Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disease of the central nervous system. The experience in children is limited. We retrospectively reviewed our experience with 20 ADEM patients (10 females, 10 males) with age of onset before 18 years old in Taiwan to clarify the clinical manifestations, neuroimaging findings, and the relationship between ADEM and multiple sclerosis (MS). The age at onset ranged from 4 months to 15 years. Seventeen (85%) children had a recent infectious prodrome. Children presented most often with acute consciousness disturbance (70%) and motor deficits (55%). Seizures occurred in 10 (50%), but only one child developed epilepsy in follow-up. Brain magnetic resonance imaging (MRI) evaluations done in all patients revealed multifocal lesions, mainly in subcortical white matter (80%), brainstem (65%), basal ganglia (55%), cerebellum (45%), thalamus (40%), and periventricular white matter (35%). Spinal cord MRI was performed in 9 patients and all of them showed abnormal lesions. Eleven patients were treated with high-dose intravenous methylprednisolone pulse therapy, and only one had mild long-term neurological sequelae. Among the 20 patients, five had long-term neurological sequelae and one died. Three patients fit the criteria of multiphasic disseminated encephalomyelitis, in which two developed MS in follow-up. Another patient with ADEM turned out to be MS two years later. We concluded that seizures are not uncommon in ADEM, but the subsequent development of epilepsy is rare. Long-term prognosis of ADEM is generally good. Because recurrence of ADEM is not uncommon, long-term follow-up of those children with ADEM is needed to distinguish between ADEM and MS.
 ...
PMID:Acute disseminated encephalomyelitis in children: one medical center experience. 1692 30

It is known that rotavirus gastroenteritis can accompany some neurological manifestations, including encephalitis/encephalopathy or seizures. However, the detailed pathogenesis involved has not been fully understood. To date, acute cerebellitis associated rotavirus gastroenteritis has not been previously reported, except for one case. Herein, we describe two cases of acute encephalitis/encephalopathy and concurrent cerebellitis, associated rotavirus gastroenteritis. Following vomiting and diarrhea, case 1 experienced convulsions and consciousness disturbance and case 2, transient loss of consciousness with eye deviation. After these symptoms subsided, cerebellar signs became evident and a brain MRI showed cerebellar involvement in both cases. Both cases showed speech disturbances, such as mutism, slow speech and dysarthria. In this report, we will discuss the possible pathogenesis of rotavirus associated acute encephalitis/encephalopathy and concurrent cerebellitis.
 ...
PMID:Rotavirus associated acute encephalitis/encephalopathy and concurrent cerebellitis: report of two cases. 1753 86

Reversible posterior encephalopathy syndrome (RPES) is a clinical entity characterized with headache, nausea, vomiting, seizures, consciousness disturbance, and frequently visual disorders associated with neuroradiological findings, predominantly white matter abnormalities of the parieto-occipital lobes. The central nervous system manifestations of systemic lupus erythematosus (SLE) are highly diverse. However, SLE-associated RPES has been seldom reported. Here, we report a case with RPES in SLE and lupus nephritis with exclusive involvement of parietal and occipital cortices. A systematic review of the literature on the pathogenesis and treatment of SLE-associated RPES is included.
 ...
PMID:Reversible posterior encephalopathy syndrome in systemic lupus erythematosus and lupus nephritis. 1845 82

A 66year-old man with sustained fever was diagnosed as having acute myeloid leukemia with multilineage dysplasia. Induction therapy with etoposide and AraC was initiated, but was ineffective. Although fever had persisted for more than a few days, there was no evidence of any infection on radiological examination or culture studies. The patient was disorientated and demonstrated personality change. After a severe convulsive seizure, the patient died. Autopsy findings showed that the leukemic cells had permeated the Virchow Robin space, but without a mass lesion in the cerebral parenchyma. He was diagnosed as having had central nervous system leukemia (CNSL) that provoked sustained fever, consciousness disturbance and convulsive seizure. These findings suggested that the Virchow Robin space plays a particular role in the development of CNSL. Even with repeated cerebrospinal fluid examinations and radiological tests, we were unable to correctly diagnose CNSL before death, which may indicate the intractability of diagnosing CNSL spread along the Virchow Robin space. This case provides useful information about the pathophysiology and diagnosis of CNSL.
 ...
PMID:[Acute myeloid leukemia invasion of the central nervous system, detected only along the Virchow Robin space]. 1857 12

Valproate-induced hyperammonemic encephalopathy is an unusual but serious complication that may occur in people with normal liver-associated enzyme levels, despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl who had absence seizure and complained about progressive dizziness and general malaise several days after restarting valproate. Then, she presented vomiting and decreased consciousness three weeks after valproate use. Notably, her serum ammonia level was five times the upper limit of normal (184 micrommol/L), with normal liver-associated enzyme and supra-therapeutic valproate level. EEG showed continuous generalized slowing. The tandem mass analysis revealed carnitine deficiency. Consciousness improved after emergent hemodialysis. Ammonia level and EEG also returned to normal. Possible mechanisms, risk factors and the treatments of valproate-induced hyperammonemic encephalopathy are described. Physicians should consider this possibility when consciousness disturbance occurs in patients treated with valproate.
 ...
PMID:Valproate-induced hyperammonemic encephalopathy treated by hemodialysis. 1879 59

Valproate-induced hyperammonemic encephalopathy is an unusual but serious complication that can occur in people with normal liver-associated enzyme levels, and despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl suffering from absence seizures, who complained of progressive dizziness and general malaise several days after restarting valproate. She developed vomiting and decreased consciousness after 3 weeks of valproate use. She had a serum ammonia level five times higher than the upper normal limit, normal liver-associated enzymes, and a supra-therapeutic valproate level. Electroencephalography (EEG) showed continuous generalized slowing. Tandem mass spectrometry analysis revealed carnitine deficiency. Her consciousness improved after emergent hemodialysis. Her ammonia level and EEG also became normal. Possible mechanisms, risk factors and treatments of valproate-induced hyperammonemic encephalopathy are described. Physicians should consider this possibility when consciousness disturbance occurs in patients treated with valproate.
 ...
PMID:Valproate-induced hyperammonemic encephalopathy. 1913 74

Two newly proposed infectious encephalitis/encephalopathy syndromes, in which magnetic resonance imaging (MRI) is essential for the diagnosis, have been reviewed. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is reported only in East Asian infants, characterized by a febrile seizure (usually >30 min) as the initial neurological symptom on day 1, followed by secondary seizures at day 4 to 6; affected children display variable levels of neurological sequelae. MRI shows no acute abnormality during the first two days; reduced diffusion appears in the frontal or fronto-parietal subcortical white matter during days 3 to 9, then disappears between days 9 and 25. Excitotoxic injury with delayed neuronal death is hypothesized as a possible mechanism based on MR spectroscopic findings. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is characterized by a reversible lesion with homogeneously reduced diffusion in the corpus callosum (at least involving the splenium), sometimes associated with symmetrical white matter lesions. The most common neurological symptom is delirious behavior, followed by consciousness disturbance, and seizures, all of which completely recover within a month. The reason for the transiently reduced diffusion within the lesions is unknown; possibilities that have been postulated include intramyelinic edema, interstitial edema in tightly packed fibers, and a transient inflammatory infiltrate.
 ...
PMID:Two newly proposed infectious encephalitis/encephalopathy syndromes. 1933 28

Hashimoto's thyroiditis (HT) is the most common disorder affecting the thyroid gland. Encephalopathy associated with abnormal thyroid functions, such as myxedema encephalopathy, is treatable. Hashimoto's encephalopathy (HE) was recognized as a new clinical disease based on an autoimmune mechanism associated with HT, and steroid treatment has been successfully administrated. Recently, we discovered serum autoantibodies against the NH2-terminal of a-enolase (NAE) as a specific diagnostic marker for HE. We analyzed these serum anti-NAE autoantibodies and the clinical features in 84 cases of HE. The 84 patients consisted of 26 men and 58 women, from many institutions throughout Japan and other countries. A total of 37 patients carried anti-NAE antibodies (44%). The age was widely distributed between 19 and 87 years old, with two peaks (around 20-30 and 50-70 years old). Most patients were in euthyroid states, and all patients had anti-thyroid (TG) and/or anti-thyroid peroxidase (TPO) antibodies, and anti-TSH receptor (TSHR) antibodies in some cases. Only 20% of patients had past histories of HT. The acute encephalopathy form was the most common clinical feature, and subacute or chronic psychiatric forms and other forms such as pure ataxia, limbic encephalopathy, and Creutzfeldt Jakob-like forms followed. The patients with anti NAE antibodies tended to exhibit acute encephalopathy. The most common neuropsychiatric features were consciousness disturbance, psychiatric symptoms, and seizures. Involuntary movements (tremor, myoclonus, or choreoathetosis) or ataxia occasionally occurred. Abnormalities, especially the slowing of background activities, on EEG and elevated levels of protein/IgG in cerebrospinal fluid (CSF) were common and useful laboratory findings for the diagnosis, while abnormalities on brain MRI were rare and non-specific in HE. Immunotherapies such as glucocorticoid, immunosuppressants, immunoglobulin, and plasma exchange, were recommended and effective for HE treatment. HE belongs to a part of a clinical spectrum consisting of individuals with anti-thyroid antibodies, overlapping the clinical spectrum of HT. Anti-NAE autoantibodies were positive in 44% of patients with HE. Considering the overall findings, we should be aware of the possibility of autoimmune encephalopathy associated with thyroid disorders (HE) in patients with an unknown etiology of neuronpsychiatric symptoms with/without a past history of HT.
 ...
PMID:[Anti-NAE autoantibodies and clinical spectrum in Hashimoto's encephalopathy]. 1936 98

A 9-year-old boy diagnosed as having Rasmussen syndrome had congenital IgA deficiency and juvenile alopecia. He developed auditory hallucination and consciousness disturbance with intractable complex partial epileptic status. Anti-glutamate receptor epsilon2 antibodies were detected in his serum and cerebrospinal fluid. He was administered immunomodulatory agents and his seizures were treated with an intravenous anticonvulsant for 2 months. Subsequently, he developed a nephrotic syndrome, which proved to be membranous nephropathy and was treated with cyclophosphamide. Anti-basement membrane antibodies were detected in his serum. The boy died at the age of 14 years, and autopsy revealed diffuse brain atrophy with neuronal loss, infiltration of glial cells in the cerebrum, and loss of Purkinje cells in the cerebellum. A kidney specimen contained many sclerotic glomeruli, indicative of progressive membranous nephropathy. The patient was considered to have multimodal autoimmune disorder producing juvenile alopecia, autoimmune encephalitis, and a membranous nephropathy, based on the congenital IgA deficiency.
 ...
PMID:Rasmussen syndrome combined with IgA deficiency and membranous nephropathy. 1943 84

Impaired consciousness has long been considered the hallmark of epileptic seizures. Both generalized seizures and complex partial seizures are characterized by a multifaceted spectrum of altered conscious states, in terms of the general level of awareness and the subjective contents of consciousness. Complete loss of consciousness occurs when epileptic activity involves both cortical and subcortical structures, as in tonic-clonic seizures and absence seizures. Medial temporal lobe discharges can selectively impair experience in complex partial seizures (with affected responsiveness) and certain simple partial seizures (with unaffected responsiveness). Electrical stimulation of temporal lobe structures has been shown to evoke similar subjective experiences. Findings from neurophysiological and brain-imaging studies in epilepsy have now demonstrated that involvement of the bilateral thalamus and upper brainstem leads to selective impairment of frontoparietal association cortices and midline 'default mode' networks, which results in ictal loss of consciousness. The spread of epileptic discharges from the medial temporal lobe to the same subcortical structures can ultimately cause impairment in the level of consciousness in the late ictal and immediate postictal phase of complex partial seizures. This paper reviews novel insights into the brain mechanisms that underlie alterations of consciousness during epileptic seizures and the implications for clinical practice in terms of diagnosis and management.
 ...
PMID:Brain mechanisms of altered conscious states during epileptic seizures. 1948 84


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>