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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thrombotic thrombocytopenic purpura (TTP) is a syndrome with numerous neurological manifestations including altered mental status and seizures. At least 10% of the patients with TTP seen at our institution had nonconvulsive status epilepticus as a cause of or associated with their altered mental status. We propose that altered mental status secondary to nonconvulsive status epilepticus requiring electroencephalographic diagnosis and antiepileptic medication occurs in a substantial proportion of patients with TTP.
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PMID:Altered mental status in thrombotic thrombocytopenic purpura is secondary to nonconvulsive status epilepticus. 877 8

Cerebral involvement is typical for thrombotic microangiopathies like haemolytic uremic syndrome (HUS) and thrombotic-thrombopenic purpura (Moschcowitz disease or TTP). Symptoms are irritation, restless behaviour, disorientation, disturbance of consciousness, seizures, and focal neurological deficits. The lack of typical imaging changes or pathological observations may explain the unknown pathophysiological cascade leading to the neurological symptoms. We describe the development of HUS/ TTP in a 52-year-old woman after acute pneumonia caused by Diplococcus pneumoniae. The patient showed an increasing psycho-organic syndrome with disorientation, followed by severe loss of consciousness and coma. Initially, computed tomography showed slight diffuse brain oedema, which was not found in later follow-up images. Magnetic resonance imaging was normal. The TCD examination revealed general velocity increases and vasospasms (especially MCA, ACA and PCA bilateral and BA). The reduction in blood flow velocities in the basal arteries was accompanied by a marked clinical improvement. The development of vasospasms may be an explanation for the neurological deficits in HUS/TTP. The origin of the vasospasms may be found in disturbed prostacyclin production, increased serotonin or platelet factor IV release, and leucocyte activation with consecutive endothelial damage.
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PMID:[Involvement of the central nervous system in hemolytic uremic syndrome/thrombotic thrombocytopenic purpura]. 903 62

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is more common in women, and commonly occurs during pregnancy and the immediate postpartum period. An important clinical issue is the distinction of TTP-HUS from the more common obstetric complications, preeclampsia and HELLP syndrome (hemolysis, elevated liver function tests, low platelets). Clinical suspicion of TTP-HUS requires urgent intervention with plasma exchange treatment, a procedure with substantial risk, while preeclampsia and HELLP syndrome typically resolve spontaneously following delivery. Since clinical features of these syndromes can be similar, especially if preeclampsia becomes severe or if seizures (defining eclampsia) occur, the differential diagnosis may be arbitrary. This review addresses the evaluation and management of these syndromes and describes a clinical approach for determining when plasma exchange is appropriate.
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PMID:Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy. 1183 17

Classical late-infantile neuronal ceroid lipofuscinosis (LINCL) is caused by mutations in tripeptidyl peptidase I (TPP-I), a pepstatin-insensitive lysosomal protease, resulting in neurodegeneration, acute seizures, visual and motor dysfunction. In vitro studies suggest that TPP-I is secreted from cells and subsequently taken up by neighboring cells, similar to other lysosomal enzymes. As such, TPP-I is an attractive candidate for enzyme replacement or gene therapy. In the present studies, we examined the feasibility of gene transfer into mouse brain using recombinant adenovirus (Ad), feline immunodeficiency virus (FIV) and adeno-associated virus (AAV) vectors expressing TPP-I, after single injections into the striatum or cerebellum. A dual TPP-I- and beta-galactosidase-expressing adenovirus vector (AdTTP-I/nlsbetagal) was used to distinguish transduced (beta-galactosidase positive) cells from cells that endocytosed secreted TTP-I. Ten days after striatal injection of AdTTP-I/nlsbetagal, beta-galactosidase-positive cells were concentrated around the injection site, corpus callosum, ependyma and choroid plexus. In cerebellar injections, beta-galactosidase expression was confined to the region of injection and in isolated neurons of the brainstem. Immunohistochemistry for TPP-I expression showed that TPP-I extended beyond areas of beta-galactosidase activity. Immunohistochemistry for TTP-I after FIVTTP-I and AAV5TTP-I injections demonstrated TPP-I in neurons of the striatum, hippocampus and Purkinje cells. For all three vectors, TPP-I activity in brain homogenates was 3-7-fold higher than endogenous levels in the injected hemispheres. Our results indicate the feasibility of vector-mediated gene transfer of TPP-I to the CNS as a potential therapy for LINCL.
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PMID:Viral-mediated delivery of the late-infantile neuronal ceroid lipofuscinosis gene, TPP-I to the mouse central nervous system. 1252 35

Thrombotic thrombocytopenic purpura (TTP) is a well-described entity in adults but is rarely observed in children. The authors describe a series of seven children with suspected acquired TTP. Clinical findings included petechiae, purpura, or jaundice ( 6), central nervous system events ( 5), fever ( 3), diarrhea ( 3), renal insufficiency ( 2), and hematuria ( 2). Significant central nervous system events included cerebral vascular accidents ( 2), altered mental status ( 2), seizures ( 1), and hemiparesis ( 1). Patients were treated with daily plasma infusions (1/7) or plasma exchange (5/7). Response was prompt, although relapses were frequent. Decreased vWF-protease activity was found in four of five cases and vWF-protease inhibitors were found in three of five cases. Although rare, TTP is a life-threatening illness that does occur in children and should be considered in the differential diagnosis of thrombocytopenia with hemolytic anemia.
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PMID:Case series of thrombotic thrombocytopenic purpura in children and adolescents. 1267 53

Thrombotic thrombocytopenic purpura (TTP) is an ischemic vasculopathy frequently associated with neurological dysfunction including seizures. However, status epilepticus (SE) has rarely been reported in this condition. We report on a 70-year-old woman with fulminant TTP who developed convulsive SE despite high therapeutic serum levels of phenytoin and phenobarbital. Her electroencephalogram (EEG) was characterized by bilateral independent periodic lateralizing epileptiform discharges (BIPLEDs) propagating into clinical and electrographic seizures. She recovered completely after intensive plasmapheresis and treatment with pentobarbital induced coma for 5 days. This case illustrates that aggressive treatment with pentobarbital and plasmapheresis may prevent permanent neurologic deficits when TTP is complicated by SE and that periodic lateralizing epileptiform discharges (PLEDs) in this syndrome can be the manifestation of a reversible ischemic insult.
Seizure 2004 Dec
PMID:Full neurologic recovery after fulminant thrombotic thrombocytopenic purpura with status epilepticus. 1551 14

Thrombotic thrombocytopenic purpura (TTP) is a rare and often fatal disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, mental status changes, and renal dysfunction. Central diabetes insipidus (CDI) is a rare disease due to anatomic lesions of the hypothalamicpituitary system associated with various underlying diseases, or trauma. We present an unusual case of TTP and CDI in a 47 year-old African American female who was admitted to our hospital with crampy abdominal pain and nausea. The patient had tachycardia, fever and hypotension. The patient subsequently became confused, developed seizures, and her renal function deteriorated. Bone marrow analysis showed adequate megakaryocytes while a peripheral smear revealed severe thrombocytopenia, polychromasia and schistocytes. The diagnosis of thrombotic thrombocytopenic purpura (TTP) was made and plasmapharesis initiated. Over the next few days, the patient developed severe polyuria with a rise in serum sodium. Central diabetes insipidus was diagnosed and DDAVP (desmopressin acetate, 1-deamino-8-D-arginine vasopressin) was given. However, DDAVP was stopped several times due to worsening thrombocytopenia. Renal function worsened and the patient expired. A review of the literature revealed only one case of report of TTP and central diabetes insipidus. Our case was the only one reporting the use of DDAVP in such a setting.
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PMID:Unique case of thrombotic thrombocytopenic purpura and diabetes insipidus. 1641 83

A postpartum mare and foal were presented for evaluation of fever and lethargy in the mare. The mare was diagnosed with endometritis and initially responded well to treatment. On the second day of hospitalization, the mare developed renal insufficiency characterized by oliguria, azotemia, hemolysis, and thrombocytopenia. Concurrently, the foal developed rapidly progressive central nervous system signs culminating in refractory seizures. Both animals failed to respond to treatment and were euthanized. Thrombotic microangiopathy involving glomeruli was evident on microscopic examination of the mare's kidneys. Microscopic evidence of brain edema was the principal postmortem finding in the foal. No specific etiology was confirmed in either case. Notably, Escherichia coli 0103:H2 was isolated from the mare's uterus and the gastrointestinal tracts of both animals. To the authors' knowledge, this is the first report in which an organism implicated as a cause of hemolytic-uremic syndrome was isolated from an animal with clinical signs and postmortem findings consistent with the disease.
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PMID:Hemolytic-uremic syndrome in a postpartum mare concurrent with encephalopathy in the neonatal foal. 1831 42

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy leading to microvascular occlusion resulting in ischemic dysfunction of various organs. Pregnancy has been thought to precipitate it during second and third trimester, but its reports in first trimester are extremely rare. We report the clinical protocol of a young lady with 5-week period of gestation, who presented with fever, seizures and altered sensorium, and succumbed to her illness during an episode of seizure. Complete autopsy findings are also presented in detail.
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PMID:Thrombotic thrombocytopenic purpura in a patient with very early pregnancy: clinical presentation and autopsy findings. 1883 19

Thrombotic microangiopathy (TMA) is a rarely reported complication of acute pancreatitis. The prognosis is generally good, if diagnosis is made early and treatment is adequate. We present the case of a 74-year-old man who visited our emergency department due to acute abdominal pain. He had no history of alcohol abuse or pancreatitis. Blood tests indicated elevated lipase and amylase. An abdominal computerized tomography (CT) indicated mild pancreatitis. After admission, the patient suffered a seizure and developed anemia, thrombocytopenia, elevated lactic dehydrogenase (LDH) and elevated unconjugated bilirubin. A peripheral blood smear indicated fragmented red blood cells. We diagnosed the patient as having TMA. After plasma exchange and plasma infusion therapy, the LDH and platelet levels gradually improved. A differential diagnosis of disseminated intravascular coagulation (DIC) and TMA following pancreatitis is necessary because of the different treatment strategies. Our patient had a good prognosis following therapy for TMA. Such therapy may include plasma exchange, plasma infusion, corticosteroid therapy and splenectomy.
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PMID:Acute pancreatitis preceding an acute episode of thrombotic microangiopathy. 2017 26


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