grave's opthalmopathy

05/03/2023UNIVERSITY OF MISSOURI HEALTHCARE 1

Dr. Naresh Mullaguri, MDResident Physician PGY2

Department of Neurology

University of Missouri

A CASE OF PAINFUL OPTHALMOPLEGIA


DISCLOSURES• None


HISTORY OF PRESENT ILLNESS• Chief complaint: New onset headache for 5 days

• History of present illness:

48 year old African American female with history of Hypothyroidism and Hypertension otherwise in good health presented to the university ER with the new onset headache for the past 5 days which is located on the Right temporal region and was about 10/10 in intensity, started slowly and gradually progressed. Pain is throbbing with occasional sharp character over the right temple with no radiation. Sleep and IBUPROFEN make the pain better and looking towards the right makes the pain worse. No changes with position, coughing or bearing down, diurnal variations or with chewing. The headache is also associated with light sensitivity and occasional sonophobia, Kinesophobia. She also complained of double vision when looking to the right side associated with pain with the false image on the outer side of the original image.

Her eyes have been red, right-sided greater than left side since the same day when the headache started initially. Complained of gritty sensation, dryness.

Negative history: she denied any fever, chills, neck stiffness, passing out spells, lateralized weakness or numbness. Denied any stress. No fatigue, dizziness, lightheadedness, loss of appetite, weight or recent weight gain. No recent URI symptoms or dental infections.


HISTORY CONTINUED…

• PMH/PSH: Hypertension, Hypothyroidism S/p Thyroidectomy in 1981 for Grave’s Disease

• Family History: Father had MI and died of Stroke

• Paternal grandfather with Seizure, No family history of Migraines or AVMs

• Strong family history of Grave’s disease with affected Grand Mother, Mother and three sisters

• Social History: Works at Woodhaven NH and cares for mentally disabled

• Smoking currently 1Pack per 2-3 days, restarted recently but never a heavy smoker

• Denied EtOH and illicit drug use.

• No known drug allergies


MEDICATIONS• Atenolol for Hypertension

• Levothyroxine for Hypothyroidism

• Review of systems:

• Generalized weakness

• Hot flashes with regular menstrual cycles


PHYSICAL EXAMINATION• Vital signs:

• Temperature: 36.8*C

• Heart rate: 79bpm

• Respiratory rate: 16 breaths/min

• Blood pressure: 151/96 mm of Hg


PHYSICAL EXAM CONTINUED…• General: African-American female appears to be at stated age lying hunched over in hospital gown in

emergency department gurney with lights off and an ice pack on head. Answered questions with soft voice.

• HENT: normocephalic head. No tenderness over sinuses, mastoid region. No tenderness on Palpation over the right scalp region and said it is helping her pain.

• Eye exam: bilateral proptosis, R>L with bilateral conjunctivae injection with no visible corneal, Iris, anterior chamber lesions. No lid lag. No pallor or icterus noticed. Fundoscopic exam showed clear disc margins with no signs of papilledema.

• Neck: No signs of rigidity or tenderness in the occipital groove. No carotid bruits. Transverse linear scar of thyroidectomy in the lower portion of the neck.

• Cardiovascular: Regular rate and rhythm with no murmurs. Peripheral pulses are normal and equal.

• Respiratory: Clear to auscultation bilaterally

• Abdominal: soft, no tenderness, No visceromegaly

• Extremities: No cyanosis, clubbing or pedal edema.

• Skin: No rashes, pretibial myxedema

• Psychiatric: Normal affect and mood.


NEUROLOGICAL EXAM• Higher mental functions: Alert, awake and oriented X 4, in mild distress from the headache.

Memory is intact to recent and remote memories as she is able to narrate her family events and important dates in her life. Comprehension is intact. Speech is fluent with no dysarthria.

• Cranial nerves:

• Optic Nerve: visual acuity is normal. Color vision is normal. Pupils 42mm with both direct and consensual light reflex with no relative afferent pupillary defect.

• Occulomotor, Abducens and Trochlear: Extra ocular movements were intact except for decreased abduction to the right side and associated with pain. Mild to moderate lid retraction, No nystagmus.

• Trigeminal: No facial sensory loss. Jaw jerk is with in normal limits, Medial and lateral pterygoids were strong.

• Facial: no facial asymmetry, orbicularis oculi is strong bilaterally, able to whistle

• Vestibulocochlear: No vertigo, hearing is normal to finger rub bilaterally

• Glossopharyngeal and Vagus: uvula is in midline and elevates symmetrically. Strong voice.

• Spinal accessory: Shoulder shrug is 5/5 and sternocleidomastoids 5/5 bilaterally

• Hypoglossal: Tongue protrudes to midline with no atrophy


OPTHALMOLOGY FINDINGS• Visual acuity 20/25- in right eye and 20/30+ in left eye, Mild to moderate proptosis, IOP is 14 in right eye and 13

in left eye.

• Hertels: 25mm in the right eye and 23mm in the left eye.

• Ishihara color plates: 15/15 OU

• Mild lid retraction

• Sclera/Conjunctiva: 1-2+ injection OD>OS near muscle insertions

• Cornea: No edema, few scattered Superficial punctate keratitis OU, moderate dryness OU with decreased Tear break up time

• Anterior chamber: Deep and quiet OU

• Lens: Clear OU

• Vitreous: Clear OU

• Fundus exam:

Disk: crisp margins 0.4C:D OU

Vessels: appear healthy : Macula: Flat and healthy OU : Peripheral : attached and healthy OUCourtesy: Dr. Wilson from Ophthalmology.


NEUROLOGICAL EXAM CONTINUED…

• Motor system Exam: Bulk is equal bilaterally. Tone is with in normal limits in both the flexors and extensors in bilateral upper and lower extremities.

• Strength is 5/5 in bilateral upper and lower extremities. No pronator drift.

• Reflexes: 2+/4 in bilateral biceps, brachioradialis, Knees and ankles. Plantars were down going bilaterally.

• Sensory system examination: intact to light touch, pin prick and vibration in all the extremities. Romberg’s sign is negative.

• Gait and Station: Normal

• Coordination: intact to finger to nose test bilaterally.


DIFFERENTIAL DIAGNOSIS

• Grave’s Opthalmopathy

• Retro orbital tumor/Hematoma

• Cavernous sinus thrombosis

• Migraine

• Vasculitis(Giant cell arteritis)


LABS• CBC:

• Hb: 13g/dl

• HCT: 38.8%

• WBC: 6.2

• PLT: 328

• CMP:

• Na: 137

• K+: 2.7

• Cl: 92

• CO2: 31

• Glucose: 145

• BUN: 10

• Creatinine: 0.71

• Calcium: 6.1

• AST: 17; ALT: 17; ALP: 51

• TSH: 2.5

• Free T4: 1.29


LABS CONTINUED…

• Ionized calcium: 0.69(1.12-1.3)

• Magnesium: 1.6(1.7-2.3)

• 25 OH Vit-D: 54

• Intact PTH: 5 (ref: 15-65)


STRUCTURES IN ORBIT


CT of the Head showing bilateral basal ganglion calcifications and enlarged Right medial rectus


RADIOLOGY

CT Head:

• Scattered hyperdense foci visualized in bilateral basal ganglia and subcortical white matter of the bilateral occipital lobes and within cerebellum. Right rectus muscle is slightly more prominent. Findings consistent with Fahr’s disease.

MRI of the Brain:

• Bilateral symmetrical calcification in bilateral basal ganglia, thalami, cerebellar dentate nuclei suggestive of Fahr’s disease.

• Right sided proptosis with enlargement and increased enhancement of the inferior rectus and medial rectus. Increased enhancement of the left superior oblique muscle. Primary consideration is Thyroid orbitopathy / Pseudotumor / Myositis.


Enlarged medial rectus


Contrast

enhancement in

the right Medial

rectus and

Inferior rectus


Diffuse calcifications involving all the basal ganglia

FAHR’S DISEASE SEC. TO SECONDARY HYPOPARATHYROIDISM


GRAVE’S OPTHALMOPATHY• Incidence of 2.9-16.0 cases per 100,000 per year

• Female preponderance.

• Smoking increases the risk of developing thyroid eye disease by seven to eight fold.

• Anterior displacement of the globe by the edematous extra ocular muscles and orbital fibro fatty tissues results in exophthalmos and lower lid retraction. These symptoms in turn may lead to impaired lid closure and corneal ulceration, especially if the levator muscle is also infiltrated and its excursion restricted.

• Edematous extra ocular muscles lose compliance, restrict eye movements, and can compress the optic nerve at the orbital apex

• Common early symptoms include altered periocular appearance, symptoms related to the eye surface (grittiness, photophobia, and excessive lacrimation), double vision, especially at the extremes of gaze, and retro- orbital ache.

• Rundle’s curve is a fundamental concept in understanding and managing thyroid eye disease, and it determines choice of treatment.


Pathophysiology of Grave’s opthalmopathy: Role of Orbital fibroblast


Atypical eye features requiring confirmation of the diagnosis of thyroid eye disease by orbital imaging (CT or MRI)

• Unilateral disease

• Unilateral or bilateral disease in patients with no previous or present evidence of thyroid dysfunction

• Absence of upper eyelid retraction

• Divergent strabismus

• Diplopia sole manifestation

• History of diplopia worsening towards the end of the day

Other ophthalmological conditions that can be misdiagnosed as thyroid eye disease

• Allergic conjunctivitis

• Myasthenia gravis

• Orbital myositis

• Chronic progressive external ophthalmoplegia

• Orbital tumors (primary or secondary)

• Carotid-cavernous fistula

• Any inflammatory orbitopathy (such as Wegener’s granulomatosis)


MANAGEMENT• Once the diagnosis of thyroid eye disease is made, initial management consists of three

steps, in this order of priority:

• Ensuring that the patient has neither of the sight threatening eye complications: corneal ulceration or optic neuropathy during which high dose IV Steroid pulse therapy salvages the optic Neuropathy.

• Avoiding factors that exacerbate thyroid eye disease by introducing smoking cessation strategies (if the patient is a smoker), correcting thyroid dysfunction, and protecting the corneas with lubricants.

• Referring suitable patients to specialist centers based on European Group on Graves’ Orbitopathy (EUGOGO)


PREVENTION

• Smoking, uncontrolled thyroid dysfunction, and radioiodine therapy are risk factors for development or exacerbation of thyroid eye disease.

• In the absence of clinical evidence of thyroid eye disease, radioiodine can be used safely in patients with Graves’ disease. Expert advice should be sought before use of radioiodine in patients with established thyroid eye disease. Passive smoking may be a risk factor for development of thyroid eye disease in childhood.

Petros Perros, consultant endocrinologist1, Christopher Neoh, consultant ophthalmologist2, Jane Dickinson, consultant ophthalmologist2 1Department of Endocrinology, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP 2Department of Ophthalmology, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP


FAHR’S DISEASE• Fahr, a German pathologist from Hamburg, described in 1930 an 81-year-old patient with

a long history of dementia who presented to the hospital with fever and immobility and, later, died. Examination of the brain revealed evidence of calcification in the striatum. Although the description of bilateral basal ganglia calcification has previously been described, Fahr's name has become associated with all forms of bilateral calcification in the basal ganglia and other parts of the brain. Fahr's disease refers to idiopathic bilateral calcification of the basal ganglia. These symmetrical calcifications of the strio-pallido-dentate system are detectable in 0.7-1.2% of routine cerebral computed tomography (CT)-scan and may be caused by different conditions. A review of the literature revealed that one-third of patients with bilateral strio-pallido-dentate calcinosis (BSPDC) were clinically asymptomatic, while the most common symptom was movement disorder.


My sincere thanks toDr. Shenker, MD, Ph.D

Dr. Shah, Syed, MD

Dr. Wilson, Phil, MD

Dr. Buzzola, Rino, MD

grave's opthalmopathy

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