Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus
Comorbid hyperammonemic encephalopathy (HE) and status epilepticus (SE) leading to extensive cortical diffusion restriction (CDR) on MRI have not been previously reported. We describe a patient with HE who subsequently developed provoked SE. Sequential MRIs demonstrated a progressive CDR that involv...
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Wiley
2014-01-01
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| Series: | Case Reports in Neurological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2014/257094 |
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| author | Adam de Havenon Kris French Safdar Ansari |
| author_facet | Adam de Havenon Kris French Safdar Ansari |
| author_sort | Adam de Havenon |
| collection | DOAJ |
| description | Comorbid hyperammonemic encephalopathy (HE) and status epilepticus (SE) leading to extensive cortical diffusion restriction (CDR) on MRI have not been previously reported. We describe a patient with HE who subsequently developed provoked SE. Sequential MRIs demonstrated a progressive CDR that involved the entire bilateral supratentorial cortex, thalami, and basal ganglia, resulting in death from cerebral edema and brain herniation. Diffuse CDR is most frequently seen after hypotension or hypoxia, which our patient did not experience. Such findings have also been described in both HE and SE (Milligan et al. (2009), Chatzikonstantinou et al. (2011), U-King-Im et al. (2011), and Bindu et al. (2009)), but not to the extent seen in our patient. Additionally, our patient had distinct radiologic features of both disease processes, suggesting a cumulative effect. The diagnosis of HE and SE in the setting of extensive CDR should not be missed and could lead to improved outcomes for two progressive, malignant, and treatable illnesses that can be easily overlooked. |
| format | Article |
| id | doaj-art-5f534335f2cc4a9aa2cfa6bc5e0971c0 |
| institution | Kabale University |
| issn | 2090-6668 2090-6676 |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
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| series | Case Reports in Neurological Medicine |
| spelling | doaj-art-5f534335f2cc4a9aa2cfa6bc5e0971c02025-08-20T03:34:22ZengWileyCase Reports in Neurological Medicine2090-66682090-66762014-01-01201410.1155/2014/257094257094Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status EpilepticusAdam de Havenon0Kris French1Safdar Ansari2Department of Neurology, University of Washington, Box 359775, 325 Ninth Avenue, Seattle, WA 98104, USADepartment of Neurology, University of Utah, 50 North Medical Drive, Salt Lake City, UT 84132, USADepartment of Neurology, University of Utah, 50 North Medical Drive, Salt Lake City, UT 84132, USAComorbid hyperammonemic encephalopathy (HE) and status epilepticus (SE) leading to extensive cortical diffusion restriction (CDR) on MRI have not been previously reported. We describe a patient with HE who subsequently developed provoked SE. Sequential MRIs demonstrated a progressive CDR that involved the entire bilateral supratentorial cortex, thalami, and basal ganglia, resulting in death from cerebral edema and brain herniation. Diffuse CDR is most frequently seen after hypotension or hypoxia, which our patient did not experience. Such findings have also been described in both HE and SE (Milligan et al. (2009), Chatzikonstantinou et al. (2011), U-King-Im et al. (2011), and Bindu et al. (2009)), but not to the extent seen in our patient. Additionally, our patient had distinct radiologic features of both disease processes, suggesting a cumulative effect. The diagnosis of HE and SE in the setting of extensive CDR should not be missed and could lead to improved outcomes for two progressive, malignant, and treatable illnesses that can be easily overlooked.http://dx.doi.org/10.1155/2014/257094 |
| spellingShingle | Adam de Havenon Kris French Safdar Ansari Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus Case Reports in Neurological Medicine |
| title | Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus |
| title_full | Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus |
| title_fullStr | Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus |
| title_full_unstemmed | Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus |
| title_short | Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus |
| title_sort | extensive cortical diffusion restriction in a 50 year old female with hyperammonemic encephalopathy and status epilepticus |
| url | http://dx.doi.org/10.1155/2014/257094 |
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