Neuro-Ophthalmic Disease

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Jasmine Bradley
8 years ago
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1 Neuro-Ophthalmic Disease Dr. Carlo J. Pelino Assistant Professor The Eye Institute The Pennsylvania College of Optometry Philadelphia PA To provide useful clinical information in the diagnosis and treatment of neurological disorders Retina has 10 layers: Optic nerve- Intraocular, Intraorbital, Intracanilicular, Intracranial Optic Chiasm Nasal fibers cross, Temporal fibers do not Optic tract - Contains retinal fibers from both eyes Optic Radiations -Travel through the Temporal, Parietal and Occipital Visual Cortex - Termination of all of the nerve fibers. Found in the Occipital lobe Visual field loss secondary to retina and optic nerve disease = usually occur monocular Examples: ARMD, Glaucoma, Optic Neuritis or Tumors Case History: Reports 3 week history of blurred vision OD Notices especially when reading Right-sided weakness Visual acuities 20/20 (Right) 20/20 (Left) PERRL (trace +) RAPD right eye Confrontation fields: right homonymous hemianopia denser superiorly Medical history : Hypertension / pipe smoker Bow Tie Optic Atrophy: Optic tract lesion Ipsilateral ST/IT pallor Contralateral band pallor (temporal VF defect) From nasal macular fibers (papillomacular bundle) May have small RAPD in contralateral eye

disorders Retina has 10 layers: Optic nerve- Intraocular, Intraorbital, Intracanilicular, Intracranial Optic Chiasm Nasal fibers cross, Temporal fibers do not Optic tract - Contains retinal fibers

2 Incongruous homonymous hemianopia Internal Capsule cortical spinal tract and spinothalamic / medial lemniscus tract Tumors of the CNS: Incidence is 15 per 100,000 for intracranial tumors In Adults 50% are primary brain tumors In Adults 50% are metastatic brain tumors In children, nearly all brain tumors are primary Adult Tumors The most common primary CNS neoplasm in adults: Astrocytomas = comprise 80% of all CNS tumors 1. Pilocytic Astrocytomas childhood and cerebellar Fibrillary Astrocytomas (25 years old) Anaplastic Astrocytoma Glioblastoma Multiforme most aggressive Case History: 5 yrs ago, C/D was 0.3 x 0.3 both eyes 3 years ago C/D ratio increased with normal IOP Due to findings, optometrist spoke with PCP, and PCP ordered MRI of brain and orbits with and without contrast MRI reported to be normal HVF has shown only mild progression Pt is now noticing a blurry spot in OS Denies any new symptoms Pt no longer has insurance Bi-Temporal Field Loss: Pituitary Adenoma 55%

3 Craniopharyngioma 25% Meningioma 10% Glioma 7% Arachnoid Cyst Cavernous sinus Contents Brain Tumor The greatest concern for most headache patients Patients with primary or metastatic brain tumors have a headache at the time of diagnosis (~30%) Severe headache pain worse in the morning, nausea and vomiting = seen in 20% of patients Most often the headaches are intermittent, dull ache, unilateral and mild initially but usually occur daily Headache occurs in 70% of brain tumor patients The most common primary brain tumor is an astrocytoma Brain tumor headaches usually do not disrupt sleep The headache worsens with a change in body position, coughing, straining LYMPHOCYTIC CHORIOMENINGITIS Rodent-born viral infectious disease Primary host is a common house mouse 5% of mice in the US carry LCMV Person to person transmission hasn t been reported Meninges Arachnoid, Pia, Dura mater Types: Bacterial Viral

unilateral and mild initially but usually occur daily Headache occurs in 70% of brain tumor patients The most common primary brain tumor is an astrocytoma Brain tumor headaches usually do not disrupt

4 Meningitis Fungal Parasitic Carcinoma Will cause pleocytosis = increase in white blood cells in the CSF Acute = Hours to days Chronic = 4 weeks or more Aseptic meningitis = No CSF bacteria found (example:enterovirus) Viral Meningitis Herpes Virus meningitis is diagnosed more frequently than before because of better techniques. PCR HSV meningitis is more common than HZV meningitis CSF work up through PCR (polymerase chain reaction) Treatment: IV Acyclovir Q 8 hrs x 14 days Bacterial Meningitis ~ 30,000 new cases diagnosed each year in the United States Strepto pneumoniae (pneumococcus)=most frequent etiology Neisseria meningococcus Haemophilus influenzae Treatment: IV ampicillin, cephalosporin, chloramphenicol Patient Information: Comprehensive eye examination 16 y/o AA female Chief complaint D > N, cc Headaches Temporal, infrequent/long-standing, Tylenol

PCR HSV meningitis is more common than HZV meningitis CSF work up through PCR (polymerase chain reaction) Treatment: IV Acyclovir Q 8 hrs x 14 days Bacterial Meningitis ~ 30,000 new cases diagnosed

5 Denies diplopia, eye pain/strain, photophobia, flashes, floaters, LOV, difficulty reading Ocular history Spectacles, 2 y/o Last CEE ~ 2 years TEI Other causes of Meningitis Lyme Syphilis TB Listeria Amoeba (swimming) West Nile Virus Causes of Roth Spot s Leukemia Bacterial endocarditis Anemia Diabetes Sickle cell disease Scurvy Connective tissue disorders (SLE) Lumbar puncture/spinal fluid analysis Opening pressure: 440mm H20 (<250 mmh20) Protein CSF: 12 mg/dl (L) (15-45 mg/dl) Routine chemistry Glucose 137 mg/dl (H) ( mg/dl) Fasting plasma glucose >126 mg/dl Diabetes Non-fasting glucose >200 mg/dl Diabetes CBC RBC: 4.44 ( ) WBC: 7.2 ( ) Platelet: 209 ( ) Hematocrit: 37.2 ( ) Hemoglobin: 12.5 g/dl ( ) Restoration of visual acuity and resolution of papilledema

fluid analysis Opening pressure: 440mm H20 (<250 mmh20) Protein CSF: 12 mg/dl (L) (15-45 mg/dl) Routine chemistry Glucose 137 mg/dl (H) (70-105 mg/dl) Fasting plasma glucose >126 mg/dl Diabetes

6 Weight loss ~6-10% reduction in body weight Medication Acetazolamide 500mg tablets PO BID Gradually increased up to 500mg QID if tolerated Side effects Topiramate, Furosemide Surgical procedures Therapeutic lumbar puncture Optic nerve head sheath fenestration Neurosurgical shunting procedure Modified Dandy s criteria - Pseudo Tumor Cerebri The patient is awake and alert Patient has signs and symptoms of increased ICP (nausea, vomiting, headache and disc edema) Absence of neurological signs except for CN 6 palsy Normal neuro-imaging (MRI, MRV, CT must be normal and done before the LP) Cerebro-spinal pressure must be greater than 200 mm H2O (average opening LP pressure in PTC patient is ) (CSF must be normal in composition) Modified Dandy s criteria - Pseudo Tumor Cerebri The patient is awake and alert Patient has signs and symptoms of increased ICP (nausea, vomiting, headache and disc edema) Absence of neurological signs except for CN 6 palsy Normal neuro-imaging (MRI, MRV, CT must be normal and done before the LP) Cerebro-spinal pressure must be greater than 200 mm H2O (average opening LP pressure in PTC patient is ) (CSF must be normal in composition) CN 3 Palsy Complete pupil involvement Send to ER. 20% die within 48 hrs Relative Pupil involvement Pupil spared

symptoms of increased ICP (nausea, vomiting, headache and disc edema) Absence of neurological signs except for CN 6 palsy Normal neuro-imaging (MRI, MRV, CT must be normal and done before the LP)

7 Presentation: Ocular: Ptosis Levator muscle is affected Limited ocular motility Complete or relative sparing of the pupil Cranial Nerve 3 palsy Pupil involved ophthalmoplegia Symptoms: Vertical and horizontal diplopia Pain (aneurysm).variable with diabetic 3rd Acute = aneurysm or ischemic vascular Progressive = infiltrative or compressive Treatment: Referral to Neuro-Ophthalmologist immediately Possible MRI / MRA / CT Catheter Cerebral angiography if aneurysm suspected ESR if Giant Cell Arteritis is suspected 90% of aneurysmal 3rd N palsies have the pupil affected. 50% of patients diagnosed with aneurysmal third-nerve palsies have a subarachnoid hemorrhage within 2 weeks. 50% of those patients who hemorrhage will die. Catheter Cerebral Angiography is the best imaging tool, however, it will cause stroke or myocardial infarction in 1% - 2% of patients The lack of anisocoria in an isolated third-nerve palsy does not exclude an aneurysm or compressive lesion. Malignant Hypertension Headache is dull or sometimes pounding HA is cardinal feature of hypertensive encephalopathy Systemic Findings: Extremely high blood pressure >210/120 Possible change in mental status Seizures may occur

variable with diabetic 3rd Acute = aneurysm or ischemic vascular Progressive = infiltrative or compressive Treatment: Referral to Neuro-Ophthalmologist immediately Possible MRI / MRA / CT Catheter

8 Treatment: Urgent admission to an ER / PCP for slow lowering of the BP Grade 1 Retinal vessels narrowed > 90 and < 110 Diastolic BP Grade 2 Nicking of retinal vessels > 90 and <110 Diastolic BP Grade 3 CWS, Hemes, Lipid exudates > Diastolic BP Grade 4 Grade 3 + Optic disc swelling > 130 Diastolic BP Always question the malignant hypertensive patient Hypertensive encephalopathy Syncope Seizures Focal weakness Speech problems Hypertensive Cardiac involvement Chest pain Palpatations Cough Dyspnea Hypertensive renal problems Change in renal volume Hematuria, abdominal pain Organ dysfunction uncommon if DBP is less than 120 mmhg Types of Optic Neuritis: Retrobulbar optic neuritis Papillitis Perioptic neuritis Is idiopathic optic neuritis Multiple Sclerosis???? ~ 50% of MS patients get optic neuritis

Seizures Focal weakness Speech problems Hypertensive Cardiac involvement Chest pain Palpatations Cough Dyspnea Hypertensive renal problems Change in renal volume Hematuria, abdominal pain Organ

9 MS is an autoimmune inflammatory cascade Oligodendrocytes (make myelin CNS can not fight a virus) White matter dysfunction loss of myelin Disease disseminated both in time and space The risk for MS increases further lives from the equator Optic Neuritis Profile Female ( 77% ) Male ( 23% ) Caucasian ( 85% ) African American ( 15% ) severe, worse visual prognosis Age: years old ( median age is ~ 32 years old ) Nadir of visual acuity is ~ 5 days Improvement in ~ 3 weeks with final recovery at 3 months Fellow eye is involved ~ 20% of the time. Pain occurs in 92% of cases If Multiple Sclerosis is suspected: MRI (the strongest predictor of MS) - try obtain within 1 wk ONTT correlated the # of brain lesions with the risk of MS 0 lesions = 16 % risk 1-2 lesions = 37 % risk 3 or more lesions = 51 % risk ONTT ( Optic Neuritis Treatment Trial ) IV methylprednisolone (3 days) Oral Prednisone (11 days)

is ~ 32 years old ) Nadir of visual acuity is ~ 5 days Improvement in ~ 3 weeks with final recovery at 3 months Fellow eye is involved ~ 20% of the time.

10 CHAMPS ( Controlled High-Risk Subjects Avonex MS Prevention Study ) IM weekly 30 mg if > 2 UBO seen on MRI INO is a lesion in the MLF Lesion is located on the side with the adduction deficit Skew deviation is a supranuclear lesion Lesions can vary but known to include MLF Convergence Spared = posterior Impaired = anterior The End

with the adduction deficit Skew deviation is a supranuclear lesion Lesions can

Optic Neuritis. The optic nerve fibers are coated with myelin to help them conduct the electrical signals back to your brain.

Optic Neuritis. The optic nerve fibers are coated with myelin to help them conduct the electrical signals back to your brain. Optic Neuritis Your doctor thinks that you have had an episode of optic neuritis. This is the most common cause of sudden visual loss in a young patient. It is often associated with discomfort in or around