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Horner’s Syndrome Secondary to Mycobacterium Avium Complex Infection
Abstract
Clinical examination of a 65-year old female demonstrates a left Horner’s syndrome. Chest CT
reveals a large cavitary lesion of the left lung apex secondary to a Mycobacterium avium
complex infection.
Case History
 65 year-old female presents to the neuro-ophthalmic disease department for evaluation of
ptosis and anisocoria
 She reports a ptosis of her left eyelid for about 9 months, but did not seek care due to
personal issues at home. The ptosis has been stable since the onset.
 Systemic history is remarkable for a recent diagnosis of a Mycobacterium avuim complex
(MAC) lung infection attributed to Adalimumab (Humira® AbbVie Inc., North Chicago
IL) use, Crohn’s disease, asthma and hypertension.
 Ocular history is remarkable for a laser peripheral iridotomy OU secondary to narrow
anatomical angles, pre-surgical cataracts OU and compound hyperopic astigmatism with
presbyopia OU.
 Current medications include azithromycin, ethambutolol, rifampin, hydrochlorothizide,
and albuterol.
 She was previously being treated with Adalimumab (Humira® AbbVie Inc., North
Chicago IL) for Crohn’s disease, but this was stopped after she was diagnosed with the
MAC infection.
 She is currently under the care of pulmonology
 She is a current every day smoker for the past 40 years
Pertinent Exam Findings
 Best-corrected visual acuities:
o OD: 20/20-1, OS: 20/20
 Extraocular motilites:
o Full and smooth OU
 Confrontation fields:
o Full to finger count OU
 Palpebral apertures
o OD: 11mm, OS: 9mm
 Pupillary testing:
o Bright illumination
 OD: 3.25mm, OS: 2.75mm
o Dim illumination
 OD: 4.25mm, OS: 3.25mm
o (-)RAPD
 Diagnostic Pupil Testing with 0.5% Apraclonidine (images will be included)
o After 30 minutes
 Bright illumination
 OD: 3.00mm, OS: 3.00mm
 Dim illumination
 OD: 4.25mm, OS: 3.75mm


o After 1 hour
 Bright illumination
 OD: 3.0mm, OS: 3.75mm
 Dim illumination
 OD: 3.75mm, OS: 4.5mm
 Reversal of anisocoria confirms left Horner’s Syndrome
Anterior Segment
o Patent LPI OU
o 2+ nuclear sclerosis, 1+ anterior cortical spoking OU
Posterior Segment
o Optic nerve: 0.45/0.45 perfused, healthy, distinct OU
o Macula: flat and intact OU
o Vasculature: normal course and caliber OU
Differential Diagnosis:
 Horner’s syndrome: damage along the long course of the sympathetic nerve from the
hypothalamus to the eye
o Inflammation at the lung apex secondary to diagnosis of MAC infection versus
mycobacterium tuberculosis
 History of weight loss, chronic productive cough and arm/shoulder pain
o Pancoast tumor
 History of arm/shoulder pain
 History of smoking
o Trauma to the head/neck
o Osteoarthritis of the neck with bony spurs
o Aortic aneurysm
o Stroke
o Space-occupying mass
o Multiple sclerosis
o Vasculopathic
o Cluster headache
o Carotid cavernous fistula
o Carotid artery dissection
o Idiopathic
 Argyll-Robertson
 Ciliary spasm
 Myasthenia gravis
 Partial cranial nerve III palsy
 Dermatochalasis
 Congenital ptosis
Additional Testing:
 Bloodwork:
o Elevated glucose (135 mg/dL)
o Elevated globulin (3.8 g/dL)
o Elevated RDW (15.2%)
o Elevated platelet count (408 thousand/uL)

o Elevated absolute neutrophils (8127 cells/uL)
Chest CT: (images will be included)
o Large cavitary lesion of left lung apex. Left parahilar mass or adenopathy.
Interstitial nodular disease bilaterally (left greater than right).
Diagnosis and Discussion:
 Based on the location of the lung lesion on the CT scan, the left-sided Horner’s syndrome
can be confirmed to be secondary to the MAC infection of the left lung.
 A Horner’s syndrome is classically defined as a triad of ipsilateral ptosis, pupillary miosis
and anhydrosis.
 A Horner’s syndrome can be confirmed with diagnostic pupil testing in office with 0.5%
or 1.0% topical apraclonidine. It is important that no other topical medications are
instilled prior to the testing as it is a measure of suprasensitivity
 Apraclonidine is a direct alpha α2 against with weak α1 activity, which is why it does not
dilate a normal eye. In an eye with a Horner’s, there is an up-regulation of α1 receptors,
causing dilation, resulting in a reversal of the anisocoria.
 The clinical signs of a Horner’s syndrome occur due to interruption of the sympathetic
nerve along its long course from the hypothalamus to the eye.
 The sympathetic nerve originates in the hypothalamus and descends through the reticular
formation in the brainstem to the ciliospinal center of budge in the spinal cord. The preganglionic fibers then exit the spinal cord and pass over the apex of the lung to enter the
sympathetic chain in the neck to synapse in the superior cervical ganglion. Here, arise the
post-ganglionic axons, which travel with the carotid artery up the neck and into the
cranium to eventually form the long and short posterior ciliary nerves to the eye.
 Since pre-ganglionic fibers cross over the apex of the lung, any mass or infection here
can cause damage.
 Mycobacterium avium complex (MAC) is a non-tuberculous mycobacterial (NTM)
pulmonary infection with increasing incidence worldwide.
 Although the disease is most commonly seen in association with immunosuppressive
diseases like HIV, the increasing therapeutic use of tumor necrosis factor-alpha (TNF-α)
medications has been associated with an increase in NTM infections.
 The clinical symptoms of the infection include a chronic or recurrent cough with
expectoration of mucoid sputum, generalized weakness, malaise, low energy, low-grade
fevers and weight loss.
 A chest CT in patients with active MAC infections commonly demonstrates fibrocavitary
lesions involving the upper lobe of the lung, which can predispose a patient to a Horner’s
syndrome.
 To the best of our knowledge, this is the first case report of a Horner’s syndrome
secondary to a Mycobacterium avium complex infection.
Treatment and Management
 When a Horner’s syndrome is suspected, a thorough history can help localize the
underlying pathology.
 If the patient is asymptomatic, a normal work-up will include neuro-imaging of the entire
course of the sympathetic nerve including an MRI/MRA of the head and neck and CT
scan of the chest.
 Treatment varies based on the underlying pathology found.


In this case, the treatment includes a 1-year course of azithromycin 250mg, ethambutolol
400mg and rifampin 300mg
We are interested to see if the Horner’s syndrome resolves with treatment of the MAC
infection, as this has been demonstrated in patients with Mycobacterium tuberculosis
infections
Conclusion
 If a Horner’s syndrome is suspected, pharmacologic testing with 0.5% or 1.0%
apraclonidine should be performed to confirm the diagnosis prior to proceeding with an
extensive work-up.
 The course of the sympathetic nerve is long, so a targeted history should be obtained to
tailor the neuro-imaging towards the underlying pathology.
 It is important to consider all patients on anti-TNF-α as immunocompromised since these
medications increase their risk of secondary infections, including tuberculosis.
 Although Mycobacterium tuberculosis infections must always be ruled out in patients
with a chronic cough, malaise, weight-loss and a Horner’s syndrome, a non-tuberculous
infection, like MAC, may also be the culprit.
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