Complex Ophthalmoplegia

History Taking

Chief Complaint:

• Double vision

• Difficulty moving eyes

• Drooping eyelids

• Vertigo or balance issues

History of Presenting Complaint:

• The onset, duration, and progression of symptoms, such as sudden onset of diplopia or gradual difficulty in moving eyes in certain directions

• Eye pain or discomfort, particularly when looking in different directions

• Changes in vision, such as blurring, distortion, or loss of vision

• Associated symptoms such as ptosis (drooping eyelid), proptosis (bulging eye), or changes in the size of the pupils

• Symptoms may worsen with fatigue or towards the end of the day

System Review:

• Neurological symptoms such as weakness, numbness, tingling, unsteady gait, difficulty swallowing, slurred speech, or confusion

• Symptoms of thyroid dysfunction such as weight changes, heat or cold intolerance, palpitations, anxiety, or changes in bowel movements, hair, or skin texture (Graves' disease)

• Symptoms of muscle weakness or fatigue, particularly if worse after exercise or improving with rest (myasthenia gravis)

• Signs of infection such as fever, chills, malaise, headaches, neck stiffness, or rash (infectious causes)

Past Medical History:

• History of neurological disorders like multiple sclerosis, Guillain-Barré syndrome, or stroke

• History of autoimmune conditions, such as Graves' disease or myasthenia gravis

• History of metabolic disorders, such as diabetes

• History of infections such as Lyme disease or syphilis

Past Surgical History:

• History of eye or orbital surgery, brain surgery, or other neurosurgery that might affect the cranial nerves

• History of thyroid surgery or radioiodine treatment (Graves' disease)

Drug History:

• Medications that can cause ophthalmoplegia, such as corticosteroids, antiepileptics, antipsychotics, or statins

• Medications for autoimmune diseases, such as immunosuppressants or corticosteroids

• Medications for diabetes, such as insulin or oral hypoglycemics

Family History:

• Family history of neurological disorders, autoimmune diseases, or eye disorders

• Family history of myasthenia gravis, Graves' disease, or other autoimmune diseases

• Family history of hereditary disorders that can cause ophthalmoplegia, such as mitochondrial myopathies or oculopharyngeal muscular dystrophy

Personal History:

• Smoking and alcohol consumption, which can impact overall health and contribute to disorders associated with ophthalmoplegia

• Any specific hobbies or occupations involving exposure to toxins or radiation

• Stress level, as stress can exacerbate symptoms of many disorders, including autoimmune diseases

Social History:

• Living conditions, such as access to clean water and sanitation (relevant for infectious causes)

• Potential exposure to ticks or other vectors of infectious disease

• Level of physical activity, as sedentary lifestyle could contribute to metabolic conditions like diabetes

Travel History:

• Travel to areas with high prevalence of tick-borne diseases such as Lyme disease

• Travel to areas with endemic infectious diseases that can cause ophthalmoplegia

Physical Examination

General Examination:

• Observe the patient's general condition: level of comfort, hygiene, orientation, and overall demeanor

• Notice any obvious dysarthria or dysphagia which may indicate a neurological cause

• Look for signs of weight loss, cachexia or muscle wasting

Hands & Arms:

• Observe for any tremor, muscle weakness or muscle wasting

• Check muscle tone, power, and reflexes

Face:

• Eyes: Look for ptosis, squint, or nystagmus. Observe eye movements in all directions, looking for any restriction or lack of coordination

• Pupil examination for size, symmetry, and reaction to light

• Notice any facial asymmetry, drooping, or muscle weakness that could indicate a neurological disorder

• Observe for signs of thyroid disease such as lid lag or exophthalmos

Neck:

• Look for any scars from previous surgeries, especially thyroid surgery

• Examine thyroid gland for size, symmetry, nodules or tenderness

Cardiac Examination:

• Auscultate for any abnormal heart sounds, which could be indicative of conditions like myotonic dystrophy

Respiratory Examination:

• Auscultation of the lungs to assess breath sounds. Decreased breath sounds may be present in patients with muscle weakness affecting the diaphragm

Abdominal Examination:

• Palpate for hepatosplenomegaly which may suggest a systemic cause

Neurological Examination:

• Cranial nerve examination, particularly III, IV and VI which control eye movements, and VII for facial symmetry

• Motor examination for any muscle weakness, particularly proximal muscles. Check for muscle tone, power, bulk, and reflexes

• Sensory examination to identify any areas of altered sensation

• Coordination and gait examination

Additional Examination (If required):

• Examine the skin for any rashes or lesions that could be associated with conditions causing ophthalmoplegia, such as Lyme disease or sarcoidosis

• Fundoscopy to assess for papilledema or other retinal changes

• Bedside testing for diplopia using cover-uncover test and testing of visual fields

Investigations

Laboratory Tests:

• Complete Blood Count: to identify any systemic infection or anaemia.

• Thyroid Function Tests: to rule out thyroid disease, such as Graves'.

• Autoantibodies: Anti-acetylcholine receptor antibodies for myasthenia gravis, or anti-TSH receptor antibodies for Graves' disease.

• Glucose and HbA1C: to identify diabetes mellitus which may cause mononeuritis multiplex.

• Creatine kinase: elevated in myopathies.

• Lyme disease serology: if history or geography suggests possible exposure.

• HIV and syphilis serology: as these can cause ophthalmoplegia.

Imaging:

• MRI of brain and orbits: to visualize any lesions, inflammation, or masses that might be affecting the cranial nerves or ocular muscles.

• CT scan: might be used if MRI is contraindicated.

• Chest X-ray or CT: if considering conditions such as thymoma in myasthenia gravis or sarcoidosis.

Invasive:

• Lumbar puncture: to check for signs of infection or inflammation in the central nervous system, useful if considering conditions like Guillain-Barré syndrome.

• Muscle or nerve biopsy: to diagnose certain myopathies or neuropathies.

Other Tests:

• Electrodiagnostic studies (EMG/NCS): can be useful in diagnosing myasthenia gravis or neuropathies.

• Tensilon (Edrophonium) test: can help diagnose myasthenia gravis.

• Ocular ultrasound: if considering a structural issue within the eye.

• Visual field testing: if there's concern for involvement beyond the ocular muscles.

• Pulmonary function tests: If considering conditions that could cause muscle weakness, such as myasthenia gravis.

Management

General Management:

• Patient education: Explanation of the condition, prognosis, and management options.

• Eye care: Regular check-ups, use of lubricating eye drops if required, use of glasses/prisms for diplopia.

• Multidisciplinary approach: Coordination with ophthalmologists, neurologists, endocrinologists as required.

• Lifestyle modifications: Quitting smoking, maintaining a balanced diet, and regular exercise.

Medical Management:

• Corticosteroids: For inflammatory or autoimmune conditions such as myasthenia gravis or Guillain-Barré syndrome.

• Anticholinesterase medications: For myasthenia gravis, drugs such as pyridostigmine.

• Immunosuppressants: For autoimmune conditions, such as myasthenia gravis, if unresponsive to first-line therapy.

• Antibiotics or antivirals: For infections such as Lyme disease, syphilis or HIV.

• Thyroid medications: For thyroid-related ophthalmoplegia, drugs like carbimazole or propylthiouracil.

• Control of blood glucose: In cases of diabetic mononeuritis multiplex.

Surgical Management:

• Decompression surgery: For thyroid eye disease causing severe ophthalmoplegia.

• Thymectomy: Considered for certain cases of myasthenia gravis.

• Neurosurgical interventions: For cases caused by tumors or vascular malformations.

Other Management:

• Physiotherapy: Can help maintain muscle strength and flexibility, beneficial in cases of muscle weakness.

• Occupational therapy: Assistance with adaptations to daily living, if required due to visual disturbance.

• Regular follow-ups: To monitor the progress of the condition and adjust treatment as necessary.

Causes of Complex Ophthalmoplegia

Neurological Conditions:

• Stroke, particularly brainstem strokes

• Multiple sclerosis

Autoimmune Diseases:

• Myasthenia gravis

• Graves' disease (Thyroid Eye Disease)

Metabolic and Mitochondrial Disorders:

• Diabetes

• Thyroid disorders

Structural or Mechanical Causes:

• Trauma or injury to the eye or orbit

• Orbital cellulitis

Vascular Conditions:

• Giant cell arteritis