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What Is the Diagnosis?
A 12-year-old girl with systemic lupus erythematosus (SLE) is brought to the emergency department by her parents due to difficulty awakening her. She needed constant stimulation to stay awake. Over the past 2 days, she had a flu-like illness and vomiting, with the inability to take her corticosteroid and antihypertensive medications. Vital signs show:
Temperature: 37°C (98.6°F)
Pulse: 60/min
Respirations: 40/min
Blood pressure: 180/130 mmHg
She is obtunded but responds to painful stimuli. Funduscopic examination reveals bilateral papilledema with flame hemorrhages. Her deep tendon reflexes are brisk and symmetric. The erythrocyte sedimentation rate (ESR) is 12 mm/h.
Question: Which of the following is the most likely diagnosis?
A) Brain neoplasm
B) Cerebral vasculitis
C) Hypertensive encephalopathy
D) Idiopathic intracranial hypertension
E) Intracerebral hematoma
F) Reye syndrome
Answer: C. Hypertensive Encephalopathy
SLE (Systemic lupus erythematosus)
The autoimmune condition causes systemic inflammation and multisystem involvement.
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Flu-like illness & vomiting (due to SLE) + Inability to take antihypertensive medications
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Hypertension ↑ is due to a lack of control of blood pressure.
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Hypertension (180/130 mmHg) →
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Affects brain autoregulation 🧠
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Cerebral edema ↑
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↑ Intracranial pressure (ICP) → Papilledema 🧠 (evident on the funduscopic exam).
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Papilledema with bilateral flame hemorrhages
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Indicates damage to the small vessels in the brain due to ↑ ICP and uncontrolled hypertension.
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Brisk reflexes & obtunded state
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Signs of hypertensive encephalopathy 🧠.
Why the Other Options are Wrong:
A) Brain Neoplasm
No progressive symptoms: Brain neoplasms typically present with gradually worsening symptoms like headaches, seizures, or focal neurological deficits over weeks to months.
No mass effect signs: Funduscopic findings in neoplasms may show papilledema, but flame hemorrhages are not characteristic.
B) Cerebral Vasculitis
Usually involves systemic symptoms: Cerebral vasculitis associated with SLE would present with multifocal neurological deficits (e.g., weakness, seizures) rather than just altered consciousness.
ESR elevation: In vasculitis, the ESR would be markedly elevated due to active inflammation (12 mm/h here is too low).
D) Idiopathic Intracranial Hypertension (IIH)
No hypertension: IIH typically occurs in patients with normal blood pressure.
Not related to SLE: IIH is more common in obese young women and isn't associated with SLE or missed medications.
E) Intracerebral Hematoma
Acute focal neurological deficits: Intracerebral hematomas cause sudden focal deficits (e.g., hemiparesis), not the gradual obtundation seen here.
No trauma or bleeding tendency: There's no history of trauma, coagulopathy, or bleeding disorder.
F) Reye Syndrome
Post-viral in children with aspirin use: Reye syndrome is typically seen in children with aspirin use following a viral illness, not in a patient with SLE.
Liver failure symptoms: Reye syndrome usually involves vomiting, confusion, and liver dysfunction, which are absent here.
Flashcards
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Dr. Shoaib