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Acute Hemorrhagic Leukoencephalitis

Ear Patella Short Stature Syndrome


Presentation

  • The patient presented with mild, then rapidly worsening, brainstem impairment to a locked-in syndrome.[ncbi.nlm.nih.gov]
  • Results: The patient presented with mild, then rapidly worsening, brainstem impairment to a locked-in syndrome.[mayoclinic.pure.elsevier.com]
Unconscious State
  • She was referred in an unconscious state. On examination, she was febrile 101 F blood pressure 110/70 mm Hg. She had purulent discharge from the suture wound. She was comatose with small size pupils reacting to light and normal doll's eye movements.[jdrntruhs.org]
Vertical Gaze Palsy
  • On the initial neurological examination, the patient was drowsy but still arousable and showed a vertical gaze palsy. During the following 2 days, he developed a left-sided hemiparesis, and became stuporous.[thieme-connect.com]
Headache
  • A 37-year-old female physician was admitted to the hospital with severe headache, facial and hand paresthesias, dysarthria, and ataxia. Neurologic examination disclosed signs of brain stem dysfunction.[ncbi.nlm.nih.gov]
  • Almost always preceded by a respiratory infection, characterized by the abrupt onset of fever, headache, confusion, and nuchal rigidity, soon followed by focal seizures, hemiplegia, or quadriplegia, brainstem findings, and coma; the CSF shows evidence[medical-dictionary.thefreedictionary.com]
  • Symptoms usually come on quickly, beginning with symptoms such as fever, neck stiffness, fatigue, headache, nausea vomiting, seizures, and coma.[diseaseinfosearch.org]
  • Symptoms usually come on quickly and may include fever, neck stiffness, fatigue, headache, nausea, vomiting, seizures, and coma.[rarediseases.info.nih.gov]
Stupor
  • During the following 2 days, he developed a left-sided hemiparesis, and became stuporous.[thieme-connect.com]
  • Clinical presentation is characterized by the abrupt onset of fever and headache, followed by a rapid progression to stupor or coma. The CSF pressure is typically elevated and analysis reveals numerous neutrophils.[path.upmc.edu]
  • Most patients with ADEM will recover but AHLE progresses rapidly from confusion to stupor to coma; death usually occurs an average of six days after the onset of symptoms.[ispub.com]
  • […] encephalopathy among patients in intensive care units. [1] Encephalopathy is a diffuse cerebral dysfunction characterized by altered consciousness an acute onset of an impairment of cognitive function, which can vary from inattention, disorientation, agitation, stupor[jdrntruhs.org]
  • The three cases died due to; myalgia, jaundice, conjunctival hemorrhage, hematuria, oliguria, shortness of breath, became stuporous, acute respiratory distress syndrome (ARDS), acute kidney injury and metabolic acidosis [ 55 ], intracranial tension [[journals.plos.org]
Altered Mental Status
  • . • Acute disseminated encephalomyelitis • Presents with – Altered mental status/encephalopathy (irritability to obtunded) – Acute/subacute onset of focal symptoms based on lesions (max neuro symptoms over 4-7 days) – Typically still during febrile illness[slideshare.net]
  • Thus, it results in an altered mental status and focal neurologic findings in ADEM patient such as paralysis [ 3 ]. Although ADEM causes a significant impact on dengue patients, data about this complication is still lacking.[journals.plos.org]
Decerebrate Posturing
  • After the second lumbar puncture, the patient was reported to have bilateral decerebrate posturing, and a repeated cranial CT scan showed transtentorial herniation, the eventual cause of death.[nejm.org]

Workup

  • Extensive clinical workup of two patients presenting with AHLE revealed complement abnormalities, specifically related to the alternative pathway and its regulator, FI.[ncbi.nlm.nih.gov]
  • Presents with – Altered mental status/encephalopathy (irritability to obtunded) – Acute/subacute onset of focal symptoms based on lesions (max neuro symptoms over 4-7 days) – Typically still during febrile illness (typically URI) – 1/3 with seizures • Workup[slideshare.net]
Cerebrospinal Fluid Abnormality
  • The child was treated with high dose intravenous steroids and made a full recovery, with a parallel disappearance of all of her cerebrospinal fluid abnormalities and almost all of her abnormalities on MRI.[ncbi.nlm.nih.gov]
Ischemic Changes
  • changes adjacent to necrotic postcapillary venules hemorrhage more prominent than demyelination no specific immunohistochemical pattern 4 MRI This is the gold-standard and may depict aforementioned morphologic changes such as: large tumefactive lesions[radiopaedia.org]

Treatment

Prognosis

  • Aggressive surgical and medical therapy, including high-dose corticosteroid therapy and continuous intracranial pressure monitoring may yield favorable outcomes in an entity previously associated with dismal neurologic prognosis.[ncbi.nlm.nih.gov]
  • Prognosis - Acute hemorrhagic leukoencephalitis AHL has a very poor prognosis, with rapid deterioration and death usually occurring within days to one week after onset of symptoms because of severe inflammation in the brain.[checkorphan.org]
  • Treatment and prognosis Acute hemorrhagic leukoencephalitis is associated with a very poor prognosis with the majority of affected individuals succumbing to the disease.[radiopaedia.org]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Clinical, clinico-pathologic, and pathologic aspects of the disease are discussed, and etiologic factors mentioned. Some thoughts on the relationship of this disease to experimental allergic encephalomyelitis are mentioned.[ncbi.nlm.nih.gov]

Epidemiology

  • Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features . Pediatr Infect Dis J . 2004 ; 23 ( 8 ): 756 – 64 . 2. Tenembaum , S , Chamoles , N , Fejerman , N.[cambridge.org]
  • Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J. 2004;23:756-764. Tenembaum S, Chamoles N, Fejerman N.[rarediseases.org]
  • ., The epidemiology of multiple sclerosis: a general overview, Ann. Neurol.,1994, 36, S231–S243 Crossref Google Scholar [2] Brinar V. V., Habek M., Diagnostic imaging in acute disseminated encephalomyelitis, Expert Rev.[degruyter.com]
  • […] grading and staging of metastasis, the clonal origin of cancer, the growth of cancers, the molecular genetics of cancers, viruses and human cencer, chemical carcinogenesis, physical carcinogenesis, tumor immunology, systemic effects of cancer on the host, epidemiology[departamentmorfologie.ro]
  • It discussed also the epidemiology of DENV and the increasing prevalence and incidence of the disease and its extension to new countries.[journals.plos.org]
Sex distribution
Age distribution

Pathophysiology

  • Most often described as a post-infectious complication of an upper respiratory illness, its precise pathophysiology remains unclear.[ncbi.nlm.nih.gov]
  • Vartanian replies: To the Editor: The pathophysiology of necrotizing angiitis in patients with acute hemorrhagic leukoencephalitis is poorly understood.[nejm.org]
  • Although the pathophysiology of SAE is incompletely understood, it is thought to involve endothelial activation, blood — brain barrier leakage, inflammatory cell migration, and neuronal loss with neurotransmitter imbalance.[jdrntruhs.org]
  • The pathogen that caused the cocoliztli epidemics in Mexico of 1545 and 1576 is still unknown, and the 1545 epidemic may have been bacterial rather than viral. [2] [3] Pathophysiology [ edit ] Different hemorrhagic fever viruses act on the body in different[en.wikipedia.org]

Prevention

  • Prevention - Acute hemorrhagic leukoencephalitis Not supplied. Diagnosis - Acute hemorrhagic leukoencephalitis Not supplied.[checkorphan.org]
  • Page 396 - Fox JW, Lamperti ED, Eksioglu YZ, Hong SE, Feng Y, Graham DA, Scheffer IE, Dobyns WB, Hirsch BA, Radtke RA, Berkovic SF, Huttenlocher PR, Walsh CA (1998): Mutations in filamin 1 prevent migration of cerebral cortical ceurons in human periventricular[books.google.com]
  • Is chronic lung disease in low birth weight infants preventable? A survey of eight centers. ‎ Página 84 - Tanner JM, Whitehouse RH, Marubini E, Resele LF. The adolescent growth spurt of boys and girls of the Harpenden growth study. ‎[books.google.es]
  • Centers for Disease Control and Prevention (CDC).[en.wikipedia.org]

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