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Clinico-Pathological Conference
Case Study

Note:
CPC#6, originally scheduled for Tuesday, February 18, 2003, at 12 Noon in Hurd Hall, was postponed due to the snowstorm that hit the Baltimore area. It is rescheduled for Wednesday, March 19, 2003
.

CPC #6: Wednesday, March 19, 2003
Hurd Hall, The Johns Hopkins Hospital
12:00 Noon

Clinical Discussant:   David Newman-Toker, MD
Pathologist Resident:   Saeid Movahedi-Lankarani, MD
Pathologist:   Edward Weir, MD
Radiologist:   Gary Gong, MD
Moderator:   Charles Weiner, MD

Chief Complaint

A 39-year-old female with AIDS who presents with decreased alertness and focal neurologic abnormalities.

History of Present Illness

The patient is a 39-year-old African-American female with long standing history of AIDS with poor medical compliance. She is known to have a history of crack cocaine and alcohol abuse. She was first tested for HIV after an acute outbreak of genital herpes simplex virus infection in January 2001 and was found to have a CD4 count of 9 and HIV RNA viral load of 326,882 copies/mL. She received care at the Moore Clinic, but she did not start HAART therapy due to continued drug abuse. She had multiple AIDS-associated illnesses in the next year including weight loss, MAI infection, decreased memory, Candida esophagitis, chronic diarrhea, and thrush.

In February 2002, after several days of heavy crack cocaine use, she was admitted to an outside hospital for memory loss, decreased responsiveness, and left sided weakness. A head CT scan performed at outside hospital revealed six enhancing lesions, largest in left basal ganglia, while a chest CT scan revealed multiple calcified nodules in the lungs bilaterally. She was noted to have a CD4 count of 3 and HIV RNA viral load greater than 750,000 copies/mL. Sputum cultures were negative. She was placed on dilantin after development of several seizures during hospitalization. She was discharged home to her family six days after admission on dilantin and fluconazole. She was brought to the Moore clinic the following day by family member due to neurologic deterioration. She was unable to provide care for herself, was noted to bite herself and have repeated bouts of incontinence. She was admitted to The Johns Hopkins Hospital.

Review of Systems

At the time of admission the patient was unable to respond to questions.

Past Medical History

  • Polysubstance abuse including crack cocaine, alcohol, and tobacco.
  • History of sexually transmitted diseases including gonorrhea (1980s), chlamydia (1980s), and PID (1997).
  • Vague history of right-sided CVA in the 1980s with postpartum hypertension and no residual deficits.
  • Asthma and chronic bronchitis
  • Gastroesophageal reflux disease
  • History of pyelonephritis during pregnancy
  • Chronic cutaneous folliculitis

Past Surgical History

Bilateral partial salpingectomy (1985)

Social History

The patient lives with her brother and has four children. She is unemployed. She has worked as cashier, telephone solicitor, and janitor in past. She has an 11th grade education.

The patient has smoked 5 cigarettes per day for 18 years. She has been drinking several beers a day since her teenage years. She has a 15-year history of crack cocaine use. She denies any IVDA.

Family History

The patient's father died many years ago from cirrhosis. Her mother died with history of "lung disease". There was no other family history of CNS, cardiac, gastrointestinal, immunologic, or hematologic disease.

Physical Exam on Admission

  • Vital Signs: T 99.3(F, R 32, P 129, BP 95/73, SaO2 88% on RA
  • General: Cachectic, not responsive to commands, NAD
  • Skin: Warm, herpetic lesions in vulvar area
  • HEENT: Eyes clear, conjunctiva slightly pink, left pupil reactive to light while right pupil is sluggish. Patient was unable to follow commands for extra-ocular movements. Oral exam not performed.
  • Neck: Supple. No meningeal signs. No lymphadenopathy.
  • Cardiovascular: Tachycardia with regular rhythm. Normal S1 and S2. No murmurs, rubs, or gallops
  • Respiratory: Rhonchi throughout right side, otherwise clear to auscultation.
  • Abdomen: Soft, nondistended with normal bowel sounds. Slightly tender to palpation in epigastric area. No rebound tenderness and no masses.
  • Extremities: Nonedematous. No Janeway or Osler lesions. No splinter hemorrhages.
  • Neurologic: Unable to respond to verbal commands. Not oriented to person, place, or time. Eyes deviate to the right with sluggish right pupil. Left arm flaccid while right arm and both legs are not flaccid. DTRs are 2+ throughout.

Laboratory Values on Admission

Chemistry: Na+ 132 mEq/L, K+ 5.3 mEq/L, Cl- 93 mEq/L, HCO3- 19 mEq/L, BUN 39 mg/dL, Cr 1.3 mg/dL, Glu 104 mg/dL, Ca2+ 10.1, PO43- 6.5, lactate 1.3
TB 0.4 mg/dL, TP 7.8 g/dL, Alb 3.9 g/dL, AST 41 IU/L, ALT 75 IU/L, Alk Phos 67 IU/L, PT 12.9 (INR 1.1)
CBC: WBC 11,400 cells/mm3, Hgb 14.0 g/dL, Hct 42.9%, Plt 308,000 cells/mm3
Urine: 3-5 WBCs, occasional yeast, no bacteria.
CXR: Scattered nodular masses, bilateral lungs.

Hospital Course

Upon admission the patient was placed on ceftriaxone 2 mg qd for presumed sepsis and started on trimethamine for possible Toxoplasmosis infection. She became more hypoxic and developed increasing oxygen requirements. Radiographic studies, including a brain CT and MRI, were performed. The brain CT (Figure 1) revealed multiple foci of hypodensities with a small hyperdense lesion in the right frontal lobe. The brain MRI (Figures 2A,B,C) revealed multiple masses with a heterogeneous pattern of enhancement, some demonstrating rim enhancement, some with patchy enhancement, and some without enhancement. Masses were identified throughout the infratentorial and supratentorial brain including left cerebellum, bilateral basal ganglia, right thalamus, and right pons. Blood and CSF cultures remained negative but the patient became febrile by third day of admission and developed worsening oxygen requirements as well as developing worsening LFTs, including AST 1471 IU/L, ALT 1055 IU/L, Alk Phos 170 IU/L. Hepatitis viral serologies were all negative. A chest and abdominal CT was performed and revealed multiple bilateral, peripherally based, enhancing lesions in the lungs as well as a filling defect within the right lower lobe pulmonary artery (Figures 3A,B). Trimethoprim was added for possible Pneumocystis carinii infection and patient was started on heparin for probable pulmonary emboli. Due to worsening condition, code status was discussed with family and patient was changed to DNI/DNR. On the seventh day of hospitalization, the patient continued to be febrile and became hypotensive with a Tmax of 40.5°C and systolic blood pressure in the 70-80 range. She developed more labored breathing, became unresponsive without spontaneous movements and expired.

Figure 1
Figure 1

Figure 2A
Figure 2A

Figure 2B
Figure 2B

Figure 2C
Figure 2C

Figure 3A
Figure 3A

Figure 3B
Figure 3B

What was the cause of her neurologic condition?
What is the most likely cause of death?

See Answer to CPC #6

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