PURPOSE: The primary goal of this study was to evaluate the MR findings of systemic lupus erythematosus (SLE) patients with neuropsychiatric symptoms. MATERIALS AND METHODS: The MR images of 38 patients with SLE were evaluated based on the presence of the following abnormal lesions: the locations of the abnormal signal intensity lesions in the white matter, infarctions, a small vessel vasculopathy, leukoencephalopathy, hemorrhage, abscess, and other lesions. RESULTS: The MR images showed an abnormality in 22 of 38 (58%) episodes. Abnormal signal intensities were noted in the subcortical and periventricular white matter in six cases, acute territorial infarctions in five cases, multiple small acute embolic infarctions in four cases and a brain abscess in two cases. A reversible posterior leukoencephalopathy was found in one case. In addition, another patient had vasogenic edema with focal central cytotoxic edema at the pons. The entire cerebral and corpus callosum volumes were significantly smaller in four patients with SLE as compared to the volumes in healthy control subjects. CONCLUSION: SLE may induce variable MR imaging findings of the CNS. Recognition of the variable findings is helpful for easy diagnosis and prompt treatment.
Abscess ; Brain ; Brain Abscess ; Brain Diseases ; Central Nervous System ; Corpus Callosum ; Edema ; European Continental Ancestry Group ; Glycosaminoglycans ; Hemorrhage ; Humans ; Infarction ; Leukoencephalopathies ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System ; Pons

Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare white matter disorder, first described in the early 1990s. The brain in patients with MLC appears swollen on MRI, with diffuse white matter abnormalities; in addition, there is an invariable presence of subcortical cysts, primarily in the anterior temporal region sparing the deep white matter, basal ganglia, thalami, and cerebellum. Patients with MLC present with macrocephaly and neurological abnormalities such as motor deterioration, ataxia, spasticity, and cognitive deficits. We report a twenty-month-old boy who presented with seizures and macrocephaly, delay in development, and abnormal brain MRI findings compatible with the diagnosis of MLC. The brain MRI revealed bilateral hypersignal intense subcortical white matter regions in the frontal, temporal, and parietal lobes on T2-weighted images, which were not yet associated with cystic changes. During follow-up, the frequency of seizures decreased after anticonvulsant medication was started, but the head circumference remained above the 97th percentile, and the patient continued to have developmental delay.
Ataxia ; Basal Ganglia ; Brain ; Cerebellum ; Cysts ; Developmental Disabilities ; Follow-Up Studies ; Head ; Hereditary Central Nervous System Demyelinating Diseases ; Humans ; Leukoencephalopathies ; Macrocephaly ; Muscle Spasticity ; Parietal Lobe ; Seizures

No abstract available.
Brain* ; Central Nervous System Diseases* ; Central Nervous System* ; Child* ; Humans

No abstract available.
Brain* ; Central Nervous System Diseases* ; Central Nervous System* ; Child* ; Humans

Kidney impairment due to acute kidney injury or chronic kidney disease is a potent risk factor for stroke which is a leading cause of morbidity and mortality worldwide. Patients with kidney impairment have various neurologic complications, including uremic encephalopathy, polyneuropathy, and cognitive impairment as well as higher rates of ischemic and hemorrhagic stroke and frequent seizures. Due to hypertension, coagulopathy, platelet dysfunction, and vascular disease, patients with kidney impairment are at high risk for types of catastrophic intracranial hemorrhages and strokes that typically lead to intracranial hypertension and cerebral herniation syndrome. Kidney impairment can alter drug pharmacokinetics and pharmacodynamics, and consequently patients with kidney impairment are at risk of experiencing adverse effects. Several central nervous system imaging modalities are not recommended in patients with compromised kidney function. Therefore, management of acute neurological conditions requires special attention in patients with kidney impairment. Given these common acute neurological conditions, physicians who care for patients with kidney impairment must be aware of evaluation and treatment of neurological diseases to achieve positive neurological outcomes.
Acute Kidney Injury ; Blood Platelets ; Brain Diseases ; Central Nervous System ; Cerebrovascular Disorders ; Cognition Disorders ; Humans ; Hypertension ; Intracranial Hemorrhages ; Intracranial Hypertension ; Kidney Diseases ; Kidney ; Mortality ; Pharmacokinetics ; Polyneuropathies ; Renal Insufficiency, Chronic ; Risk Factors ; Seizures ; Stroke ; Vascular Diseases

Previous concepts of immune-privileged sites obscured the role of peripheral immune cells in neurological disorders and excluded the consideration of the potential benefits of immunotherapy. Recently, however, numerous studies have demonstrated that the blood-brain barrier in the central nervous system is an educational barrier rather than an absolute barrier to peripheral immune cells. Emerging knowledge of immune-privileged sites suggests that peripheral immune cells can infiltrate these sites via educative gates and that crosstalk can occur between infiltrating immune cells and the central nervous system parenchyma. This concept can be expanded to the testis, which has long been considered an immune-privileged site, and to neurogenic bladder dysfunction. Thus, we propose that the relationship between peripheral immune cells, the brain, and the urologic system should be considered as an additional possible mechanism in urologic diseases, and that immunotherapy might be an alternative therapeutic strategy in treating neurogenic bladder dysfunction.
Blood-Brain Barrier ; Brain ; Central Nervous System ; Immune System* ; Immunotherapy ; Nervous System Diseases* ; Testis ; Urinary Bladder, Neurogenic ; Urologic Diseases ; Urology*

We report an 11-month-old male who presented with recurrent seizures, subdural bleed, skull fracture, lightly pigmented hair, and fair lax skin. Copper and ceruloplasmin levels were low and gross deletion of ATP7A gene was found confirming the diagnosis of Menkes disease. The presence of subdural bleed and skull fracture prompted a referral to the Child Protection Unit to rule out child abuse.
Human ; Male ; Female ; Adult ; Infant ; MENKES KINKY HAIR SYNDROME ; NERVOUS SYSTEM DISEASES ; CENTRAL NERVOUS SYSTEM DISEASES ; BRAIN DISEASES ; BRAIN DISEASES, METABOLIC ; BRAIN DISEASES, METABOLIC, INBORN ; CERULOPLASMIN ; COPPER

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease that is a significant source of morbidity and mortality when it manifests in the central nervous system. The early detection and treatment of neuropsychiatric SLE (NPSLE) is very important, but a confirmative diagnostic tool has yet to be developed. CASE REPORT: We report here a case of neuropsychiatric manifestations in a patient that were associated with SLE, and evidence of reversal of bilateral amygdala and parahippocampal lesions in the brain revealed by 18fluorodeoxy glucose-positron emission tomography. CONCLUSIONS: We are suggestive of 18fluorodeoxy glucose-positron emission tomography appear to be more sensitive in detecting subtle brain changes in NPSLE.
Amygdala* ; Autoimmune Diseases ; Brain* ; Central Nervous System ; Humans ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System* ; Mortality

A central nervous system (CNS) relapse is a rare but mostly fatal complication in patients with diffuse large B cell lymphoma (DLBCL). CNS involvement can occur as an isolated event or can be combined with progression of systemic disease. There are limited data on treatment outcomes of patients with DLBCL and secondary CNS involvement. We report the clinical data, treatments, and outcomes of two DLBCL patients with isolated CNS relapses involving the brain parenchyma. Isolated CNS disease involving the brain parenchyma may be potentially treatable as the initial relapse site after complete remission from systemic treatment.
Brain* ; Central Nervous System ; Central Nervous System Diseases ; Humans ; Lymphoma, B-Cell* ; Lymphoma, Large B-Cell, Diffuse ; Recurrence*

An increasing number of neuronal autoantibodies which target cell surface or synaptic proteins have been discovered over the last decade. Autoimmune encephalitis refers to this new category of autoimmune-mediated neurological disorders, which involve the central nervous system. Recent studies have established that autoimmune encephalitis is now the major cause of encephalitis, which was previously considered to be encephalitis of an unknown etiology. Moreover, the fact that autoimmune encephalitis is potentially treatable with immunomodulating therapy has changed the paradigm for the diagnosis and treatment of acute encephalitis syndrome. We herein review the pathophysiology, clinical manifestations, diagnosis, and treatment of autoimmune encephalitis with a focus on corticosteroid therapy as the first-line immunotherapy. In addition, regarding the diagnostic approach, we emphasize the differentiation between autoimmune and infectious encephalitis, because this distinction is not necessarily clear-cut in real clinical practice and should be considered when determining the initiation and type of immunotherapy.
Autoantibodies ; Autoimmune Diseases of the Nervous System ; Central Nervous System ; Diagnosis ; Encephalitis ; Glucocorticoids ; Immunotherapy ; Infectious Encephalitis ; Nervous System Diseases ; Neurons