|Year : 2015 | Volume
| Issue : 3 | Page : 196-198
Sepsis presenting as acute hemorrhagic leukoencephalopathy: A rare case report
Om Prakash Lekhra1, Pranav Chowdhary2
1 Department of Neurology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India
2 Department of Radiology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India
|Date of Web Publication||15-Sep-2015|
Om Prakash Lekhra
Quince 205, Shalimar Township, AB Road, Indore, Madhya Pradesh
Source of Support: Nil., Conflict of Interest: None declared.
Sepsis-associated encephalopathy (SAE) refers to a clinical spectrum of acute neurological dysfunction that arises in the context of sepsis. 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. SAE is associated with a high risk of mortality. Imaging studies using MRI and CT have demonstrated changes in the brains of patients with SAE that are also seen in disorders such as stroke.
Keywords: Encephalopathy, hemorrhagic leukoencephalopathy, sepsis
|How to cite this article:|
Lekhra OP, Chowdhary P. Sepsis presenting as acute hemorrhagic leukoencephalopathy: A rare case report. J NTR Univ Health Sci 2015;4:196-8
| Introduction|| |
Encephalopathy is a common complication of sepsis. It is the most common form of encephalopathy among patients in intensive care units. 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, and coma. Occasional focal deficits and seizures may be there. It is usually the result systemic metabolic impairment. Sepsis associated encephalopathy (SAE) is often used when encephalopathy develops in the setting of identified extracranial infection and septicemia. It is due to action of inflammatory mediators on the brain. SAE is not used when there is direct brain infection (e.g., meningitis, brain abscess, or cerebritis). It varies from a transient, reversible encephalopathy to irreversible brain damage. The specific and reliable imaging parameters do not exist for SAE patients so far. Only few reports of various degrees of leukoencephalopathy, ischemic lesions and posterior reversible encephalopathy syndrome (PRES) like lesions with SAE are there in the literature. We describe a case with sepsis presenting with interesting and rarely associated radiological finding of acute hemorrhagic leukoencephalopathy.
| Case Report|| |
A 28-year-old female had abdominal pain 15 days back for she took abdominal massage. After few days pain reappeared and then she was subjected to laparotomy for ruptured peritonitis. In the postoperative period she developed fever and altered sensorium. 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. She had no meningeal signs. On deep painful stimuli she had minimal movements in upper limbs, but no movement in the lower limbs. Plantars were bilaterally extensor. Further investigation revealed complete blood count (CBC) 1st: Hemoglobin (Hb) 11.9 g% total lymphocyte count (TLC) 1200 N70, L26 M2 E2 B0, red blood cell (RBC) 4.76 mill/cmm, packed cell volume 37.7, mean corpuscular volume 79.2 cmm, mean corpuscular haemoglobin (MCH) 25.0 pg, MCH concentration 31.6% platelets count 1.70 lacs/cmm, CBC 2nd: Hb 10 g%, RBC 4.15 mill/cmm, TLC 14,900, N80, L11 M4 E5 platelets 1.58 lacs/cmm. Malaria smear and antigen negative. Renal and liver function tests were almost normal. Blood urea 24 mg/dl, serum creatinine 0.8 mg/dl. serum electrolytes were normal limits except hypokalemia at few instances, serum bilirubin 1.0 mg/dl, total proteins. Proteins-5.0 Alb-2.6, Glob-2.4, serum glutamic pyruvic transaminase 34 IU/L. D-dimer 0.68 (N< 0.3 mg/L), blood culture: Escherichia More Details coli sensitive to imipenem, central tip c/s magnetic resonance spectroscopy growth seen, urine examination normal. Subsequent pus cultures revealed E. coli/Klebsiella pneumonia. Two-dimensional echo normal, cerebrospinal fluid (CSF) analysis clear 3 cells/cmm all lymphocytes: Proteins 26 mg%: Glucose 58%: 58 mg% Pandy's test negative: Adenosine deaminase 5.68 unit/L, serum B12 level >2000 pg/ml. Ultrasonography Abd: Mesentery in the right iliac fossa and pelvic region is inflamed and is suggestive of peritonitis. Computed tomography (CT) abdomen revealed bilateral mild pleural effusion with underlying collapse and consolidation of right lower basal segment and features of peritonitis. Subsequent pus cultures revealed E. coli and K. pneumonia at two different occasions. Magnetic resonance imaging (MRI) diffusion-weighted imaging (DWI) showed extensive cytotoxic edema of white matter over centrum semiovale and periventricular area [Figure 1]a and susceptibility-weighted imaging (SWI) showed multiple small petechial hemorrhages in the affected white matter [Figure 1]b. She needed intensive management with antibiotics, antiedema measures for few weeks and had total neurological recovery after about 2 weeks. Repeat MRI brain showed almost resolution of the cytotoxic edema seen in DWI [Figure 2]a and petechial spots also diminished in SWI [Figure 2]b. This MRI appearance suggested the possibility of acute hemorrhagic leukoencephalopathy.
|Figure 1: (a) Diffusion-weighted imaging show extensive cytotoxic edema of white matter over centrum semiovale, periventricular region, (b) susceptibility-weighted imaging show widespread punctate blooming hypointensities suggestive of microbleeds in the subcortical white matter|
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|Figure 2: (a) Repeat diffusion-weighted imaging show a signifi cant reduction in white matter cytotoxic edema, (b) repeat susceptibilityweighted imaging show diminished petechial spots|
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| Discussion|| |
Clinical features of encephalopathy and encephalitis are overlapping in the sense that altered sensorium is common in both of them. Additional symptoms of fever, headache, vomiting, seizures and neck stiffness suggest encephalitis and the evidence of deranged metabolic parameters favor encephalopathy. There appears to be no method to distinguish encephalopathy and encephalitis clinically. The best way is biopsy, but it may not be ethical. At present, the only way to distinguish between the two conditions reasonably is CSF analysis. Encephalitis was ruled out in our case as CSF analysis was normal. Hence, in presence of systemic septicemia a possibility of SAE was considered.
Sepsis syndromes are associated with high morbidity and mortality rates in intensive care units. Brain dysfunction is frequently observed in sepsis as a consequence of changes in cerebral structure and metabolism, resulting in worse outcome and reduced life-quality of surviving patients.
Review of literature show only a few case reports of brain imaging abnormalities in SAE., When the blood-brain barrier gets damaged vasogenic edema develops in the extracellular space. On imaging this is seen over white matter predominantly as commonly seen in cerebral hematoma, tumor, trauma and late ischemia. Cytotoxic edema develops when blood-brain barrier is intact. Retention of sodium and water in astrocytes occurs due to sodium potassium pump failure leading to restriction of the water molecule diffusivity and loss of grey white matter interphase as seen in early ischemia and early ischemia. CT scans in SAE are mostly normal however; also white matter hypodensities are reported. MRI allows the identification of new aspects of brain damage in SAE. Brain damage occurred mainly within the white matter and corresponded to vasogenic edema, probably reflecting blood-brain barrier breakdown. Various degree of white matter edema and ischemic infarcts have been reported in a series with septic shock and acute neurological signs. The intensity of these lesions had an impact on the prognosis. Nonspecific white matter lesions are also described in other studies., Finelli and Uphoff reported cases with bilateral basal ganglia, thalamic, cerebellar, brainstem, and cerebral MR abnormalities in sepsis. Furthermore, MR abnormalities in terms of a PRES have often been found in patients with SAE. However, some of the SAE patients did not show any MRI abnormalities. MRI showed multiple petechial hemorrhages in the white matter edema over centrum semiovale and periventricular region indicating cytotoxic edema [Figure 1]a and [Figure 1]b. These petechial hemorrhages are infrequently reported in SAE. Repeat study after 4 weeks showed diminished white matter edema and the resolution of the petechial lesions. Possible explanation is that infection triggers an autoimmune response, which result into perivascular demyelination and hemorrhage in brain parenchyma. Actually, these petechial hemorrhages could be the end result of disseminated intravascular coagulation or a manifestation the fibrinoid necrosis of small blood vessels in sepsis. Once the immune response improves hemorrhages resolve as evident in the repeat images.
Thus, specific and reliable imaging parameters do not exist for SAE patients so far. Nevertheless, MRI is a valuable tool to exclude other cerebral pathologies leading to brain dysfunction in sepsis. DWI, fluid attenuated inversion recovery, SWI, contrast-enhanced, and spectroscopy sequences are usually recommended to be performed in case of SAE. This case also emphasizes the fact that the severity of MRI changes do not always signify poor outcome of the patient in status epilepticus.
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[Figure 1], [Figure 2]