Subject(s)
Parathyroid Hormone , Animals , Calcium/metabolism , Carrier Proteins/physiology , Electrolytes/metabolism , Humans , Hyperparathyroidism, Primary/etiology , Hypoparathyroidism/etiology , Kidney Tubules/metabolism , Membrane Glycoproteins/physiology , Osteogenesis , Parathyroid Hormone/biosynthesis , Parathyroid Hormone/genetics , Parathyroid Hormone/metabolism , Parathyroid Hormone/physiology , Parathyroid Hormone-Related Protein/genetics , Parathyroid Hormone-Related Protein/physiology , RANK Ligand , Receptor Activator of Nuclear Factor-kappa B , Receptor, Parathyroid Hormone, Type 1 , Receptors, Calcium-Sensing/genetics , Receptors, Calcium-Sensing/physiology , Receptors, Parathyroid Hormone/physiologyABSTRACT
Neuro-Behçet's disease shows various neuropsychiatric symptoms, but chorea has rarely been reported. We report a case of neuro-Behçet's disease in a 67-year-old woman with depression and chorea that occurred 22 years after the onset of intestinal Behçet's disease. Brain magnetic resonance imaging (MRI) using a fluid-attenuated inversion-recovery (FLAIR) sequence demonstrated lesions more clearly than did T2-weighted MRI. Some of the lesions appeared as small ring-like foci, i.e. low-intensity spots rimmed with remarkable hyperintense signals, in the periventricular white matter and basal ganglia. A review of the literature revealed that the onset of chorea in cases of Behçet's disease varied from the time of onset of Behçet's disease to 31 years after onset of the disease. Psychiatric manifestations have often been associated with neuro-Behçet's disease. In the present patient, treatment with prednisolone resolved the chorea, suggesting that the chorea was caused by an autoimmune mechanism. It seems likely that the long-term development of vasculitis in patients with Behçet's disease results in the formation of these particular brain lesions on FLAIR MR images. Chorea should be taken into consideration as one of the manifestations of Behçet's disease, even many years after remission of the disease.
Subject(s)
Behcet Syndrome/complications , Brain Diseases/complications , Chorea/etiology , Intestinal Diseases/complications , Aged , Behcet Syndrome/drug therapy , Behcet Syndrome/pathology , Brain/blood supply , Brain/pathology , Brain Diseases/drug therapy , Brain Diseases/pathology , Chorea/drug therapy , Chorea/pathology , Female , Glucocorticoids/therapeutic use , Humans , Intestinal Diseases/pathology , Intestines/blood supply , Intestines/pathology , Magnetic Resonance Imaging/methods , Prednisolone/therapeutic use , Remission InductionABSTRACT
Paraneoplastic neurological syndromes are frequently associated in patients with small cell lung cancer (SCLC) and antineuronal antibodies are involved in the autoimmune mechanism. Multiple syndromes are sometimes complicated in a single patient with SCLC. However, little is known about non-SCLC-associated neurological manifestations. We report two patients with complicated paraneoplastic neurological syndromes. Patient 1 showed paraneoplastic limbic encephalitis (PLE), paraneoplastic sensory neuropathy (PSN) and Lambert-Eaton myasthenic syndrome (LEMS) associated with SCLC. Patient 2 developed opsoclonus-ataxia and probable PLE associated with non-SCLC. Analysis of various antineuronal antibodies revealed that anti-Hu and P/Q-type voltage-gated calcium channel (VGCC) antibodies were positive in Patient 1 but any antibodies were not in Patient 2. Brain MRI demonstrated high intensity signals in temporal lobes particularly on fluid-attenuated inversion recovery (FLAIR) or diffusion-weighted images. These findings suggest that complicated paraneoplastic neurological syndromes occur in non-SCLC as well as SCLC and that unidentified antineuronal autoantibodies may underlie the pathophysiology.
Subject(s)
Carcinoma, Non-Small-Cell Lung/diagnosis , Carcinoma, Small Cell/diagnosis , Lambert-Eaton Myasthenic Syndrome/diagnosis , Limbic Encephalitis/diagnosis , Lung Neoplasms , Neuritis/diagnosis , Ocular Motility Disorders/diagnosis , Paraneoplastic Syndromes, Nervous System/diagnosis , Autoantibodies/blood , Calcium Channels, N-Type/immunology , Carcinoma, Non-Small-Cell Lung/immunology , Carcinoma, Small Cell/immunology , Diffusion Magnetic Resonance Imaging , Dominance, Cerebral/physiology , ELAV Proteins , Humans , Image Enhancement , Lambert-Eaton Myasthenic Syndrome/immunology , Limbic Encephalitis/immunology , Magnetic Resonance Imaging , Male , Middle Aged , Nerve Tissue Proteins/immunology , Neuritis/immunology , Neurologic Examination , Ocular Motility Disorders/immunology , Paraneoplastic Syndromes, Nervous System/immunology , RNA-Binding Proteins/immunology , Temporal Lobe/immunology , Temporal Lobe/pathologySubject(s)
Magnetic Resonance Imaging , Methylergonovine/adverse effects , Oxytocics/adverse effects , Puerperal Disorders/chemically induced , Puerperal Disorders/diagnosis , Substance Withdrawal Syndrome/diagnosis , Substance Withdrawal Syndrome/etiology , Vasospasm, Intracranial/chemically induced , Vasospasm, Intracranial/diagnosis , Adult , Electroencephalography , Female , Humans , Pregnancy , Time Factors , Tomography, X-Ray ComputedABSTRACT
Inhalation of organic solvents has long been known to damage various nervous systems, including cerebellum, brainstem, and pyramidal tract. However, little is known about the damage of the dopaminergic system. We report two patients with occupational long-term solvent exposure who developed postural instability without other features of parkinsonism. The concentration of HVA in CSF was decreased and the retropulsion was dramatically improved after the administration of levodopa. These findings indicate that the nigrostriatal dopaminergic neurons were disturbed by chronic solvent exposure, resulting in the loss of postural reflexes.
