Case Presentation by Dr. Brian Holowecky
History of Present Illness:
A 13-year-old obese woman with a past medical history of chronic headaches presents to the emergency department for five days of a headache that is associated with nausea and vomiting. The headache started in the frontal region and now involves the entire head. The pain was initially improved by acetaminophen and ibuprofen, but now is persistent despite these medications. According to mom, the patient is confused, is walking into walls, is vomiting all day, and has new onset right sided weakness in the upper and lower extremities. The patient complains of tingling in her right hand and reports weakness in her right foot. She also reports photophobia, dizziness, and an unsteady gait. There is no history of trauma, international travel, or dental problems. She is pre- enarchal. She also is complaining of severe intermittent sore throat and chest discomfort x3 weeks. “It feels like something is getting stuck in my throat.” She has been treated by her pediatrician for GERD x 3 weeks. One day prior to admission she was dx’d by pediatrician with viral etiology of sore throat with a negative Rapid Strep Test.
Review of Systems:
General: Vomiting, difficulty sleeping, nausea
HEENT: sore throat, congestion, blurry vision twice in past 7 days. No toothache
Cardiac: No chest pain or palpitations
Lungs: Cough x 1 week
Abd: Denies blood in stool, diarrhea
G/U: No blood in urine. No dysuria, hematuria, nocturia
Skin: Some bruises from softball practice.
Neuro: Headaches x 6 months
Heme/Immunologic: No allergies, no bleeding
Past Medical History:
Chronic migraines, seasonal allergies, GERD
Mom – Schizophrenia vs Borderline Personality Disorder, depression, agoraphobia
Brother – “Hole in his heart at birth that went away.”
Father – No medical problems.
Paternal Grandfather – Wilson’s Disease
Pt lives at home with mom, dad, brother and dog. Older sister who doesn’t live at home has pet pythons. Patient plays competitive softball and is usually very active. Father is a truck driver who delivers loads between the Ohio River Valley and the American Southwest. Pt occasionally rides with him on trips. The family extensively gardens.
Vitals: Temp 38.2 HR 125 RR 20 BP 144/83 saturation 99%
General: Obese adolescent girl appears in moderate distress. Confused/Anxious
HEENT: Bilateral Papilledema. No conjunctival hemorrhages. No photophobia. No sinus
tenderness to percussion. Oropharynx clear of lesions with normal healthy dentition.
Neck: Supple. No lymphadenopathy. No masses. No meningismus.
Lungs: Lungs CTAB. No retractions. Breathing non-labored.
Cardio: Regular rate and rhythm. No systolic murmur.
Abdomen: Soft. Epigastric/LUQ tenderness. No guarding or rebound. No organomegaly.
Musculoskeletal: R side weakness upper and lower extremity. Weakness appears more
proximal. No clonus. No babinski.
Neuro: Right sided facial weakness with decreased nasolabial folds without forehead sparing. Slight R tongue deviation. EOMI. PERRL. Other cranial nerves intact. Right Upper
Extremity 2-3/5 strength. Right lower extremity 3/5 strength. Left upper/lower
extremities 5/5. Diffuse symmetical hyporeflexia 1+. Sensation intact. Abnormal gait
– favoring the right side.
Patient was referred from outside hospital for suspicious lesions on head CT. IV access was obtained. Blood work sent. Patient sent to CT for confirmation. Also blood cultures, UA, CXR, urine electrolytes. Consult was placed to neurosurgery and infectious disease.
CBC – 13.57/11/32/411
Electrolytes – 129/4.0|97/21|8/0.67
AST/ALT – 12/13
INR – 1.2
ESR/CRP – 56/227 (elevated)
CXR – No acute process.
UA – ketonuria, proteinuria. No other abnormalities.
Urine electrolytes WNL
The patient was started on dexamethasone secondary to her neurological findings. She was empirically treated with ceftriaxone, vancomycin, metronidazole, and bactrim.
Figure 1. Head CT
Later in her extensive hospital course, this patient was found to have hilar lymphadenopathy an esophagomediastinal fistula. Plasma serology came back positive for histoplasmodium.
There is extravasation of contrast on the right side proximally to the third rib. Contrast is seen collecting in a small featureless structure just to the right and superior to the actual fistula/or leak. The esophagus is displaced to the left likely due to fluid collection, abscess, lymphadenopathy or a combination of the above.
1. Which of the following is an absolute contraindication to performing a LP?
a) Elevated ESR/CRP
d) Gait disturbance
2. Which finding explains the mechanism of hematogenous spread from a mediastinal lesion to
the formation of brain abscesses?
a) systolic ejection murmur
b) microcytic anemia
d) feculent vomit
3. Which of the following raises the greatest suspicion for a space occupying lesion in the
a) unilateral distribution
b) not relieved by hydromorphone
c) vomiting in the morning
One Step Further:
How is Histoplasma transmitted between hosts?
a) Spores in the arid Southwest USA
b) Fecal/Oral Transmission of endospores
c) Inhalation of microconidia in dust
d) Inhalation of pidgeon feces
This is a case of an obese adolescent girl from Grand Rapids, MI, who was found to have 2 discrete brain abscesses and a splenic abscess with positive cultures for Viridans Streptococci. Her serum was positive for histoplasma, which is endemic to the upper Midwest. The nidus of infection is presumed to be from histoplasma lymphadenitis that led to an esophagomediastinal fistula, which then allowed normal flora of the oropharynx to become a disseminated bacterial infection of normal flora in an immunocompetent young girl.
Her social history was uniquely important in her case. She had multiple classic social history risk factors for a variety of rare and unusual possible pathogens. Her father being a truck driver in the midwest (histoplasmosis) and traveling to the arid Southwest (cocciodides). Her sister had exotic pets. She was a gardener which is a classic history for a patient with a nocardia infection. Bactrim was added for nocardia coverage.
Hospital Course: Admitted with consult to infectious disease. Patient started on broad-spectrum antibiotics eventually with the addition of Amphotericin B, to her regimen of Vancomycin, Rocephin, Bactrim, Metronidazole. Complications included Acute Kidney Failure from her antifungal medication, red man syndrome from Vancomycin.
Her neurological symptoms resolved over the course of one week, with persisten bilateral papilledema. She underwent a head/neck ULS to rule out obstruction as a cause for venous congestion and papilledema, which was normal.
Figure 3. MR with spectroscopy of brain lesions.
An MR with spectroscopy was ordered to determine the etiology of the brain lesions. An MR signal produces resonances that corresond to various excited states of various metabolites and isotopes. It was used to confirm that the lesions were indeed due to abscesses rather than a neoplasm or other etiology. There was a large lactate doublet which was interpreted by infectious disease and radiology to be infectious in nature.
Patient underwent an extensive workup that revealed:
—Histoplasma positive serology testing
–splenic and brain abscesses positive for S. viridans.
Echo revealed no PFO or shunt with bubble study. There were no lung lesions.
The working diagnosis is histoplasma mediastinal lymphadenitis that caused an erosion leadingto an esophagomediastinal fistula, which lead to bacteremia and hematogenous spread to discrete lesions in the spleen and brain.
Learning points from this case:
Differential for ring-enhancing lesions on Head CT:
Ring enhancing brain lesions are suggestive of a variety pathologies. The ring enhancement is usually due to edematous changes. The rings suggest that the pathology is chronic or resolving as in the case of an abscess, or hematoma. Multipe infections can lead to ring enhancing lesions most notably
M – Metastasis
A – Abscess (toxoplasmosis, cryptococcus, coccidiodes, blastomycosis, neurocysticercosis, nocardia)
G – Glioma
I – Infarct (resolving)
C – Contusion/Hematoma (Resolving)
D – Demyelinating Disease
R – Radiation Necrosis
Figure 4. Head MRI.
Shows two discrete ring enhancing lesions. The lesion in the internal capsule on the left side is the likely etiology of her right sided weakness. The MRI also reveals edematous changes in the optic nerve consistent with papilledema.
Figure 4. Head MRI (continued)
Hematogenous Spread of Infection
Hematogenous spread of infection is a common source of disseminated bacterial or fugal disease. However, there are only a few mechanisms responsible for the spread of disease from the infracranial region to the brain. The most common mechanism is hematogenous spread through the systemic and pulmonary circulation. However, this invariably leads to pulmonary nodules as some of the bacteria seed the lungs prior to entering the heart, which would have been seen on CT.
The presence of a shunt can explain the lack of pulmonary findings. The most common type of shunt that bypasses the pulmonary circulation is a vegetation that passes from the right atrium to the left atrium through a patent foramen ovale. The most common murmur in patient with a PFO is a midsystolic ejection murmur that is heard best at the upper sternal border. An echo with bubble study can also be used for confirmation.
1. C) Papilledema is an absolute contraindication to an LP. Any clinical finding that suggests an acute elevation of intracranial pressure is a contraindication to LP. The risk of performing an LP in a patient with elevated ICP is the potential for a decrease in pressure of the spinal cavity resulting in herniation of the cerebellum and subsequent brainstem impingement. This can lead to rapid deterioration and death.
Intoxication is not an absolute contraindication to LP.
ESR/CRP elevation is a nonspecific finding. Elevations are due to an increase in inflammation,but do not indicate that the inflammation originates in the nervous system.
Gait Disturbance should raise your suspicion for increased intracranial pressure, especially from hydrocephalus. However, it is not an absolute contraindication to lumbar puncture.
2. A) systolic ejection murmur. May indicate an atrioseptal defect or patent foramen ovale. The presence of a PFO allows for infectious agents to bypass the lungs and enter the systemic circulation. When there is no shunt, the infectious agent often becomes lodged in the distal pulmonary vasculature causing pulmonary lesions on chest radiography.
Microcytic anemia is not associated with hematogenous spread of bacteria. Macrocytic anemia is associated with splenomegaly due to erthyrocyte sequestration.
Feculent vomit should raise your suspicion for a bowel obstruction.
Splenomegaly may be due to a splenic abscess, but does not explain the mechanism of hematogenous spread to the brain.
3. C) Morning vomiting is a concerning sign of an intracranial lesion. Intracranial pressure is highest in the morning after sleeping through the night in a recumbent position.
Unilateral distribution of a headache is a nonspecific finding. It may be seen in migraine type headaches, cluster headaches, or even tension headaches.
Phonophobia is classically associated with a migraine headache and extracranial tumors of the vestibular system.
Hydromorphone is not indicated for headache treatment.
One step further: C) Inhalation of microconidia in dust. Histoplasma is endemic to the Ohio and Mississippi River valleys. It is spread by inhalation of microconidia in dust contaminated with excreta from bats, starlings, or chickens. It is classically associated with spelunkers and chicken farmers.