Central Nervous System Involvement in Thrombotic Thrombocytopenic Purpura

Druschky, A.; Erbguth, F.; Strauss, Ronald G.; Helm, Gisela; Heckmann, Jens; Neundörfer, B.

doi:10.1159/000007983

Cited by 6 publications

(3 citation statements)

References 23 publications

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“…Although rare, there are also cases of congenital TTP [11]. Although the involvement of the central nervous system (CNS) is more frequent in TTP [1,12], in which it is detected in 63% to 87% of the cases [13][14][15][16][17], up to 20% of HUS patients also present with some degree of neurological manifestations [18].…”

Section: Introductionmentioning

confidence: 99%

Neurological manifestations in thrombotic microangiopathy: Imaging features, risk factors and clinical course

et al. 2022

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Background and purpose Thrombotic microangiopathy (TMA) is a group of microvascular occlusive disorders that presents with neurological involvement in up to 87% of the cases. Although the central nervous system (CNS) is an important target organ in TMA, the role of neurological manifestations in the disease clinical course is not well established. In this study, we described the neurological manifestations and CNS radiological aspects in patients with a first, acute TMA event. We also examined the association between severe neurological involvement and adverse clinical outcomes in TMA. Methods A cohort of patients diagnosed with a first TMA event between 1995 and 2016 was included, their medical charts and imaging tests were retrospectively evaluated. Results A total of 49 patients were included, 85.7% were women and the mean age was 36.5 years-old (SD 13.0). Neurological manifestations were described in 85.7% of the patients, most of them (88%) were considered severe and consisted of confusion, compromised sensorimotor function, stupor, seizures, and personality change. Imaging tests were performed in 62% of the patients with neurological manifestations and detected acute CNS lesions, such as posterior reversible encephalopathy syndrome, hemorrhagic and ischemic stroke were observed, in 7 (27%) of them. While the need for intensive care unit admission was greater and longer among patients with severe neurological manifestations, the number of plasma exchange sessions, the total duration of hospitalization and in-hospital death were similar between groups. Conclusions Severe neurological manifestations are common in first TMA events and are responsible for a worse disease presentation at admission. While the effect of neurological manifestations on acute TMA clinical course seems to be modest, these manifestations may have an important impact on the development of chronic cognitive impairment, which highlights the need for proper diagnosis and treatment.

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Section: Introductionmentioning

confidence: 99%

Neurological manifestations in thrombotic microangiopathy: Imaging features, risk factors and clinical course

et al. 2022

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“…Thrombotic thrombocytopenic purpura (TTP) classically presents with a triad of thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and fluctuating neurological symptoms [1]. Therapeutic plasma exchange (TPE) has reduced mortality of TTP from 90% to about 20% [2].…”

Section: Introductionmentioning

confidence: 99%

Relapsed thrombotic thrombocytopenic purpura presenting as an acute cerebrovascular accident

Downes

Yomtovían

Tsai

et al. 2004

J of Clinical Apheresis

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Thrombotic thrombocytopenic purpura (TTP) is an uncommon but severe disorder that classically presents with microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and fluctuating neurological changes. Previously, it was impossible to make a diagnosis of TTP in the absence of thrombocytopenia or microangiopathic hemolysis (MAHA). We describe two cases of relapsing TTP that presented with acute cerebrovascular accident (CVA) without concurrent thrombocytopenia or MAHA after initial classical presentation of TTP. In both cases, the diagnosis of TTP as the cause of the CVA was attributed to severe deficiency of the von Willebrand factor cleaving protease, ADAMTS13 in plasma (11 and 12%, normal 79-127%). Each patient had a dramatic clinical improvement in response to therapeutic plasma exchange. The experience in these two cases suggests that TTP should be considered as a potential cause among patients presenting with a CVA, particularly if the patients have a history of TTP.

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“…• genetisch determinierte Systemerkrankungen wie MELAS-Syndrom und CADASIL [13,80]. Neben den genannten einzelnen Ursachen zeigt der klinische Alltag oft auch Kombinationen verschiedener Ursachen, oder aber es liegen äußerst seltene Ischämieursachen zugrunde, wie zum Beispiel die Marcumar ® -Nekrose des Gehirns, die zerebrale Luftembolie, die thrombotisch-thrombozytopenische Purpura oder das dissezierende Aortenaneurysma [14,15,32,45]. Trotz detaillierter ergänzender apparativer Diagnostik läßt sich in ca.…”

Section: Klinik Und Pathogenese Der Zerebralen Ischämieunclassified

Die Hirndurchblutung aus klinischer Sicht Historischer Überblick, Physiologie, Pathophysiologie, diagnostische und therapeutische Aspekte

Heckmann¹,

Erbguth²,

Hilz³

et al. 2001

Med Klin

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Strokes have been known since ancient times. Today, stroke is the second most frequent cause of death and the most frequent cause of invalidity. In about 80% of cases, stroke is caused by cerebral ischemia and in about 20% by intracerebral hemorrhage, subarachnoidal hemorrhage, venous thrombosis and other cerebrovascular diseases. The brain is one of the most richly perfused tissues and depends fundamentally on the supply of oxygen and glucose. In order to assure adequate cerebral blood flow, the brain is capable of autoregulation through the interaction of diverse autoregulatory mechanisms (myogenic, neurogenic and metabolic factors, blood viscosity, renin-angiotensin-system and endothelium). Reduction of cerebral blood flow below the threshold of about 25 ml/100 g x min leads to an impairment of the functional metabolism and later to impairment of the structural metabolism. Pathophysiologically, a large number of isolated pathobiochemical processes (loss of energy, lactate acidosis, excitating amino acid release, ion balance disorders, calcium overload, free radical release, etc.) start to interfere with each other. Delayed edema and inflammation lead to secondary brain damage. Apoptosis is probably induced by ischemia and can cause secondary deterioration. The basic principles in the treatment of ischemia are firstly the rapid restoration of cerebral blood flow (lysis, carotid endarterectomy) and secondly--following infarction--a limitation of brain damage (preservation of ischemic but not necrotic brain tissue, prevention of secondary complications). Stroke treatment requires profound diagnostic and therapeutic expertise and interdisciplinary cooperation of neuroradiologists, neurosurgeons, vascular surgeons and cardiologists. Stroke can best be managed in special "stroke units", which have now been established in nearly all parts of Germany. Beside acute management of stroke and neurological rehabilitative treatment, emphasis has to be laid on primary (public information, education, treatment of risk factors) and secondary prophylaxis (treatment with antiaggregants, anticoagulants, a. o.).

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Central Nervous System Involvement in Thrombotic Thrombocytopenic Purpura

Cited by 6 publications

References 23 publications

Neurological manifestations in thrombotic microangiopathy: Imaging features, risk factors and clinical course

Neurological manifestations in thrombotic microangiopathy: Imaging features, risk factors and clinical course

Relapsed thrombotic thrombocytopenic purpura presenting as an acute cerebrovascular accident

Die Hirndurchblutung aus klinischer Sicht Historischer Überblick, Physiologie, Pathophysiologie, diagnostische und therapeutische Aspekte

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