Cranial Nerve Disorder - Clinical Patient Case of the Week
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Try this case and test your knowledge of cranial nerve disorders.
A 38-year-old woman without past medical history presents to you with a rapid-onset headache. This headache is not the worst of her life.
You note that the patient has her head tilted to the left on your initial inspection. When asked, she states that when she holds her head straight, she has diplopia. Further examination reveals the inability to move the eye downward and laterally, indicating a trochlear (fourth cranial nerve [CN-IV]) palsy.
There is no other neurologic deficit or papilledema present. Because isolated CN-IV palsy is usually traumatic, you ask the patient about this but she denies any injury. Her blood glucose is normal and a pregnancy test is negative.
Computed tomography reveals no pathology. A "champagne tap" lumbar puncture shows no xanthochromia.
Question:
The most likely diagnosis is...
Answer Options:
ammonia poisoning
central venous thrombosis
Cotard delusion
malingering syndrome
See the Answer:
The correct answer is:
Central venous thrombosis
Educational Objective:
Recognize the symptoms and diagnostics associated with cerebral venous thrombosis.
Key Point:
Cerebral venous thrombosis is associated with headaches and many neurologic findings. It is important to remember this entity and that, even once subarachnoid hemorrhage has been ruled out, serious underlying pathology may exist, and neurologic consult is indicated.
Explanation:
The trochlear nerve innervates the superior oblique muscle, which moves the eye down and laterally while also providing medial rotation (see below image). When it is damaged, the eyeball tends to drift up due to unopposed superior rectus muscle function (CN III). This leads to head tilt in an attempt to bring both eyes back into the same horizontal plane. Even though the trochlear nerve contributes to lateral eye movement, palsy of this nerve is not sufficient to lose all lateral movement. The above patient must also have an abducens nerve (CN IV) palsy, which is causing the lack of lateral gaze. Both of these nerves (and often also oculomotor nerve CN III) are often affected by cavernous sinus thrombosis.
Central venous thrombosis is a rare disease and can have multiple neurologic findings. Headache is the most prominent. Papilledema is a helpful diagnostic sign, but it is present in less than one-half of cases. Likewise, seizure with headache may be indicative, but it is present only 50% of the time.
In Cotard delusion, patients believe they are already dead and can usually smell the stench of their own corpse.
Malingering is not feasible because voluntary control of the extraocular muscles is not unilateral.
Ammonia poisoning gives primarily respiratory symptoms. With a normal blood sugar, diabetic neuropathy is also unlikely.
Figure 1.
The above image comes from an educational video about eye movements.
References:
Stettler B. Brain and cranial nerve disorders. In: Walls RM, et al. Rosen’s Emergency Medicine: Concepts and Clinical Practice. 9th ed., 2018:1289-1297.e2.
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