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BACKGROUND
A 50-year-old woman presents to the emergency department
(ED) because of an altered level of consciousness. The
patient is confused and can only say that "something is
wrong" and that she needs help. A family member provided
a medical history of mood disorder and a seizure
disorder that started about 4 weeks ago, at which time
treatment with phenytoin and clonazepam was started. The
patient had also been admitted to another hospital 7
days ago for status epilepticus. She was intubated for
less than 48 hours. When she awoke from sedation, she
left the hospital against medical advice. She had
undergone a workup including nonenhanced head CT and MRI
and magnetic resonance angiography (MRA) of the brain.
Her family reports that all images were "negative."
The patient's family reports several ED visits to
different hospitals over the last 4 weeks for various
neurologic complaints, though no definitive diagnosis
was made and the patient's condition has not improved.
The main reason for today's presentation in the ED is a
new onset of confusion.
On physical examination, the patient is afebrile with
stable and normal hemodynamic vital signs. She seems
distant and preoccupied and is oriented to only her name
and that she is in a hospital and not to the year or
date. Examination of the head, ears, eyes, nose, and
throat (HEENT) revealed no evidence of trauma or other
abnormality. The cardiovascular and pulmonary
examinations were unremarkable, and the abdomen was soft
and nontender. Vision is noted to be severely impaired
with counting fingers in the right eye and only
recognizing hand motion in the left eye. On neurologic
examination, the patient is noted to have normal
cranial-nerves except her eyes as noted above. She has
preserved and symmetric deep tendon reflexes and normal
5/5 gross motor function in her upper and lower
extremities on the left but is noted to have slightly
increased reflexes with 3/5 weakness in the right arm
and leg. She is also noted to have clonus with upgoing
toes in right lower extremity. Her sensory findings are
normal to the extent that the patient can cooperate, and
she is able to touch her finger to her nose without
noticeable dysfunction. Further neurologic evaluation
including evaluation for ataxia is not possible because
the patient is unable to cooperate.
The patient is admitted for confusion of unknown
etiology. In addition to routine laboratory
investigations (including a urinalysis and a chemistry
panel), nonenhanced head CT is ordered. The scans show
nonspecific findings with no evidence of mass effect or
a localizing, space-occupying lesion. Lumbar puncture is
performed and reveals a mildly elevated protein level
without pleocytosis. As the workup continues, a MRI of
her brain is ordered (see Image).
What is the
diagnosis? |
Hint
This disease is as bad as it looks on the MRI. |
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Authors:
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Gil Z. Shlamovitz, MD,
Department of Emergency Medicine, UCLA/Olive View-UCLA Emergency
Medicine Residency Program, David Geffen School of Medicine at UCLA
Rick G. Kulkarni, MD, Assistant Professor, Yale School of Medicine,
Section of Emergency Medicine, Department of Surgery, Attending
Physician, Medical Director, Department of Emergency Services, Yale-New
Haven Hospital |
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eMedicine Editor:
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Arastoo Vossough, PhD, MD,
Division of Neuroradiology, Department of Radiology, Massachusetts
General Hospital, Boston
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