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Horner's syndrome

Horner's syndrome is a clinical syndrome caused by damage to the sympathetic nervous system. more...

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Symptoms

It results in ptosis (drooping upper eyelid), miosis (constricted pupil), and occasionally enophthalmos (the impression that the eye is sunk in) and anhidrosis (decreased sweating) on one side of the face.

In children Horner's syndrome sometimes leads to a difference in eye color between the two eyes (heterochromia). This happens because a lack of sympathetic stimulation in childhood interferes with melanin pigmentation of the melanocytes in the superficial stroma of the iris.

History

It is named after Dr Johann Friedrich Horner (1831-1886), the Swiss ophthalmologist who first described the syndrome in 1869. Several others had previously described cases, but "Horner's syndrome" is most prevalent. In France, Claude Bernard is also eponymised with the condition being called "syndrome Bernard-Horner".

Causes

Horner's syndrome is usually acquired but may also be congenital. Although most causes are relatively benign, Horner's syndrome may reflect serious pathology in the neck or chest (such as a Pancoast tumor) and hence requires workup.

Horner's Syndrome is due to a deficiency of sympathetic activity. The site of lesion to the sympathetic outflow is on the ipsilateral side that the symptoms are on. The following are examples of conditions that cause the clinical appearance of Horner's Syndrome:

  • First-order neuron disorder: Central lesions that involve the hypothalamospinal pathway (e.g. transection of the cervical spinal cord).
  • Second-order neuron disorder: Preganglionic lesions (e.g. compression of the sympathetic chain by a lung tumor).
  • Third-order neuron disorder: Postganglionic lesions at the level of the internal carotid artery (e.g. a tumor in the cavernous sinus).

Diagnosis

Three tests are useful in confirming the presence and severity of Horner's syndrome:

  1. Cocaine test - Cocaine blocks the reuptake of norepinephrine resulting in the dilation of a normal pupil. The pupil will fail to dilate in Horner's syndrome.
  2. Paredrine test
  3. Dilation lag test

Read more at Wikipedia.org


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Diary: from a week in practice
From American Family Physician, 3/15/05 by Paul Gross

Monday

I never trust a simple diagnosis, especially in a complicated patient. Seventy-five-year-old Esmeralda Gonzalez, who usually sees a colleague of mine, visits me today because of a problem that just can't wait. I know Mrs. Gonzalez. She's blind and has diabetes, hypertension, and renal insufficiency, plus an impressive collection of symptoms and laboratory abnormalities that could keep any family physician--plus a few subspecialists--happily occupied for years. Today it's something new. "Diarrhea," she says, "for about a month." Her history is tantalizingly specific: The diarrhea only happens in the morning, when she has about five loose stools after breakfast. The rest of the day she's fine. What does she have for breakfast? "Toast," she says, "and a glass of milk." Milk? "Do you drink milk at any other time of the day?" I ask. "No." Is she sure? "No, just in the morning." It's too good to be true. I enter a tentative diagnosis of "lactose intolerance" in the chart and suggest that Mrs. Gonzalez refrain from drinking milk for a couple of weeks while monitoring her symptoms. Experience tells me that this solution won't work--either the history will change by the next visit or the intervention will turn out to be useless--but still, it's nice to fantasize that I've finally cured Mrs. Gonzalez of something.

Tuesday

The management of warfarin (Coumadin) drives me crazy. Am I alone? Take Mr. Santos, a 79-year-old with a-trial fibrillation whose warfarin dosing is complicated by the fact that he can't read and that we don't run International Normalized Ratios (INRs) in our office. Mr. Santos was therapeutic on 5 mg until he visited the Dominican Republic and returned with hematuria and an INR of 5.2. I adjusted his dose down to 4 mg and watched his INR trail off to 1.98, then to 1.7. Despite energetic coaching about some missed doses, his INR continued to slide. When it reached 1.36, I brought him into the office last Tuesday to make an adjustment. "Take one-and-a-half pills on Monday and Friday," I told Mr. Santos, "and one pill the other nights." When Mr. Santos nodded, I quizzed him ("How many pills will you take tonight ...? And tomorrow night ...? And Fri-day ...?") until I felt comfortable sending him home. But first, I drew another INR--just in case. Imagine my surprise when Mr. Santos returns today and I discover: (1) a lab report showing that his INR last Tuesday, the day I increased his dose, had jumped to a therapeutic 2.22; and (2) that he's taking "one-and-a- half pills every night, just like you told me!" I grab a fistful of my hair. "Let's go back to ONE pill every night. UNA pastilla. Cada noche!" Do I recheck his INR today? No, I'm afraid to look.

Wednesday

Phyllis Martin's appearance gives me an unpleasant, fluttery sensation. I'm not sure what she has, but I'm fairly certain that it's bad--and Mrs. Martin already has her share of troubles. This 55-year-old woman has already lost a daughter to suicide and a son to lawlessness; she and her husband make meager incomes between two part-time jobs and are behind on the rent; and she has no insurance to pay for medical visits or for blood pressure and diabetes medicines. "It's my left eye," Mrs. Martin says. "For a month I've been seeing two." But this isn't a simple diabetic extraocular muscle palsy. No, Mrs. Martin's face is markedly asymmetric with ptosis of her left eyelid. I consider a differential: Cerebrovascular accident? Bell's Palsy? Horner's syndrome? Her extraocular movements are normal, her cranial nerves are intact, and she has no facial droop. But there's puffiness below the eyebrow. When I ask her to squeeze her eyes shut, instead of the eyelids closing tight, the left eyelid bulges dramatically, as if something were displacing the globe outward. My flutters worsen as I imagine a tumor in her orbit. I call our chief of radiology to arrange for an urgent MRI. When I mention her lack of insurance, he doesn't hesitate. "She needs to have it done; send her up."

Thursday

Today, I get a call about Phyllis Martin's MRI. "There's no mass," our chief of radiology says, "just diffuse swelling in the left orbit--possibly an inflammatory pseudotumor." No mass? No mass! I giddily do an Internet search for "inflammatory pseudotumor" and learn that it typically presents with a red eye (which she does not have), that its cause is uncertain, and that it's often treated with steroids. When Mrs. Martin returns for follow-up, I feel like dancing around her. "Good news!" I say. "No cancer. Just some swelling that an eye doctor will help us figure out." Instead of smiling, Phyllis tears up. "It's good news!" I repeat, not comprehending her sadness. "What's the matter?" She fixes me with a watery gaze. "Are you sure you're telling me everything?" I put my hand on her shoulder and assume my gravest doctor voice: "If you had cancer, I wouldn't keep it from you." Finally, her expression relaxes and I go on. "But here's a really important question: What about the MRI bill?" I've learned that her scan may generate a charge of several thousand dollars, an amount I could imagine triggering a heart attack. She can't even afford a $20 prescription. "I don't know," she sighs, as I make myself a note to look into it. For now, I'm grateful for this reprieve, for a bullet dodged, for this whispered promise of life.

Friday

I'm touched by patient correspondence. The written word suggests that extra time and thought were given. There's also something intimate about reading letters; it's like peering down on someone's inner landscape. In the case of 76-year- old Eustace Conrad, his reason for writing is more prosaic: he hates the telephone. So, I routinely get notes from him- -punctuated with the exclamation "Ha!"--printed on lined yellow pages torn from a spiral pad. Here's one from 1998: "Hey! How are you and the family doing? Me, okay. Pressure still around 145/88. Paris was great. How many 70-year-old patients of yours still think and act like teenagers? The only problem is, this one can't perform like a teenager. Ha! Speaking of my rocket booster pill--Ha!--I need a prescription." He also sent a photo he'd snapped of the Eiffel Tower. It's now propped in my office. Where to put the letter--in his chart? In the garbage? I tucked it into a drawer at home--among Christmas cards and birth-day notes from my daughters--an indication of my soft spot for this lively, unique patient. A more recent communique came after an elevated prostate-specific antigen level (and a family history of prostate cancer) prompted a referral to a urologist. The message reads: "Hey! Young Dr. Poppa. Just wanted to let you know that the 'finger doctor' said everything looked good. Ha!" Another classic to add to my drawer. Ha!

Saturday/Sunday

With the end-of-year deadline less than two weeks away, I finally visit the American Board of Family Medicine Web site (http://www.abfp.org) to tackle my first Self-Assessment Module (SAM) and Clinical Simulation, yearly online tests that I now need to pass to maintain certification. Beginning in 2003, each "class" of Board candidates has taken on, in addition to the certifying examination, this requirement that's now staring me in the face. I choose a module on hypertension, a subject I've been teaching to medical students for years. Over several days I return to the site many times, taking bold stabs at daunting questions until I've finished. My score, calculated instantly, is 47 percent, not the ego boost I'd imagined--more like ice cubes down the back (passing is 80 percent). That's the bad news. The good news is that I'm immediately invited to revisit missed questions, receive online coaching, and try again. By completing this process, I now can modestly boast that I passed my SAM with a perfect score. The Clinical Simulation is more straightforward, although the program reproaches me when I forget to offer my computer-generated patient any explanation whatsoever of his medical condition. (Might it be because this humanoid doesn't seem quite ... lifelike?) In any event, I've completed the assignment with days to spare and have learned my lesson. This year, my procrastinating will begin much, much earlier.

For the past 13 years, Dr. Paul Gross has been on the residency faculty of New York Medical College at St. Joseph's in Yonkers, New York, a city of 196,000. He divides his time between patient care, resident supervision, teaching, and life with his own family--a wife and two daughters.

Address correspondence to Paul Gross, M.D. (e-mail: pgross@pol.net).

To preserve patient confidentiality, the patients' names and identifying characteristics have been changed in each scenario.

COPYRIGHT 2005 American Academy of Family Physicians
COPYRIGHT 2005 Gale Group

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