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Cerebral Thrombotic Thrombocytopenic Purpura


Presentation

  • However, large vessel infarctions with long standing deficits remain a rare presentation of TTP. This case adds to the sparse literature of ischemic stroke as an acute presentation of TTP.[shmabstracts.com]
  • Approximately half of the patients at presentation and eventually 90% of them demonstrated neurological involvement [ 3 ].[academic.oup.com]
  • Found At: Wiley Version: Author's manuscript Abstract: Case Presentation A 54 year‐old man with hemoglobin SC disease (HbSC) and a history of substance abuse presented to the Emergency Department from a nursing home with two days of progressive weakness[scholarworks.iupui.edu]
  • A 36-year-old male, who has diabetes and had recent left middle cerebral artery (MCA) infarction, presented to the emergency room with recurrent generalize tonic clonic seizer.[scopemed.org]
Fever
  • He developed a fever, hypoxia, and tachycardia on the day of admission. He did not have any recent changes in medications, and his family as well as the nursing home staff denied any access to illicit drugs.[scholarworks.iupui.edu]
  • —We recently admitted a 27-year-old woman because of recurrent attacks of right-sided central facial palsy (five in 36 hours), accompanied by slight fever. When she was not experiencing the attacks, her neurologic examination was normal.[jamanetwork.com]
  • TTP’s classic pentad includes thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal failure and fever.[shmabstracts.com]
  • INTRODUCTION Thrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by a classic pentad—thrombocytopenia, haemolytic anaemia, neurological involvement, fever and renal impairment.[academic.oup.com]
Anemia
  • Investigations revealed evidence of thrombocytopenia, microangiopathic hemolytic anemia and acute kidney injury. MRV brain showed cerebral venus sinus thrombosis.[scopemed.org]
  • Blood parameters revealed severe hemolytic anemia (hemoglobin concentration, 57 g/L [5.7 g/dL], corrected reticulocyte count, 0.15 [15%]), severe thrombocytopenia (platelet count, 20 X 10 6 /L [20 000/mm 3 ]), and mild disseminated intravascular coagulation[jamanetwork.com]
  • TTP’s classic pentad includes thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal failure and fever.[shmabstracts.com]
  • Loose strands of platelets and fibrin are deposited in multiple small vessels and damage passing platelets and RBCs, causing significant thrombocytopenia and anemia (microangiopathic hemolytic anemia).[merckmanuals.com]
Gangrene
  • Her clinical condition was further complicated by gastrointestinal bleeding caused by stress ulcer and gangrenous change of the toes accountable by the thrombotic phenomenon of TTP.[academic.oup.com]
  • […] nonthrombocytopenic purpura seen mainly in children, usually after an infectious disease, marked by fever, shock, anemia, and sudden, rapidly spreading symmetrical skin hemorrhages of the lower limbs, often associated with extensive intravascular thromboses and gangrene[medical-dictionary.thefreedictionary.com]
Bleeding Gums
  • In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums.[medical-dictionary.thefreedictionary.com]
  • He was diagnosed to have ITP 1-month back when he presented with petechial spots, bleeding gums, and hematuria. Bone marrow examination had shown megakaryocyte hyperplasia consistent with ITP.[ijccm.org]
Upper Limb Weakness
  • She developed left upper limb weakness on Day 3 of admission. Serial CT brain revealed a small subarachnoid haemorrhage in the cerebral falx but no acute infarct. Follow-up CT scan was unremarkable.[academic.oup.com]
Purpura
  • […] posterior leukoencephalopathy syndrome in thrombotic thrombocytopenic purpura , Intern Med , 2008 , vol. 47 (pg. 1931 - 4 ) 11 Thrombotic thrombocytopenic purpura―then and now , Semin Thromb Hemost , 2006 , vol. 32 (pg. 81 - 9 ) The Author 2015.[academic.oup.com]
  • Key words: Thrombotic thrombocytopenic purpura, generalized tonic clonic seizer, cerebral venus sinus thrombosis[scopemed.org]
  • KEYWORDS: Cerebral microbleeds; MRI; ischemic stroke; thrombotic thrombocytopenic purpura[ncbi.nlm.nih.gov]
  • DIAGNOSTIC NEURORADIOLOGY 126 Downloads 5 Citations Abstract Abnormalities in the brain of patients with thrombotic thrombocytopenic purpura (TTP) are infrequent on MRI, often reversible and mainly limited to symptomatic stages of the disease.[link.springer.com]
Hirsutism
  • Case Presentation: 67 year old right-handed female with past medical history of morbid obesity, hirsutism and pre diabetes mellitus presented to the Emergency Department with a chief complaint of acute onset of slurred speech.[shmabstracts.com]
Epistaxis
  • […] and kidneys important underlying risk factor, but requires a secondary trigger triggers drugs (quinine, ticlopidine, clopidogrel, and cyclosporine) SLE infections AIDS malignancies Presentation Symptoms (recall pentad) thrombocytopenia easy bleeding epistaxis[medbullets.com]
  • Moschcowitz's pentad: Thrombocytopenia, splenomegaly, DIC, fever, neurologic signs–eg, headache, confusion, aphasia, transient paresis, ataxia, sensory defects and coma, as well as pallor, jaundice, fibrin thrombi in renal vessels, heart, liver and spleen, epistaxis[medical-dictionary.thefreedictionary.com]
  • Headache and epistaxis were the most common adverse events (Table S6 in the Supplementary Appendix ). Most adverse events were considered to be unrelated to the study drug or unlikely to be related to the study drug.[nejm.org]
Confusion
  • She was admitted to her local hospital with a 3 day history of drowsiness, confusion, epistaxes, and spontaneous bruising, having been noted to be increasingly agitated and disoriented over the preceding 6 weeks.[jnnp.bmj.com]
  • An EEG is particularly indicated for patients with persistent or recurrent confusion and stupor, especially after a clinically overt seizure.[epilepsy.com]
  • TTP SYMPTOMS Initial symptoms of TTP are often confused with a stroke.[youhavealawyer.com]
  • Critical Essential Core Tested Community Questions (4) (M2.HE.102) A 45-year-old man is brought to the emergency room by his wife because he has been acting confused for the past day.[medbullets.com]
  • If they are forming in the brain, they can result in neurological changes: personality changes, headaches, confusion, and slurred speech. If they form in the heart, cardiovascular problems can occur, such as an abnormal heart rhythm.[schmidtlaw.com]
Neurologic Manifestation
  • This intensive handbook is meant to give clinicians a source reference that will enable them to gain a thorough knowledge and understanding of the clinical features and management of the many neurological manifestations of stroke disorder.[books.google.it]
  • It is estimated that 50% of patients with TTP present with neurologic manifestations that include seizures and fluctuating neurologic deficits from small vessel infarctions.[shmabstracts.com]
  • Abstract We describe 5 cases of thrombotic thrombocytopenic purpura (TTP) with neurological manifestations.[link.springer.com]
  • Other neurologic manifestations can include headache, cranial nerve palsies, dysphasia or aphasia, paresis, confusion, stupor, coma, and seizures.[epilepsy.com]
Altered Mental Status
  • 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).[journals.lww.com]
  • The pentad of clinical findings consists of : Fever Neurological signs and symptoms Altered mental status ;, delirium Seizure, focal defects, stroke Headache, dizziness Low platelet count (i.e. thrombocytopenia ) Petechiae, purpura Mucosal bleeding Prolonged[amboss.com]
  • Neurologic features include altered mental status, seizures, coma, or focal neurologic deficit.[rheumaknowledgy.com]
  • These patients may present clinically with altered mental status, seizure, or neurologic deficits.[ajnr.org]
  • Altered mental status in thrombotic thrombocytopenic purpura is secondary to nonconvulsive status epilepticus . Ann Neurol , 40 , 245 –6. George , J. N. 2000 . How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome .[cambridge.org]
Dysarthria
  • Physical exam was notable for vitals within normal limits, partial gaze palsy, asymmetric smile, left arm motor drift, moderate dysarthria and profound right-sided neglect (National Institute of Health Stroke Scale Score of 7).[shmabstracts.com]
  • E levels Neurologic dysfunction 60% have at presentation; 90% have at some time in illness Headache, change in mental status coma, paresthesias, paresis, aphasia, dysarthria, syncope, vertigo, ataxia, cranial nerve palsies, seizures, & CVA have been reported[faculty.washington.edu]
  • He had an episode of generalized tonic clonic seizure, after which he developed right-sided hemiparesis and dysarthria.[actamedicaphilippina.com.ph]
  • Clinical examination on admission revealed a BP of 110/80 mmHg (normal range, 80–120/60-80 mmHg) and dysarthria; cognitive function, including problem solving and memory, was impaired, MMSE 17 points (university education level, 23 points indicative of[spandidos-publications.com]
Slurred Speech
  • Case Presentation: 67 year old right-handed female with past medical history of morbid obesity, hirsutism and pre diabetes mellitus presented to the Emergency Department with a chief complaint of acute onset of slurred speech.[shmabstracts.com]
  • A 58-year-old Chinese woman presented with slurred speech and multiple petechiae over lower limbs. Blood tests showed thrombocytopenia.[academic.oup.com]
  • If they are forming in the brain, they can result in neurological changes: personality changes, headaches, confusion, and slurred speech. If they form in the heart, cardiovascular problems can occur, such as an abnormal heart rhythm.[schmidtlaw.com]
  • She also referred left eye pain and slurred speech for 1 day. Her past medical history was remarkable for essential systemic hypertension, diabetes mellitus, and osteoarthritis.[thejh.org]
Oliguria
  • Headache, dizziness Low platelet count (i.e. thrombocytopenia ) Petechiae, purpura Mucosal bleeding Prolonged bleeding after minor cuts Microangiopathic hemolytic anemia Fatigue, dyspnea ;, and pallor Jaundice Impaired renal function Hematuria, proteinuria Oliguria[amboss.com]
  • (also called verocytotoxin because it damages Vero cells in culture) Main cause of childhood acute renal failure Usually infants and young children, may occur at any age Symptoms : sudden onset of influenza, bleeding (hematemesis or melena), severe oliguria[pathologyoutlines.com]
  • Treatment of D HUS Correction with isotonic saline or ringer lactate to prevent oliguria on the first four days, as well as the correction of electrolytic abnormalities by use of dialysis seem to play a relevant role in survival in cases of D HUS in the[jbn.org.br]

Workup

  • Septic workup was unrevealing. Bone marrow aspirate demonstrated reactive changes without malignant infiltrate. She developed left upper limb weakness on Day 3 of admission.[academic.oup.com]
Decreased Platelet Count
  • Subsequent CBCs revealed persistently decreasing platelet counts, initially posing a dilemma in the further work up for sagittal sinus thrombosis, which involves a 4-vessel angiogram, a more invasive procedure that poses greater risks for bleeding.[actamedicaphilippina.com.ph]
  • Decreased platelet count would support thrombotic thrombocytopenic purpura; leukocytosis might be seen in sepsis. (If heparin is used as treatment, platelet counts should be monitored for thrombocytopenia.)[emedicine.medscape.com]

Treatment

  • Four cycles of plasma exchange were commenced but she remained unresponsive to the treatment. Trans-thoracic echocardiogram revealed no vegetations.[academic.oup.com]
  • Treatment Treatment should be started after a presumptive diagnosis is made based on clinical features and initial labs (e.g., blood count, peripheral smear, creatinine ). TTP requires urgent diagnosis and treatment![amboss.com]
  • ADAMTS13 activity could potentially guide decisions about the duration of caplacizumab treatment, in addition to guiding immunosuppressive treatment.[nejm.org]
  • A study by Bukowski et al ( 10 ) first reported the successful treatment of TTP with PE in 1976, and PE has now become the first choice treatment for TTP.[spandidos-publications.com]

Prognosis

  • Important features include information on neurologic complications in organ transplant recipients, muscle and nerve injury in critical illness, status epilepticus, cerebral edema, multisystem trauma including cervical spine and traumatic brain injury, and prognosis[books.google.com]
  • Prognosis The prognosis depends primarily on prompt initiation of treatment. Timely treatment can prevent acute complications ( AKI, coma, and death), as well as progression to chronic renal failure.[amboss.com]
  • , Prevention, and Complications Prognosis remission with plasma exchange in majority of patients mortality rate 13-15% Complications permanent neurologic sequelae chronic kidney disease especially with quinine-induced TTP Please rate topic.[medbullets.com]
  • This type of treatment has greatly improved the prognosis for people who have Thrombotic Thrombocytopenic Purpura. Most people recover completely.[schmidtlaw.com]
  • Both patients were reviewed by neurological and haematological experts, who considered that the prognosis was poor.[jnnp.bmj.com]

Etiology

  • TTP-associated thrombotic microangiopathy resulting in diffuse microthrombi formation in the microcirculation and hypoperfusion might be an underlying etiology. Copyright 2016 National Stroke Association. Published by Elsevier Inc.[ncbi.nlm.nih.gov]
  • However, they have distinct etiologies, with TTP being caused by a deficiency of ADAMTS13.[amboss.com]
  • Etiology Normally a protease known as A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13 (ADAMTS13) is responsible for cleaving von Willebrand multimers and preventing the aggregation of von Willebrand factor and subsequent[radiopaedia.org]

Epidemiology

  • ]]) Epidemiology : may occur Diarrhea (see Diarrhea, [[Diarrhea]]) Epidemiology : common Note: the timing of diarrhea is crucial, as diarrhea that precedes the onset of symptoms by several days may instead be indicative of Shiga toxin-producing Escherichia[mdnxs.com]
  • Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified. Etiology Pathophysiology TTP is a thrombotic microangiopathy, a condition in which microthrombi form and occlude the microvasculature ;.[amboss.com]
  • Introduction Thrombotic microangiopathy characterized by a pentad microangiopathic hemolytic anemia acute renal failure thrombocytopenia fever neurologic abnormalities Similar to HUS but with fever and neurologic symptoms Epidemiology female male most[medbullets.com]
  • Epidemiology and clinical presentation The peak incidence of TTP occurs in persons in their thirties and occurs twice as often in women as in men.[epilepsy.com]
  • Outbreak of Escherichia coli O157:H7 diarrheal illness in a nursing home: clinical, epidemiologic and pathologic findings. J Infect Dis (in press). Wells JG, Davis BR, Wachsmuth IK, et al.[cdc.gov]
Sex distribution
Age distribution

Pathophysiology

  • . * A comprehensive source that thoroughly examines the neurological manifestations of stroke * A guide to highly studied topics, including stroke complications, syndromes, and stroke related psychiatric disorders * A better understanding of the pathophysiological[books.google.it]
  • […] second edition includes entirely new chapters on: *Evaluation of coma focused on structural causes *Neurologic complications in the critically ill pregnant patient *Ethical issues and practical problems with withdrawl of life support New material on: *Pathophysiological[books.google.com]
  • The two conditions have some pathophysiological similarities and share some clinical findings. However, they have distinct etiologies, with TTP being caused by a deficiency of ADAMTS13.[amboss.com]
  • There is possible parallel and/or overlap in the pathophysiology of TMA and PRES.[ajnr.org]
  • In the forthcoming years, new drugs stemming from a better understanding of TTP pathophysiology should advantageously complete the therapeutic arsenal.[bloodjournal.org]

Prevention

  • […] manifestations of stroke * A guide to highly studied topics, including stroke complications, syndromes, and stroke related psychiatric disorders * A better understanding of the pathophysiological basis of strokes that will help practitioners better diagnose, prevent[books.google.it]
  • References: [9] [10] Prevention Tertiary prevention following TTP Follow-up monitoring of platelet count and LDH Initiation of PEX if either indicates a relapse of TTP[amboss.com]
  • Plavix received FDA approval in 2007 for the prevention of heart attacks, strokes, and other ailments caused by blood clots.[schmidtlaw.com]
  • If recurrent disease is to be prevented, clinicians must recognize quinine as a possible cause. Ann Intern Med. Dec. 18, 2001;135:1047-1051. ( Full text ) REVIEW: Thrombotic Microangiopathies (TTP & Hemolytic-Uremic Synd.)[enotes.tripod.com]
  • Overall prevention of bleeding is the highest priority. Prompt medical care can help your child live a long healthy life. For More on ITP – Click here[healthlifemedia.com]

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