which occasionally manifest as osseous or extraosseous lesions adjacent to or within the central nervous system (CNS). While a large body of literature exists on ES of bone, data are lacking on tumors with cranial or spinal components that affect the CNS. Here, we perform a systematic review of the literature and summarize the best available evidence on diagnosis, treatment and outcomes of ES affecting the CNS with emphasis on the breadth of clinical presentations, diagnostic tools and emerging management options for these rare and challenging lesions. We include a review of known prognostic factors and propose several new considerations for prognostication of ES affecting the CNS.     

Immunolocalization of cathepsin D in the human central nervous system and central nervous system neoplasms

The cellular distribution of the lysosomal proteinase cathepsin D was studied in a series of 76 neoplasms and 18 non-neoplastic tissues from the human central nervous system, using a well-characterized polyclonal antibody in a peroxidase-antiperoxidase technique. In the normal and developing brain, cathepsin D is confined to neurons and choroid plexus epithelium. Strong granular cytoplasmic staining was present in neuronal and choroid plexus neoplasms, and in reactive macrophages. A large variety of other neoplasms also exhibited positive cytoplasmic staining, albeit usually of a weaker diffuse type. Cathepsin D cannot be considered a specific marker for neuronal or choroid plexus neoplasms, but the antiserum used in this study may be of value in antibody panels for the investigation of these tumours. Its localization may also be of value in embryological studies, particularly in the cerebellum, and in investigations of steroid hormone receptor-associated proteins in meningiomas and Schwannomas.     

Does the immune system communicate with the central nervous system? Interferon modifies central nervous activity

The present investigation determined whether an immunomodulator agent modified the central nervous system activity as measured behaviorally and neurophysiologically. Two types of interferons (IFNs), alpha (alpha) and gamma (gamma), were applied locally (microiontophoretically) into various regions of the rat brain simultaneously with single neuron recording from the cerebral cortex, hippocampus, thalamus and hypothalamus. Of the various IFNs, only alpha-IFN altered single cell activity in all brain structures in a dose-dependent manner. Moreover, systemic administration of alpha-IFN altered the naloxone-induced abstinence syndrome in morphine-dependent rats. These observations suggest that immunomodulators such as alpha-IFN are capable of influencing directly central nervous system function as well as the immune system.     

Hexachlorophene and the central nervous system

Summary A study on hexachlorophene encephalopathy in mice and baboons is reported. By light microscopy, a severe spongiform lesion of the central nervous system (CNS) was localized in the white matter, without myelin breakdown or cellular reaction. By electron microscopy, the myelin alteration was characterized by wide intralamellar spaces or splitting developed in the intraperiod line of compact sheaths. The acute changes described were induced by administration of the drug by the digestive or cutancous routes at various dosage levels in an aqueous solution or in talcum powder. The toxic effects depended on the age of the animals, the survival times and the concentrations of hexachlorophene, i.e., 6%, 3%, and 0.5%. The findings are compared with previous reports on the neurotoxicity of hexachlorophene and other chemicals in humans and experimental animals. Hexachlorophene cannot be recommended for use in young infants because of its neurotoxicity in very low doses as demonstrated in the present report.     

Vasculitis of the central nervous system

Central nervous system (CNS) involvement and management in primary vasculitis (giant cell arteritis, primary angiitis of the CNS, Takayasu’s disease, periarteritis nodosa, Kawasaki disease, Churg-Strauss syndrome, Wegener’s granulomatosis) and vasculitis secondary to collagen vascular diseases, Beçhet’s disease and other systemic conditions (excluding those secondary to infections) and the use of illicit drugs are reviewed. Vasculitis is an infrequent cause of stroke even in the young age groups, and routine screening of stroke patients for vasculitis is not cost-effective. CNS vasculitis may present with isolated CNS symptoms and signs (headaches, meningeal signs, encephalopathy, psychiatric syndromes, dementia, cranial nerve palsies, seizures, strokes), neuropathy or muscle damage, multiorgan involvement or non-specific systemic symptoms or a combination of the above. Magnetic resonance is the most sensitive ancillary procedure to detect CNS damage. Many of the angiographic features found in vasculitis are non-specific. Autoantibodies and tissue biopsy are also useful to the diagnosis. There are few controlled studies on the treatment of vasculitis. Usually a combination of steroids and cytotoxic drugs is used, but there is considerable variation between centres on current therapeutic regimens.