Acute change in mental status following a four week period of constant headache and associated progressive confusion.

The patient is a 59-year old Korean woman with systemic lupus erythematosis (SLE) who presented to the emergency room in 10/04 with an acute change in mental status following a four week period of constant headache and associated progressive confusion.

Her lupus was diagnosed in 1986 after she presented with fatigue, PIP joint pain and swelling. She was initially placed on Plaquenil; however, that was discontinued secondary to macular degeneration. She had many episodes of serositis of the abdomen and pleura between 1986 and 1991 that required hospitalization. In 2003, she presented with lower extremity edema, shortness of breath, and proteinuria (8 g/day) and had a renal biopsy that demonstrated focal proliferative and membranous glomerulonephritis (WHO class III and class V). She was started on mycophenolate mofetil (2g/d) and prednisone (60 mg/d). Over the ensuing year, her proteinuria improved significantly with the protein to creatinine ratio falling from 8 to <1. Her prednisone dose was tapered to7.5 mg/d and she continued mycophenolate mofetil. Her joint disease had been quiet and she felt well symptomatically until 9/04.

On 10/11/04 she was admitted to an outside hospital because of 2 weeks of headache, confusion, aphasia, and altered mental status. Her family gave a history of indolent worsening of mental function after she complained of a headache for a few days. Over the ensuing weeks she gradually spent more time in bed, was somnolent, and had difficulty articulating requests. This was a distinct change in behavior and function. She also noted night sweats but no fever or rigors. There was no loss of consciousness, seizure activity, or focal weakness. Her joints were not red, hot or tender. She had been compliant with her medications. She had not traveled outside the US, had no exposure to farm animals or unpasteurized products, and was not an active spelunker.

After consultation with her physicians at Hopkins, she was transferred to JHH for further evaluation.

SLE-related pleurisy and abdominal serositis, thrombocytopenia
Herpes Zoster
Oral Thrush
Retinal vein thrombosis which left her blind in her left eye
Hypertension
Hypothyroidism
Raynaud's phemonenon

No history of autoimmune diseases or cancer

Born in Korea. Has been living in United States for over 20 years, and works in the post office. Her husband died 2 years ago and she live with a son who is an engineer. They live in Randallstown and have 1 cat. Her other son is a neurologist and lives in San Francisco. She has a large local extended family. She is a non-smoker, does not drink alcohol or use illicit drugs. She was PPD negative in 1986.

Mycophenolate Mofetil 1000 mg bid
Prednisone 7.5 mg/day
Losartan
Synthroid
Prevacid

NKDA

Patient was unable to cooperate

Upon hospitalization, the patient's temperature was 37.5 degrees Celcius, blood pressure was 120/55, heart rate was 55, and respiratory rate 20. The patient was awake but drowsy and was not oriented to time. The physical exam was significant for right facial droop, right knee hyperreflexia, right hand, leg and foot weakness, and up-going toes, bilaterally. The patient scored 18/30 on the Mini-mental exam. The lung and heart and abdominal exam were unremarkable. No lymphadenopathy was noted. There was no nuchal rigidity.

WBC 6620, RBC 2.70, HgB 9.1, platelets 259, MCV 106 (patient's baseline).
Na 141, K 4.3, Chloride 112, CO2 19, Urea-Nitrogen 12, Glucose 124, Ca 6.9, Creatinine 0.9, Albumin 2.6.
ALT 23, AST 21, total bilirubin 0.3, INR 0.9, PTT 24.5.
ANA titer 1:160. HIV negative. Toxoplasma IgG pending.
Urinalysis: Occasional white blood cells, no significant protein.

MRI of the brain (Image 1):
FLAIR and post-GdDTPA MR images show bilateral basal ganglia masses, larger on the left side (3 cm) centered in the globus pallidus associated with a sattelite lesion anteriorly near the caudate head, and on the right side (about 1.6 cm) centered in the right caudate/anterior putamen. Both dominant masses show irregular ring enhancement, corresponding to peripheral restricted diffusion, and associated with moderate surrounding vasogenic edema and mass effect.
FLAIR--fluid attenuated inversion recovery
T1+C--T1-weighted image with contrast
DWI--diffusion weighted image
ADC--apparent diffusion coefficient

The patient developed worsening changes in mental status including loss of orientation to time and loss of short term memory.

What is your differential diagnosis for her signs, symptoms, and radiographic findings?
What diagnostic procedure would you suggest?
What factors likely predisposed her to develop this condition?