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Extensive cortical diffusion restriction in a 50-year-old female with hyperammonemic encephalopathy and status epilepticus.

de Havenon A, French K, Ansari S - Case Rep Neurol Med (2014)

Bottom Line: Diffuse CDR is most frequently seen after hypotension or hypoxia, which our patient did not experience.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, University of Washington, Box 359775, 325 Ninth Avenue, Seattle, WA 98104, USA.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus

MRI of the brain four days after Figure 1. ((a), (b), and (c)) DTI images with hyperintense cortical diffusion restriction, now also involving ((b), dotted arrows) the thalami and basal ganglia, and ((b) and (c), solid arrows) the occipital cortices; ((d), (e), and (f)) axial apparent diffusion coefficient (ADC) images with hyperintensity of the previously hypointense cortical areas, confirming the transition from cytotoxic edema to extracellular edema, consistent with cerebral edema seen on CT. However, the areas of new diffusion restriction seen on the DTI have a hypointense ADC correlate ((e) and (f), corresponding solid and dotted arrows), suggesting they are due to a more recent event.
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fig2: MRI of the brain four days after Figure 1. ((a), (b), and (c)) DTI images with hyperintense cortical diffusion restriction, now also involving ((b), dotted arrows) the thalami and basal ganglia, and ((b) and (c), solid arrows) the occipital cortices; ((d), (e), and (f)) axial apparent diffusion coefficient (ADC) images with hyperintensity of the previously hypointense cortical areas, confirming the transition from cytotoxic edema to extracellular edema, consistent with cerebral edema seen on CT. However, the areas of new diffusion restriction seen on the DTI have a hypointense ADC correlate ((e) and (f), corresponding solid and dotted arrows), suggesting they are due to a more recent event.

Mentions: Over the next two days, on hospital days 4 and 5, the patient developed febrile sepsis but was not significantly hypotensive or hypoxic. On hospital day 6, despite the cessation of cisatracurium and propofol, she remained comatose with sluggishly reactive pupils and a weak gag response. EEG showed prolonged generalized suppression alternating with brief periods of low amplitude slow wave activity, ultimately progressing to diffuse attenuation. On hospital day 7, her exam remained unchanged and a repeat MRI brain demonstrated cerebral edema with bilateral uncal herniation and the extensive CDR seen on the first MRI, but there was spread of the CDR to the occipital cortices, the bilateral thalami, and basal ganglia. The prior areas of CDR were now bright on T2 and ADC, consistent with an evolution to extracellular cerebral edema, while the new areas of CDR were dark on ADC, suggesting a more recent insult still in the phase of cytotoxic edema (Figure 2). After discussion of the patient's prognosis with her husband, supportive care was withdrawn and the patient died shortly thereafter.


Extensive cortical diffusion restriction in a 50-year-old female with hyperammonemic encephalopathy and status epilepticus.

de Havenon A, French K, Ansari S - Case Rep Neurol Med (2014)

MRI of the brain four days after Figure 1. ((a), (b), and (c)) DTI images with hyperintense cortical diffusion restriction, now also involving ((b), dotted arrows) the thalami and basal ganglia, and ((b) and (c), solid arrows) the occipital cortices; ((d), (e), and (f)) axial apparent diffusion coefficient (ADC) images with hyperintensity of the previously hypointense cortical areas, confirming the transition from cytotoxic edema to extracellular edema, consistent with cerebral edema seen on CT. However, the areas of new diffusion restriction seen on the DTI have a hypointense ADC correlate ((e) and (f), corresponding solid and dotted arrows), suggesting they are due to a more recent event.
© Copyright Policy - open-access
Related In: Results  -  Collection

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fig2: MRI of the brain four days after Figure 1. ((a), (b), and (c)) DTI images with hyperintense cortical diffusion restriction, now also involving ((b), dotted arrows) the thalami and basal ganglia, and ((b) and (c), solid arrows) the occipital cortices; ((d), (e), and (f)) axial apparent diffusion coefficient (ADC) images with hyperintensity of the previously hypointense cortical areas, confirming the transition from cytotoxic edema to extracellular edema, consistent with cerebral edema seen on CT. However, the areas of new diffusion restriction seen on the DTI have a hypointense ADC correlate ((e) and (f), corresponding solid and dotted arrows), suggesting they are due to a more recent event.
Mentions: Over the next two days, on hospital days 4 and 5, the patient developed febrile sepsis but was not significantly hypotensive or hypoxic. On hospital day 6, despite the cessation of cisatracurium and propofol, she remained comatose with sluggishly reactive pupils and a weak gag response. EEG showed prolonged generalized suppression alternating with brief periods of low amplitude slow wave activity, ultimately progressing to diffuse attenuation. On hospital day 7, her exam remained unchanged and a repeat MRI brain demonstrated cerebral edema with bilateral uncal herniation and the extensive CDR seen on the first MRI, but there was spread of the CDR to the occipital cortices, the bilateral thalami, and basal ganglia. The prior areas of CDR were now bright on T2 and ADC, consistent with an evolution to extracellular cerebral edema, while the new areas of CDR were dark on ADC, suggesting a more recent insult still in the phase of cytotoxic edema (Figure 2). After discussion of the patient's prognosis with her husband, supportive care was withdrawn and the patient died shortly thereafter.

Bottom Line: Diffuse CDR is most frequently seen after hypotension or hypoxia, which our patient did not experience.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, University of Washington, Box 359775, 325 Ninth Avenue, Seattle, WA 98104, USA.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus