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A 35-year-old shop clerk was evaluated for increasing lethargy, confusion, double vision and visual loss. She was well until 3 weeks prior when she developed body ache, intractable vomiting and dizziness. She was found to have normal visual acuity, bilateral abduction deficits and retinal hemorrhages. A magnetic resonance imaging scan and lumbar puncture were normal. During the course of the next week, she became more confused and was hospitalized because she began falling, and became lethargic. She continued to have double vision, but also complained of loss of vision. 

Past medical history was notable for hysterectomy and cholecystectomy. She had had a gastric bypass procedure four months prior to the onset of her symptoms, and had experienced an 80 pound weight loss, associated with early satiety and frequent vomiting. 

Her general examination was notable for ill appearance and frequent bouts of vomiting. She was lethargic and oriented to the year but not the exact date. She knew the name of the hospital, but not the clinic. Her speech was normal, but sparse. Cranial nerves showed visual acuity of 20/60 OD and 20/100 OS. Her color vision was reduced. Visual fields were normal peripherally, but she had central scotomata bilaterally. Extraocular movements were slow and limited in all directions, and she had gaze evoked nystagmus, as well as up-beat nystagmus in primary position. Fundus exam revealed bilateral moderate optic nerve edema, with numerous peripapillary hemorrhages. The remainder of the cranial nerves were normal. Motor exam showed mild generalized weakness, especially proximally. Deep tendon reflexes were 1+ throughout with the exception of absent ankle jerks. She had an intention tremor and moderate limb ataxia, with slowed rapid alternating movements. She was not able to walk due to instability. Babinski responses were absent. CSF was normal, and opening pressure was 18cm CSF.

After appropriate treatment, she had rapid improvement of all of her neurologic deficits, but was still not normal after 6 years. At last examination, she had 20/40 vision, full eye movements with vertical pendular and gaze evoked nystagmus, full visual fields with central depression and pale optic nerves. She was able to walk without a walker, but used a wide based gait, and was still not able to perform tandem gait.

Questions:  CASE SUMMARY 10

1.      How would you characterize her mental status?

2.      What are her neurologic deficits and how can you localize them?

3.      What is the differential diagnosis?

4.      Given the clinical circumstances, what is the most likely diagnosis, and what is the treatment?

5.      What is the well described mental sequela of this disorder, and who are most commonly affected?