Advanced treatment, close to home
Neurological Surgery, P.C. (NSPC) offers the latest diagnosis, management and treatment of Neuro-Ophthalmology Diseases. Our physicians provide compassion combined with knowledge and experience.
- Acute Vision Loss
- Bell’s Palsy
- Blepharitis and Dry Eye Syndrome
- Blepharospasm and Hemifacial Spasm
- Double Vision (Diplopia)
- Drusen and Pseudopapilledema
- Idiopathic Intracranial Hypertension / (AKA Pseudotumor Cerebri) and Papilledema
- Micro-vascular and Other / Cranial Nerve Palsies
- Ocular & Systemic Myasthenia Gravis
- Optic Neuritis
- Pituitary Tumors
- Pupillary Irregularities: Anisocoria, Horner’s Syndrome, Third Nerve Palsy & Adie’s Pupils
- Thyroid Eye Disease
- Trigeminal Neuralgia
- Tumors of the Visual Pathways
- Visual Field Disturbances
1. Acute Vision Loss:
Ischemic Optic Neuropathy and Giant Cell Arteritis (AKA Temporal Arteritis)
Unfortunately, sudden vision loss is a common presenting complaint in the neuro-ophthalmologist’s office. The causes are varied. Ischemic optic neuropathy is a common cause and is best thought of as a stroke of the optic nerve. Risk factors are similar for stroke and include smoking, atherosclerotic disease, diabetes, hypertension and obstructive sleep apnea. Giant cell arteritis (GCA, also known as temporal arteritis), is an inflammatory condition (a vasculitis) that causes lack of blood flow to various facial structures including the eye and optic nerves. If left untreated, it can progress to complete blindness in both eyes. Patients suspected of having this disease are often treated with high doses of corticosteroids while undergoing evaluation, which will likely include a biopsy of the temporal artery.
Transient vision loss may be a prelude to GCA, but may also be from occlusion of retinal arteries (known as central or branch retinal artery occlusion). If the artery spontaneously opens, it is referred to as transient monocular blindness (also known as amaurosis fugax). If the artery remains occluded, the result is an infarct of the retina termed central or branch retinal artery occlusion. Patients require imaging of their carotids arteries and a cardiac evaluation.Other causes of transient visual changes need to be considered and these include migraine, idiopathic intracranial hypertension and other processes that are considered given the presenting clinical scenario.
2. Bell’s Palsy
Bell’s Palsy is caused by dysfunction of the seventh cranial nerve, the facial nerve. The facial nerve is the major motor nerve serving facial muscles. Patients experience paralysis of the muscles served by the facial nerve, which is usually isolated to one side of the face. Additional symptoms may include a change in taste, an alteration of hearing and pain or discomfort in the jaw or ear area.
The exact cause of Bell’s Palsy is still uncertain, but many believe it is due to viral related inflammation of the facial nerve. Alternatively an immune inflammatory process has been proposed. Secondary causes may be due to Lyme infection, sarcoidosis, tumors or traumatic injuries. These are evaluated for depending on the characteristics of each individual case. The prognosis for significant recovery of function is good in most cases. Treatment is usually with a combination of anti-viral medications and oral steroids for a brief duration.
Eye care is especially important in Bell’s Palsy and may be the most important aspect of treatment. Due to incomplete closure of the eyelids, patients are at high risk for corneal injury. Patching of the affected eye during sleep, and use of lubricating eye drops and ointments are of extreme importance. Should recovery of muscles responsible for eye closure be incomplete, surgical interventions may be necessary for prevention of chronic corneal injury.
3. Blepharitis and Dry Eye Syndrome
Blepharitis, or inflammation of the eye lids and dry eyes are common findings in the neuro-ophthalmologist office. Frequently patients will complain of eye pain, discomfort or strain, or they may have blurred or otherwise abnormal vision that may be intermittent. Examination and testing of tear production will be conducted to make this diagnosis. Treatment will vary depending on the extent of the exam findings and the symptoms and patients will be given a “regimen” that includes lubricating artificial tears. Significant relief is usually attained.
4. Blepharospasm and Hemifacial Spasm
Hemifacial spasm is the abnormal contraction in muscles on one side of the face. It typically affects the muscles around the eyes initially and then spreads to lower portions of the face. In between muscle contractions the affected muscles may be weak. Hemifacial spasm is usually thought to be secondary to compression of the facial nerve by nearby blood vessels. However compression of the facial nerve by tumors or aneurysms must be considered in the appropriate situations.
Blepharospasm, or more correctly Benign Essential Belpharospasm (BEB), is the condition of abnormal contraction of muscles of eye closure and affects both eyes. This causes uncontrolled closure of the eyes which interferes with vision (although vision is otherwise normal). It is possible that this is due to a dysfunction of coordinated movement of the muscles of eye closure.
Botulinum toxin, know commonly as Botox, has been approved for the treatment of hemifacial spasm and blepharospasm. Essentially this bacterial toxin blocks transmission of electrical signals from nerve to muscle thereby decreasing contraction of muscles. Injections are given intramuscularly around the eyes, the brow and occasionally the lower face. The exact location of injections varies between patients depending on the symptoms and locations of spasm. Improvement of symptoms is generally seen within two weeks and may last approximately three months with eventual need for re-injection. Side effects of botulinum toxin include, but are not limited to, drooping of the eyelid, tearing, double vision and weakness of the muscles around the mouth causing slurred speech. As with the therapeutic effects of botulinum toxin, these side effects are generally self limited and “wear off.” Surgery can be considered for cases of Hemifacial spasm refractory to conservative management.
5. Double Vision (Diplopia)
Double vision is a common presenting symptom in the neuro-ophthalmologist’s office and has numerous causes. Ocular muscle diseases, thyroid eye disease, nerve palsies (see below) and disorders of neuro-muscular transmission (myasthenia gravis) are some of the causes. Occasionally diplopia can be caused by a decompensation of a slight congenital misalignment of the eyes. Tumors and aneurysms can also be a cause of double vision and need to be considered and evaluated when appropriate. When the double vision is secondary to an underlying process, such as myasthenia gravis or aneurysm, treatment is focused on correcting those processes. In some circumstances, such as micro-vascular disease the diplopia may resolve on its own. In other cases patients may require medications or the addition of a prism to the patient’s glasses. Occasionally surgery is warranted.
6. Drusen and Pseudopapilledema
Optic disc drusen is the condition of abnormal accumulation of protein-like material within the optic disc which then calcifies. These are usually asymptomatic and benign and are found on routine exam. Occasionally they cause mild visual field abnormalities. Neuro-ophthalmologists are frequently consulted to determine if drusen are present or if optic disc edema is present. The term pseudopapilledema is used for conditions that appear to be edema but on closer evaluation are either drusen or a variation of the normal configuration of the optic discs (termed anomalous optic disc/nerves). CAT scanning and ultrasound can be helpful in the determination of the presence of optic disc drusen. Fundus photography can be used in the initial and follow up visits for patients with pseudopapilledema to ensure no changes or progression that would indicate another ongoing process.
7. Idiopathic Intracranial Hypertension (AKA Pseudotumor Cerebri) and Papilledema
Idiopathic intracranial hypertension (IIH) is the syndrome of symptoms caused by elevated intracranial pressure not due to secondary causes such as tumors or blood clots within the brain. Common presenting symptoms are headache, blurred vision, transient episodes of vision loss or blackening and “rushing” or hearing the heartbeat in the ears (known as pulsatile tinnitus). Evaluation includes a thorough neuro-ophthalmic exam, with the usual finding of papilledema (optic disc edema due to elevated intracranial pressure). Evaluation includes an MRI and MRV (magnetic resonance venogram to look at the veins of the brain, obtained simultaneously with the MRI). After imaging, a lumbar puncture is done to document elevated spinal fluid pressure and to ensure the constituents of the spinal fluid are otherwise normal. Visual field analysis is also of great importance. Treatment is usually with oral medications but aggressive and progressive cases (despite medical therapy) sometimes require surgery.
8. Micro-vascular and Other Cranial Nerve Palsies
Micro-vascular disease of the cranial nerves that control eye movement can cause double vision with or without a drooping or closure of the eyelid. This is best thought of as a small stroke to the nerve that controls the eye muscles. Risk factors are similar to those that cause small vessel strokes in the brain and risk factor modification is important. Fortunately there is a good prognosis for full recovery over several months.
There are several diseases of a systemic nature that cause cranial nerve palsies and these are evaluated for as appropriate based on the clinical history and demographics of each individual patient. Additionally, infectious causes, such as Lyme disease, are evaluated for as appropriate.
Migraine is a common cause of disability and decreased quality of life. Migraine and other forms of headache are commonly associated with visual symptoms. It is important for your neuro-ophthalmologist to determine that your visual symptoms are related to migraine and not other forms of abnormalities of the eyes and central nervous system. Visually, patients may experience positive visual phenomena (such as wavy or zigzag lines, shimmering, sparkles etc). Less commonly patients may briefly lose vision in a small area of the visual field. Fortunately there are numerous medications that are effective in controlling the frequency and severity of migraine headaches. Referral to a neurologist who specializes in migraine and headache treatments may be beneficial.
10. Ocular & Systemic Myasthenia Gravis
Myasthenia gravis is an immune disorder whereby the communication between the nerve and muscles is not functioning properly. The body mistakenly produces antibodies to the receptor for acetylcholine, the neurotransmitter signal released by the nerves. Due to the paucity of acetylcholine receptors on the muscle, muscular contraction is less effective and this leads to weakness.
Ocular involvement is a common presenting symptom of generalized myasthenia gravis and is the major feature of ocular myasthenia gravis. Symptoms include double vision due to variable weakness and fatigue of the ocular muscles as well as drooping of the eyelids (known as ptosis). Patients must also be evaluated and asked about more generalized symptoms such as difficulty speaking, swallowing and breathing, as well as weakness of the arms or legs; more generalized symptoms create difficulties with maintenance of patient airway passages.
Evaluation of myasthenia gravis patients includes a detailed exam by your neuro-ophthalmologist to rule other causes of double vision and ptosis, laboratory studies and possible electrophysiological studies. Treatment is usually initiated with medications to decrease the metabolism of the acetylcholine neurotransmitter and is for the most part well tolerated. Occasionally patients have only a partial or no response to these medications and alternatives therapies under the guidance of a neuro-muscular neurologist is required.
11. Optic Neuritis
The optic nerves transfer visual information from the retina to the brain. Optic neuritis is an inflammation of the optic nerves and is caused by a variety of processes. Symptoms include blurred and decreased vision in one eye, to varying degrees. Color vision may also be affected and patients may have visual field deficits. Pain with eye movement is also a common presenting symptom. Optic neuritis is commonly a symptom of multiple sclerosis and is often the initial presenting symptom. However optic neuritis may also occur as an isolated event, or as part of rheumatic and connective tissue disorders. Usual evaluation includes MRI and laboratory studies. Treatment depends on the given clinical scenario and usually includes intravenous corticosteroids.
12. Pituitary Tumors
Tumors of the pituitary gland are common and usually benign in nature. The close proximity of the pituitary gland to the visual pathways, specifically the optic nerves and chiasm, causes visual dysfunction to be one of the more common presenting symptoms of such tumors. Visual symptoms can be non-specific and may even be found incidentally on routine visual testing, without prior knowledge by the patient. Classically, symptoms include decreased peripheral vision to both sides. However unilateral decreased vision or abnormal visual field is possible as well. Double vision may also occur less commonly. Visual field testing, especially automated visual fields, are of great importance. Visual prognosis can now be made with optical coherence tomography, a painless, noninvasive imaging test. Laboratory studies for hormonal levels are part of the evaluation as well. Depending on the hormonal status of the tumor and its size and impact on vision, treatment is with medication, surgical intervention, or radiation.
13. Pupillary Irregularities: Anisocoria, Horner’s Syndrome, Third Nerve Palsy & Adie’s Pupils
Abnormal pupil reactivity and unequal pupil size are a frequent reason for evaluation in the neuro-ophthalmologist’s office. Unequal size of the pupils have numerous causes and careful evaluation is needed to determine if the process is benign without need for further concern or if additional investigation is needed to rule out ongoing disease. Pupil size is controlled by several muscles which themselves are controlled by the sympathetic and parasympathetic portions of the nervous system.
Physiologic anisocoria, (meaning pupils of unequal size) is a mild asymmetry of the pupils not indicative of any disease process. Once it is determined that the inequality is physiologic, no further investigation is needed.
Adie’s or tonic pupils can occur in one or both eyes and usually affects women more commonly than men. Patients may be asymptomatic or have photophobia (light sensitivity) or difficulty focusing on close objects. The affected pupil is initially larger with poor reactivity and eventually becomes the smaller pupil. Symptoms, if present, usually resolve completely over time without the need for specific treatment. The cause is an abnormality in the “relay station” of the parasympathetic nerves of unclear etiology. Your neuro-ophthalmologist will likely use special eye drops called pilocarpine to prove that the diagnosis is indeed Adie’s pupils.
Horner’s Syndrome is a disruption in the sympathetic innervation controlling the pupil size. There are numerous areas along the course of the sympathetic nerve pathways where this disruption can occur. Special eye drops will be used by your neuro-ophthalmologist to prove the presence of this abnormality. A thorough investigation, including evaluation of the carotid arteries for dissection (a tear in the arterial wall) and radiological imaging of the chest for masses is needed.
14. Thyroid Eye Disease
Thyroid dysfunction, especially in patients with Grave’s disease, can present with ocular and visual manifestations during the course of the disease, even once thyroid hormone levels are returned to normal. Symptoms are mainly due to enlargement of the extra-ocular muscles and orbital (vascular) congestion. Patients may experience protrusion of the globes (called exophthalmus), double vision and symptoms related to orbital congestion and dry eyes. In some cases, compression of the optic nerve by the enlarged muscles can cause progressive vision loss if not treated. Treatment for thyroid eye disease is tailored specifically for each patient and the extent of their symptoms. In conjunction with the endocrinologist, thyroid function is returned to normal. In some cases, steroids, radiation therapy, and other interventions are needed. Double vision is treated with prisms but may eventually require surgical intervention in certain cases. Surgery may also be needed for incomplete closure of the eye lids that does not fully resolve.
15. Trigeminal Neuralgia
The trigeminal nerve, the fifth of the twelve cranial nerves that provide nervous control of the head and neck, is the major sensory nerve of facial structures. Trigeminal neuralgia (TN) is the condition whereby the patients experience excruciating, “electric shock” type pain in an area served by the trigeminal nerve. Trigeminal neuralgia is usually caused by compression of the trigeminal nerve by a nearby blood vessel. In younger patients TN may be part of a demyelinating event associated with multiple sclerosis. Masses or tumors causing compression of the trigeminal nerve also have to be considered. Occasionally, there is no obvious cause that can be found.
The management of trigeminal neuralgia includes use of medications and surgical interventions, sometimes in combination. At Neurological Surgery P.C. we have the ability to use all methods, including the most advanced surgical techniques, to help alleviate our patient’s pain.
16. Tumors of the Visual Pathways
As noted, the visual pathways course over a wide distribution of space in the central nervous system. Many areas of the visual system are juxtaposed to other types of tissue, including bone, meninges (the multi-layered soft tissue covering of the brain and spinal cord), blood vessels and other types of brain tissue as well. Thus masses and tumors of these structures, both benign and malignant, can affect the visual system due to these anatomical relationships. Additionally, tumors may arise from the substance of the optic nerves and chiasm themselves or their meningeal coverings. Examples of these include optic nerve gliomas and meningiomas.
Evaluation of these tumors includes neuro-imaging, such as CT scans and MRI. Occasionally biopsy is necessary. Treatment depends on the type of tumor and its location. Surgery, radiation therapy and chemotherapy may be part of the treatment. At Neurological Surgery P.C. we have the capability for accurate diagnosis and employ a multi-disciplinary treatment team, including neuro-ophthalmology, neuro-oncology and neurological surgery. Additionally state of the art treatment such as Cyberknife and Gamma Knife are available.
17. Visual Field Disturbances
Visual field loss can occur from numerous etiologies including vascular (ischemic optic neuropathy and giant cell arteritis), inflammatory (optic neuritis) and compressive causes. Compressive cases most commonly involve tumors arising along the visual pathway or adjacent to it. For example, pituitary tumors commonly present with abnormal peripheral visual fields. Severe inflammation occurring in the orbit can also cause visual field abnormalities. Optic disc edema of various etiologies including papilledema (from elevated intracranial pressure in idiopathic intracranial hypertension) is another cause of visual field loss. Additionally numerous diseases of the retina can cause visual filed abnormalities and these need to be addressed as well. When visual field loss is found, evaluation focuses on determining the etiology and initiating treatment. Additionally state of the art treatments such as Cyberknife and Gamma Knife are available.
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