What causes this strange symptom in older women?

What caused this 75-year-old woman to have ptosis in her left eye, headache for 3 days, jaw pain, and recent weight loss? That’s the diagnostic challenge faced by Janet Stowers, MD, of the University of California, San Francisco, and colleagues, who report this case in JAMA Neurology.

The patient presented with horizontal binocular diplopia, ptosis on the right side, and headache, which she noticed had worsened since starting 3 days ago. She also told the clinician that she found it difficult to open her mouth, that it was painful to chew, and that she had recently lost about 2.3 kilograms (about 5.1 pounds).

A week ago she had a drooping left eyelid; it lasted for 2 days but it resolved. Her vision was otherwise normal, she had no pain in her eyes, and she had no scalp tenderness or muscle pain. The patient’s medical history included schizophrenia, hypothyroidism, and supraventricular tachycardia.

Physical examination revealed normal vision in both eyes. Her pupils are the same size, round, and respond normally to light. Ptosis affected her right upper eyelid, and extraocular motor tests showed bilateral elevation and limited abduction.

Dilated fundus examination results were normal, as were other neurological examination results. Fatigue, rest, or ice pack testing had no effect on the patient’s ability to move the eye or ptosis.

Laboratory tests showed elevated levels of both C-reactive proteins and erythrocyte sedimentation rate – 292 mg/L (normal

Contrast brain MRI showed diffuse edema and enhancement of the scalp, skull base, neck, and paravertebral soft tissue, T2 hyperintensity and enhancement of the extraocular muscles in both eyes.

Diagnostic Considerations

Stallworth and Co. The authors considered several possible diagnoses, including anti-transfer RNA synthase myositis, immunoglobulin G4-related disorders, myasthenia gravis, and giant cell arteritis (GCA).

First, given that the inflammation and loss of muscle tissue normally associated with aminoacyl transfer RNA synthetases does not typically affect facial and extraocular muscles, and patients have no evidence of interstitial lung disease, nonerosive arthritis, or Raynaud Phenomena are usually associated with anti-synthetase syndromed The diagnosis is unlikely.

Another possibility: Although enlarged extraocular muscles may reduce eye movements in immunoglobulin (Ig) G4-related disease, the patient did not have hypertrophic dura and Signs of sclerosing lesions

Likewise, myasthenia gravis was dismissed because the patient was absent. Fatigue, ice pack test results were normal, and MRI showed soft tissue changes.

A final investigation helped the clinician make the diagnosis: they did a biopsy of the patient’s temporal artery and found signs of intimal hyperplasia and transmural inflammation with giant cells Indicates GCA. The authors note that while large and medium arteries are commonly affected by GCA, recent definitions suggest that vessels of any size may be affected.

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with GCA eye Patients who present typically present with acute visual loss, 80% of which are optic neuropathy associated with posterior ciliary artery ischemia due to arteritis anterior ischemia, Stallworth and coauthors wrote.

Data suggest that approximately 50% of GCA patients experience visual symptoms during the course of the disease, while 14-20% have permanent vision loss, although it is worth noting that this can be Diagnosis and treatment to prevent.

Among GCA symptoms, symptoms commonly reported as initial and overall symptoms include headache (33% of patients and 72% of overall symptoms), concomitant polymyalgia rheumatica, Including neck, trunk, shoulder and pelvic girdle pain (early symptoms in 25% of patients, 58%), fatigue and discomfort (20% and 56%), jaw claudication (4% and 40%), and fever (11%) and 35%).

5-15% of GCA patients report diplopia, but only a small percentage of patients have significant oculomotor nerve damage – “suggesting that the ophthalmoplegia may be transient , and underreported,” said Stallworth and co-authors.

They explain two pathophysiological mechanisms of ophthalmoplegia in GCA: arteritis with ocular motor neurovascular involvement and arteritis with vascular supply to the extraocular muscles.

The case authors add that GCA-related loss of eye movement does not tend to reflect patterns of cranial nerves. Regarding arteritis affecting the blood supply to the extraocular muscles, “extraocular muscle ischemia was observed despite abundant anastomotic vessels in both the ophthalmic and external carotid branches,” the authors wrote.

They point to a case of GCA with severe ophthalmoplegia in which histopathological evaluation of the entire oculomotor organ revealed insufficiency of the extraocular muscles while sparing the entire eye of the 3rd, 4th and 6th cranial nerves.

The ptosis associated with ophthalmoplegia in GCA may be caused by a similar mechanism, the research team suggests. “It has also been reported that ptosis is associated with Horner’s syndrome or direct granulomas of sympathetic fibers involved in vasculitis secondary to neurovasculitis affecting the supplying sympathetic plexus, as they run along the inflamed vessel wall of the internal cavernous carotid artery. run.”

Stallworth and co-authors noted that the oculomotor disturbances and ptosis observed in this patient did not fit the pattern of the oculomotor nerves, and there was no evidence of Horner’s syndrome. other signs. Examination did not reveal any ataxia or hemiparesis, and MRI findings ruled out brainstem infarction.

The patient’s presentation was most suggestive of vasculitis, resulting in blood flow to the periorbital muscle and prompt treatment with high-dose intravenous steroids led to symptom improvement within hours, the authors said.

“This case demonstrates the multifaceted presentation of GCA and a patient who maintains a high suspicion of elevated inflammatory markers, headache, and less common neuro-ophthalmic symptoms,” the team concluded.

– Control of eye movements not suggestive of typical oculomotor nerve palsy, ischemic myopathy should be considered, and corticosteroids should be started immediately to prevent vision loss and complications of GCA.