It is common knowledge that each patient needs to have a symptomspecific evaluation with each visit, but it is easy to be misled by “frequent fliers” who have presented many times with the same complaint. Take this month’s case, for example: a 37-year-old man with a headache who had four emergency department and two primary care visits before finally receiving the correct diagnosis. Accuracy and vigilance must be the goal of each patient encounter, no matter how seemingly benign the chief complaint or previous diagnoses.

Initial Visit
(Note: The following, as well as subsequent visit summaries, is the actual documentation of the providers, including punctuation and spelling errors.)
CHIEF COMPLAINT (at 11:22): Headache
Temp  Pulse     Resp      Syst        Diast
98.9              104                18                112                   68
HISTORY OF PRESENT ILLNESS (at   11:54):
Pt. is a 37 year old male who presented with complaint of 20-year history of headaches which occur about once per month. The patient was returning from church the day previously and had a constant pain in the frontal region associated with nausea and one episode of vomiting and was similar to past headaches, but lasted longer. No complaints of rhinorrhea, cough, sore throat, earache, dizziness, neck pain, rash, numbness, slurred speech or facial droop, chest pain, SOB, or abdominal pain.
PAST MEDICAL HISTORY/ TRIAGE:
PMH: Negative PSH: Negative Medications: None
SH: Works for Buckeye steel
Physical exam (at 12:00):
General: Alert and oriented X3, well-nourished, in no apparent distress

  • Plan: Continue vicodin and zithromax

 
SUMMARY OF ED VISIT 3

  • Returned two days later at 6 AM. History included ex- tensive synopsis of past visits and treatments includ- ing that pt. had seen PCP again yesterday (the 5thhealth care visit in 6 days) but the only description of current HA was “facial pressure on the right side”
  • ED course: Demerol and phenergan IM
  • Dx: Cephalgia secondary to sinusitis
  • Plan: Change ATB to augmentin, continue vicodin and phenergan

 
SUMMARY OF ED VISIT 4

  • Return same day at 4 PM (10 hours later). History now documents demographics: “37 year male from Guinea who has been in the US for 6 years”. Now complains of “fevers at home”. This is the worst HA of his
  • PE: Normal except tenderness over frontal sinuses. Temperature is 0 degrees
  • ED course: LP performed to look for atypical infec- tion
  • LP results:
    • RBC: Tube 1 = 250, tube 3 = 11
    • WBC = 5 (1 poly and 4 lymph) Gram stain negative
  • Dx: Complicated sinusitis
  • Plan: Change vicodin to Will add crypto- coccal antigen to CSF. D/C to home

 
SUMMARY OF ED VISIT 5

  • called to return to ED a few hours later with positive india ink stain for cryptococcus.
  • Additional history: 35 weight loss in 8 mo.
  • Exam: No thrush,  OHL, adenopathy
  • Dx: Cryptococcal meningitis
  • Plan: Started on amphotericin B and admitted to In- fectious Subsequent HIV test and CD4 count confirms diagnosis of AIDS

Discussion of Documentation, Diagnosis, and Patient Safety Issues
Why did the doctor miss the diagnosis?
Our patient had a case of cryptococcal meningitis from undiagnosed AIDS. His doctors had a case of “diagnosis momentum” from placing too much credence in previous physicians’ evaluations.

In 2002, Pat Croskerry, an ED physician from Canada, described specific features present in the evaluation of patients which may lead the physician astray.2 Diagnosis momentum occurs when a diagnosis becomes established without adequate supporting evidence, and then gathers momentum with each subsequent provider.
Our patient had a CT suggesting sinusitis, a sensitive but not specific finding. He was started on antibiotics and when he did not improve, the azithromycin was changed to augmentin.

If the initial antibiotic does not work for sinusitis, another antibiotic may be tried, but caution should be applied due to the minimal efficacy of antibiotics for sinusitis. The number needed to treat (NNT) with anti-biotics is five to 14 and number needed to harm is 17.3 In other words, antibiotics will only help 6% to 20% of patients and will harm 6%. If the first antibiotic does not work, the chance of the second helping is even less and the initial diagnosis should be revisited to ensure there is nothing else occurring.
 
New Guidelines from ACEP
Two  key questions
In June 2008, The American College of Emergency Physicians (ACEP) released new headache guidelines which answer several questions related to evaluation of patients with acute headache.4 The two points with the most relevance for urgent care are:

  • Which patients with headache require neuroimaging?
  • Patients with older age (over 50-60 years old) with new headache
  • Occipital location of pain
  • Worsening headache with valsalva
  • Headache waking patient from sleep
  • Headache associated with syncope, nausea, or sen- sory distortion
  • Sudden onset severe headache (reaching maxi- mum intensity over seconds to a minute
  • HIV/AIDS patients with new or different headache
  • Pregnant patients
  • Abnormal finding on neurologic examination
  • Does a lumbar puncture need to be performed in patients being evaluated for subarachnoid hemorrhage after a normal brain CT?
  • Limitations of brain CT include inability to identify small hemorrhages in areas obscured by artifact or bone, inability to diagnose other conditions such as idiopathic intracranial hypertension, meningitis, carotid or vertebral artery dissection and some cases of cerebral venous sinus thrombosis or pituitary apoplexy, and decay in sensitivity with time (sensitivity 92% day of rupture and 58% five days later).
  • Of all cases of subarachnoid hemorrhage with normal CT, between 2% and 10% will be identified by positive lumbar puncture

Conclusion (from ACEP guidelines): “The totality of the evidence suggests that lumbar puncture must still be performed after [a] negative CT scan result in patients being evaluated for subarachnoid ”

Tricks for Initial Diagnosis of HIV in Asymptomatic Patients
In an undiagnosed patient, the first clue that a patient may have HIV/AIDS is assessment of risk factors, including HIV-positive sexual contacts, injection drug use, hemophilia, multiple unknown sex partners, or travel to/from areas where HIV is endemic. White, gay men no longer represent the majority of new HIV infections in
the U.S.; over a third of recently infected individuals acquired HIV via heterosexual contact and 46% by homo- sexual contact. Over half of new infections are diagnosed in African-Americans, and 27% are in women.

History may provide clues; AIDS patients presenting with major opportunistic infections typically give a his- tory of repeated minor mucocutaneous infections, such as thrush, recurrent herpes simplex, candida vaginitis, or shingles. Weight loss, night sweats and anorexia are commonly present in late stage HIV.

Physical exam clues to HIV diagnosis depend on the CD4 count. Skin exam may show seborrheic dermatitis, especially over the malar eminences, zoster scars, genital or perianal herpes simplex virus, and tinea. Oral lesions include thrush, oral hairy leukoplakia (pathognomonic for HIV) and linear gingivitis. Generalized lymphadenopathy, with strings of 1 cm to 2 cm nodes in the posterior cervical chain, are typically found. A funduscopic exam may reveal cotton wool spots. Pa- pilledema can be seen with cryptococcus, toxoplasmo- sis, or CNS lymphoma.

In November 2002, reliable, rapid testing for HIV antibodies became available, making the diagnosis of HIV quick and simple.5 Even more recently, the Centers for Disease Control and Prevention initiated a campaign to encourage physicians to obtain HIV testing of all per- sons deemed at risk for HIV infection.

Routine laboratory studies commonly show abnormalities and can support suspicions of undiagnosed HIV infection. Leukopenia with lymphopenia is the rule; its absence argues against HIV. A normochromic, normocytic anemia is common but not universal. Thrombocytopenia is seen in 10% of patients. Patients are commonly co-infected with hepatitis, resulting in abnormal LFTs.

Opportunistic infections (OI) such as cryptococcus or toxoplasmosis typically occur in the later stages of HIV infection when the CD4 count is under 200. Since the CD4 cell count falls 60 to 100 cells per year of HIV infection, it may take years after the initial viral infection for patients to present with an OI.
 
Evaluation   of   Headaches   in   Patients   with   HIV/AIDS
In patients with AIDS, the differential diagnosis includes CNS mass lesions, and a spinal tap should be withheld un- til a head CT scan is performed, confirming there is not a midline shift. While cryptococcus would be the most common cause of subacute meningitis in an AIDS patient in the U.S., other OIs of the central nervous system include cytomegalovirus (CMV), herpes simplex virus (HSV), herpes zoster (VZV), progressive multifocal leukoencephalopathy (PML), tuberculosis (TB), Mycobacterium avium com- plex (MAC), B-cell lymphoma, toxoplasmosis, syphilis, listeria, histoplasmosis, and coccidioides. A cerebrospinal fluid (CSF) examination and cultures of the CSF are needed to help sort out these possibilities.
 
Symptoms and Diagnosis of Cryptococcal Meningitis
Cryptococcus is a ubiquitous organism with a portal of entry via the lungs. It spreads to the CNS hematoge- nously. The most common symptoms of cryptococcal meningitis in HIV patients are chronic headache, fever, and malaise.6 Our patient’s lack of nuchal rigidity is typ- ical in cryptococcal disease; less than half of patients have a stiff neck. Temperatures normally do not exceed 39° C, and are absent in a quarter of patients.7
In AIDS patients with cryptococcal meningitis, the CT scan is normal in most patients, but hydrocephalus and gyral enhancement can be found in some. Cortical atrophy is seen in a third of patients.

An LP was performed on our patient but no opening pressure was noted. This would have been helpful and may have suggested the diagnosis, as opening pressures are elevated (>200 mm of water) in three-fourths of patients with cryptococcal meningitis and AIDS. In fact, the increased intracranial pressure not infrequently causes cranial nerve palsies and visual impairment and is the main determinant of outcome.

An easy diagnostic trick is to check a serum cryptococcal antigen test, positive in about 95% of cases. This can be used to screen patients for cryptococcal disease with- out having to do a lumbar puncture.
 
Summary of Case
During the repeated visits, it seems that the history and physical exam were changing to fit his previous diagnosis of sinusitis without concerted efforts to look for other causes of headache. Pain worse when bending over (mentioned on the second visit) suggested the possibility of increased intracranial pressure, though this can also occur with sinusitis. Red flags included fever and the fact that he was of African descent (first mentioned by his doctor on his fourth ED visit).

The onset of this patient’s cryptococcal meningitis was insidious, as was his AIDS. It was only through repeat visits and good thinking that the diagnosis was found. There are some clues on history and physical exam such as fatigue, fevers, lymphadenopathy, oral thrush, and seborrheic dermatitis, which may be suggestive of immunosuppression due to HIV/AIDS, but from examining our patients charts, it is difficult to say if these processes were occurring. The correct diagnosis was eventually made and the patient was appropriately treated, but his outcome could have been far different.
 
REFERENCES

  • Sklar DP, Crandall CS, Loeliger E, et Unanticipated death after discharge home from the emergency department. Ann Emerg Med. 2007;49:735-745.
  1. Croskerry, Achieving quality in clinical decision making: Cognitive strategies and de- tection of bias. Acad Emerg Med. 2002;9:1184-1204.
  2. Snow V, Mottur-Pilson C, Hickner JM, et Principles of appropriate antibiotic use for acute sinusitis in adults. Ann Intern Med. 2001;134:495-497.
  3. American College of Emergency Clinical Policy: Critical issues in the eval- uation and management of adult patients presenting to the emergency department with acute headache. June 24, 2008. Available at: www.acep.org/workarea/download- asset.aspx?id=8802. Accessed Sept. 15, 2008.
  4. Centers for Disease Control and Notice to readers: Approval of a new rapid test for HIV antibody. MMWR. 2002;51:1051.
  5. Powderly Cryptococcosis. In: AIDS Therapy. Dolin R, Masur H, Saag M, eds. New York, NY: Churchill Livingstone, 1999:400-411.
  6. van der Horst CM, Saag MS, Cloud GG, et Treatment of cryptococcal meningitis as- sociated with the acquired immunodeficiency syndrome. N Engl J Med. 1997;337:15-21.

 

The Case of a 37-Year-Old Man with Headaches
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