Neuro-ophthalmology is a subspecialty that deals with diseases of the nervous system which manifest in the eyes. Symptoms of relevance include: unexplained loss of visual acuity, loss of peripheral (side) vision, pupillary abnormalities, double vision, and twitching of facial and eyelid muscles. Because of the difficulty of diagnosing these symptoms, it is recommended that patients seek specialized help from a neuro-ophthalmologist to receive the most effective treatment. Our neuro-ophthalmologists are board-certified and are fellows of the North American Neuro-Ophthalmology Society (NANOS).
The conditions we treat (to name a few):
Optic Neuritis (including multiple sclerosis)
A Inflammatory optic nerve diseases can be associated with multiple sclerosis (2, 3) but other systemic inflammatory diseases (3, 4) may be the cause of a patient’s visual loss. An accurate diagnosis may require a systemic immunological evaluation. Treatment to improve vision and reduce the advancement to MS must be considered.
Post Traumatic Visual Disorders
A person who has suffered a traumatic brain injury (TBI) or cerebral vascular accident (CVA) may often experience difficulties with balance, spatial orientation, coordination, cognitive function, and speech. Persons with a TBI or CVA will frequently experience symptoms of double vision, movement of print or stationary objects such as walls and floor, eye strain, visual fatigue, headaches and problems with balance, among other symptoms.
Unexplained Facial and Eye Pain
Trigeminal neuralgia (TGN) is a poorly understood condition which causes severe intermittent facial pain in the distribution of the nerve supplying sensation to the face, the trigeminal nerve. Four in every 100,000 people will develop the condition each year. It occurs more commonly on the right side (60% of cases) and rarely on both sides (1%).
It is more common after the age of 50 and it is almost twice as common in women as men.
Papilledema (including pseudotumor cerebri)
Swollen optic nerves, pseudotumor cerebri: Patients with swollen optic nerves (papilledema) can have increased intracranial pressure which causes blurred or lost vision, double vision, and headaches. In young, overweight women, the increased pressure develops without a tumor, but they can still lose vision. The diagnosis must be correctly established, eliminating lesions and dural venous sinus diseases as the cause, and treatment must be individualized. We consider pseudotumor cerebri (idiopathic intracranial hypertension) as a significant women’s health care issue that we are treating and investigating (6). Also, we can determine whether the optic disc elevation is not indicative of a significant or neurological problem.
Ischemic Optic Neuropathy (including temporal arteritis)
Ischemic optic neuropathy is a condition that usually presents with sudden onset of unilaterally reduced vision. Most patients have no associated discomfort. The condition is the result of decreased blood flow to the optic nerve (ischemia). There are two basic types: arteritic and non-arteritic ischemic optic neuropathy.
Compressive Optic Neuropathy
Orbital injuries commonly accompany facial trauma, necessitating knowledge of the spectrum of potential injures. Acute compressive optic neuropathy (ACON) is a rare but treatable complication of increased pressure within confined orbital spaces. Recognition and prompt initiation of treatment may prevent blindness.
Pituitary and Other Brain Tumors
Pituitary tumors are abnormal growths found in the pituitary gland, a small organ–about the size of a dime and located in the center of the brain–which makes hormones that affect growth and the functions of other glands in the body. Most pituitary tumors are benign – which means that they are non-cancerous – and grow slowly, not spreading to other parts of the body. A pituitary tumor may cause the pituitary gland to produce too many hormones, which can cause other problems in the body. Tumors that make hormones are called functioning tumors, while those that do not make hormones are called non-functioning tumors. Certain pituitary tumors cause Cushing’s disease in which fat builds up in the face, back and chest, and the arms and legs to become very thin. Other pituitary tumors can cause acromegaly, a condition in which the hands, feet and face are larger than normal. Another type of tumor can cause breasts to make milk even though there is no pregnancy. Symptoms of pituitary tumors may include headaches, vision problems, nausea and vomiting, or any of the problems caused by the production of too many hormones such as infertility or loss of menstrual periods in women, abnormal growth, high blood pressure, heat or cold intolerance, and other skin and body changes.
Cerebrovascular Disease (Stroke) Involving Vision
Cerebrovascular disease, or stroke, refers to brain disorders caused when the blood supply to the brain is disrupted in some way. The most common form of stroke is caused by a blockage of a blood vessel in the brain. This leads to a portion of the brain dying. A blockage may be short lived and produce symptoms lasting less than 24 hours. This is called a transient ischaemic attack (TIA). About one out of every five strokes is due to bleeding from a blood vessel. These strokes are often more severe. In half of these cases, bleeding occurs within the brain tissue (cerebral haemorrhage). In the other half, the bleeding starts within the coverings of the brain (subarachnoid haemorrhage).
Thyroid Eye Disease
Thyroid Eye Disease is an autoimmune eye condition that, while separate from thyroid disease, is often seen in conjunction with Graves’ Disease. The condition, however, is seen in people with no other evidence of thyroid dysfunction, and occasionally in patients who have Hashimoto’s Disease. Most thyroid patients, however, will not develop thyroid eye disease, and if so, only a mild case.
Most often, an acquired disorder that results in fatiguable muscle weakness, made worse by activity and improved with rest. It results from an autoimmune attack against the nerve-muscle junction. Other forms of the illness are caused by architectural changes of the nerve-muscle junction.
Ocular Motor Nerve Disorders
Diseases of the oculomotor nerve or nucleus that result in weakness or paralysis of the superior rectus, inferior rectus, medial rectus, inferior oblique, or levator palpebrae muscles, or impaired parasympathetic innervation to the pupil. With a complete oculomotor palsy, the eyelid will be paralyzed, the eye will be in an abducted and inferior position, and the pupil will be markedly dilated. Commonly associated conditions include neoplasms, craniocerebral trauma, ischemia (especially in association with diabetes mellitus), and aneurysmal compression.
Gaze Palsies and Nystagmus
The eyes remain conjugate; however, the patient cannot gaze in a specific direction or directions (right, left, up or down). Nystagmus is a rhythmical repetitive eye movement. Jerk nystagmus: This is by far the commonest variety. The nystagmus has a slow phase, where the eyes drift off the fixation point, and a fast corrective saccadic phase bringing the eyes back on target.
Facial Movement Disorders (Blepharospasm, Hemifacial Spasm)
Essential blepharospasm is a debilitating disorder of progressive involuntary spasms of the eyelid muscles (orbicularis oculi). Other muscles in the face or neck may be involved as well. Terms that sometimes are used in the description of this problem include dystonia (impaired or disordered muscle tone) and dyskinesia (a defect in voluntary movement). The onset of blepharospasm is typically in the fifth to sixth decade and is more common in women than in men. Although the cause of essential blepharospasm is incompletely understood, it is thought to be a result of “miscommunication” in the area(s) of the brain responsible for control of the involved muscles.
Double vision typically but does not always arise from a misalignment between the eyes. It may simply occur from a decompensation of a previously known or unknown strabismus, but in most cases it is a symptoms of neurological disease. Some of these disorders are transient or resolve spontaneously while others deteriorate because of serious illness, such as tumor, multiple sclerosis, aneurysms, and systemic diseases. Ocular myasthenia gravis often requires expertise to definitively diagnose and outline a treatment plan which normalizes vision with few side effects (7). Menigioma, Pituitary Tumor, Aneurysm, and Craniopharyngioma Meningioma (8), pituitary tumor (9, 10), aneurysm (11), and craniopharyngioma (12) can cause visual loss from optic pathway compression or double vision. Some cases will need surgery, others some type of radiotherapy (8) and others only require optical therapy and expectant follow up unless there is deterioration. This can be particularly difficult during pregnancy (13).