Of Neuro Ophthalmic Emergencies

U. Schiefer and H. Wilhelm

Ninety percent of clinical neuro-ophthalmology is in the taking of a history (after W.F. Hoyt). Attentive listening, specific questioning and careful evaluation of the information gained make up the foundation of what is primarily a diagnostic subspecialty. The effort invested in gathering this information saves time and avoids unnecessary, potentially dangerous and/or expensive diagnostic procedures.

History Taking

When possible, the previous records of the patient's care should be reviewed prior to beginning the interview. Usually, if the patient will allow, it helps to include in the conversation those other persons who have come to the visit, such as the patient's spouse or close relatives. These people can often provide information that the patient does not know or cannot remember. Patients are often anxious or fearful, and the physician can put them more at ease by conversing in layperson's terms rather than in the technical jargon used by clinicians.

When caring for children, the history taken from one or both parents should not take too long, as the success of the ensuing examination may be hampered by the impatience of the child. When necessary, one should defer some of the more detailed questioning until after the examination has been completed.

The proposed schema for historical questioning, given in ■ Table 1.1, provides a rough outline of the more common details to be discussed, and those that can be compressed or expanded, depending on the details of the case.

When taking the current ophthalmic history, it is of particular importance to determine as precisely as possible the point in time and the speed with which the initial symptoms presented. The longer it has been since the onset of symptoms and the more slowly they may have developed, the more difficult it will be to obtain this information. One should also obtain an accurate account of the eliciting factors, the temporal relationships, accompanying symptoms, and subsequent course of events. Knowledge of these details will allow a quick initial recognition of the more likely sources and various classes of neuro-ophthalmic disease (■ Fig. 1.1).

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Fig. 1.1. Characteristic onsets and courses of neuro-ophthalmi-cally relevant clinical syndromes

Neuro-Ophthalmic Emergencies

From the very start of history taking, one should be alert for clues to the presence of potentially life-threatening or catastrophically blinding disorders. The disorders in this category are listed in ■ Table 1.2, which also gives corresponding references to the appropriate chapters and sections of this text.

Further Reading

Purvin V, Kawasaki A (2005) Neuro-ophthalmic emergencies for the neurologist. Neurologist 11: 195-233

Onset of vision loss

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Fig. 1.1. Characteristic onsets and courses of neuro-ophthalmi-cally relevant clinical syndromes

Table 1.1. Catalog of queries to consider when taking a neuro-ophthalmic history

Current ophthalmic history:

a Current symptoms: time and date of onset, inciting factors, course since onset a Symptoms experienced during the encounter a Associated symptoms of a general (nonvisual) nature a Management of the problem to date

Comprehensive ophthalmic history (questions appropriate to the time of onset and the patient's age):

• For children: Do both eyes see equally well? Does the child have a lazy eye, or has an eye ever been patched for more than a day?

• At what age were glasses first needed, and what visual problem(s) required glasses?

• Since what age have contact lenses been used? Are they hard, semirigid, or soft?

• Has there ever been a problem with eye alignment?

• Has there been any ocular surgery? Eye injuries? Periods of ocular pain and redness?

• Has one or both eyes ever had elevated pressures? Has there been a diagnosis of glaucoma?

• Has there ever been a diagnosis of cataract?

• Is there a congenital color deficiency (for male patients)?

• Have there been other problems: loss of peripheral vision? A disturbance of reading? Photophobia? Poor dark adaptation? Problems understanding visual images?

• Ophthalmic medications? Eye drops?

Family history of eye disease? Birth defects?

• Have there ever been any severe, inherited eye diseases in the family?

• Very poor vision? Strabismus? Cataract? Retinal detachment? Elevated eye pressures? Glaucoma? Poor color vision? Optic atrophy? Blindness? Macular degeneration? Poor reading even with glasses in elderly family members?

General medical history (depending on time of onset and/or the patient's age):

• Systemic diseases: Heart? Lungs? Liver? Kidneys? GI tract? Brain? Vascular disease? Tumors?

• Operations? Hospital admissions? Accidents? Injuries?

• Metabolic disorders: high blood sugar? Overactive thyroid gland? High cholesterol? Gout?

• Hypertension?

• Tobacco, alcohol, and/or recreational drug use?

• Medications? (Particularly important!)

Social history

• Level of education completed, occupation

• Marital status/number of children

• Handicapped? Disabled? Receiving social security benefits?

Note that many of the suggestions are redundant, a tactic that improves the likelihood of discovering useful information, even if the patient does not fully understand some of the questions

The Initial Encounter: Taking a History and Recognition of Neuro-Ophthalmic Emergencies

Table 1.2. Neuro-ophthalmic emergencies and their presenting symptoms


Presenting signs and symptoms

Beware of:

Elevated intracranial pressure

• Bilateral sixth nerve palsies (see Chap. 10)

• Acuity initially unaffected - later stages marked by transient visual obscurations

Headache (increasing in recumbency; see Chap. 16)

• Vomiting while in a fasting state

• Brainstem compression

• Cardiovascular or respiratory arrest

• Hemorrhagic (retinal) infarcts in venous sinus thrombosis

Malignant hypertension

• Optic disc swelling consistent with papilledema, but accompanied by signs of systemic hypertension:

• "Copper wiring" of arterioles

• Arteriovenous crossing changes

• Branch vessel occlusions

• Hard and soft exudates

Visual acuity and general health initially unaffected

• Cerebral infarct

• Myocardial infarct

Carotid dissection

• Excruciating pain, radiating ipsilaterally into the neck, jaw, and/or ear

• Spontaneous onset (predisposed in Marfan's or the Ehlers-Danlos syndromes)

• After trauma (sports injuries or chiropractic manipulations)

• Embolic brain infarction

Pituitary apoplexy

• Hemianopic visual field defects (see Chaps. 3, 4, and 12)

• Relative afferent pupillary defect (see Chap. 2)

• Restricted ocular motility

• Trigeminal nerve involvement (nerve V1+2)

• Optic atrophy in advanced stages of visual loss

• In extreme cases, decrease in or loss of consciousness leading to coma

• Subarachnoid bleeding

• Elevated intracranial pressure that is life threatening or potentially blinding

Cerebral infarct

• Signs of elevated intracranial pressure (see Chaps. 8 and 12)

• Symptoms of hemiplegia or hemiparesis (see Chap. 21)

• Ocular motility disturbances

• Impairment or loss of consciousness

• Elevated intracranial pressure that is life threatening with loss of vital brain centers for respiration, thermoregulation and/or circulation


• Acute oculomotor paralysis with pupillary involvement (see Chap. 10)

• Abrupt and excruciating headache

• Nuchal rigidity

• Clouding or loss of consciousness (see Chap. 21)

• Subarachnoid hemorrhage

Multiple vascular occlusions

• Numerous retinal infarcts (cotton wool exudates) in the setting of a known or suspected endocarditis, paraneoplastic disorder, or vasculitis

• Cardiac arrest

• Malignancies

• Life-threatening cerebral infarcts

Wernicke's encephalopathy (thiamine deficiency)

• Oculomotor deficits

• Impaired consciousness

• Other cranial nerve deficits

• Alcoholic malnutrition

• Parenteral administration of thiamine (vitamin B1) produces a rapid recovery

• Death by multiorgan failure

Orbital cellulitis

• Painful proptosis exophthalmos (see Chap. 9)

• Restricted ocular motility

• Inflammatory optic neuropathy (see Chaps. 8, 9 and 10)

• Regional and systemic signs of inflammatory disease

• Septic cavernous sinus thrombosis (particularly dangerous: mucormycosis)

Giant cell arteritis

• Severe anterior ischemic optic neuropathy ([AION], see Chap. 8)

• Pain and tenderness in the temples or scalp, aggravated when combing or brushing hair

• Jaw claudication

• Ocular motility deficits (rectus muscle ischemia)

• Erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) markedly elevated

• Malaise

• Blindness and/or life-threatening myocardial or cerebral infarction

Table 1.2. (Continued)

Emergency Presenting signs and symptoms

Whipple's disease • Rhythmic oculomasticatory movements are a pathognomonic disturbance of ocular motility: rhythmic convergence movements in synchrony with movements of the jaw and pharyngeal musculature

• Cause: bacterial enteritis (Tropheryma Whippelii)

• Clinical scenario: presents as a malabsorption syndrome

Beware of:

Disease leads to death, when untreated, but curable with antifungal agent (clotrimazole)


Initially symptoms of a gastroenteritis (nausea, vomiting, constipation) starting 4 days after exposure (eating spoiled food) Subsequent bilateral pupillary paresis with reduced light responses and complete paralysis of accommodation (see Chap. 5), eventually developing a complete external ophthalmoplegia

Further systemic paralysis, including the pharyngeal and respiratory muscles, and xerostomia

Death by respiratory failure Also wound botulism (puncture wound with deep anaerobic sepsis). Note: The entry wound may have already healed or may have been forgotten, making it difficult to find

Chapter 2

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