Teaching NeuroImages: Herpes zoster ophthalmicus–related oculomotor palsy accompanied by Hutchinson sign

May 2, 2018 By

Teaching NeuroImages: Herpes zoster ophthalmicus–related oculomotor palsy accompanied by Hutchinson sign
Gayatri S. Reilly and Robert K. Shin
Neurology. April 13, 2010; 74 (15) RESIDENT AND FELLOW SECTION

ARTICLE
A 51-year-old woman presented with acute diplopia. Findings include right ptosis, a dilated, unreactive pupil, and impaired adduction and vertical ductions (figure 1). A skin lesion was noted on the right tip of the nose, residual from a vesicular rash over the right forehead 3 weeks earlier (figure 1). MRI demonstrated enhancement of the cisternal third nerve, obviating the need for angiography (figure 2). The oculomotor palsy resolved within 3 months.

Figure 1 Partial ptosis, mydriasis, and exotropia consistent with a right oculomotor palsy
The skin lesion on the tip of the nose (Hutchinson sign) signifies involvement of the nasociliary branch of V1, which also innervates ocular structures.

Neuro-ophthalmology Questions of the week: NOI13 Diplopia 7 – 3rd Nerve Palsy

May 2, 2018 By

Questions:
1. How are 3rd nerve dysfunctions classified?
2. What is the definition of a partial 3rd nerve palsy?
3. What are the two categories of complete 3rd nerve palsies?
4. What is meant by “a pupil-sparing 3rd nerve palsy”?
5. What tests should be done when a patient over age 50 presents with an isolated incomplete 3rd nerve palsy and the pupil is involved?
6. What should be done when a patient over age 50 presents with an isolated complete 3rd nerve palsy and the pupil is NOT involved?
7. What should be done when a patient over age 50 presents with an isolated complete 3rd nerve palsy, the pupil is NOT involved, and has normal lab (Blood glucose, CBC, platelets, ESR, CRP) is followed daily and develops pupillary involvement?
8. What is the most common cause of an isolated “pupil-sparing 3rd nerve palsy”?
9. Are microvascular 3rd nerve palsies painful?
10. What should be ruled-out when making the diagnosis of a microvascular complete 3rd nerve palsy with pupil-sparing in a patient over 50?
11. What in addition to cranial arteritis should be considered when making the diagnosis of a microvascular pupil complete 3rd nerve palsy with pupil-sparing?
12. How long does it usually take for a microvascular 3rd nerve palsy to resolve?
13. If a complete 3rd nerve palsy with pupil-sparing thought to be of microvascular origin does not clear in 4 months what should be done?
14. What test should be done in a patient under age 50 who presents with an isolated 3rd nerve palsy with or without pupillary involvement?
15. Should the pupils of a patient with an acute 3rd nerve palsy with “pupil sparing” be dilated to complete the eye exam?
16. What should be ruled-out in a patient without a history of trauma has signs of aberrant regeneration of the 3rd nerve?
17. What are the symptoms of pituitary apoplexy?
18. What are the findings when there is a unilateral lesion of the entire 3rd nerve nucleus?
19. Why does a complete unilateral nuclear 3rd nerve palsy have bilateral ptosis?
20. Why does a complete unilateral nuclear 3rd nerve palsy have bilateral elevation deficits?
21. What are the findings of a unilateral lesion of the 3rd nerve fascicle?

Teaching NeuroImages: Upright-supine test to evaluate vertical diplopia

April 26, 2018 By

Teaching NeuroImages: Upright-supine test to evaluate vertical diplopia
Nailyn Rasool and Sashank Prasad
Neurology May 12, 2015; 84 (19)
RESIDENT AND FELLOW SECTION

Article:
A 36-year-old woman presented with vertical diplopia, nausea, and disequilibrium. Maddox rod testing was performed in the upright and supine positions (figures 1 and 2).


Figure 1 Upright-supine test
During Maddox rod testing, the patient used her fingers to demonstrate the separation of images.
The vertical deviation decreased substantially (over 50%) when supine compared to upright.

Questions of the Week: NOI13 Diplopia 6 – 4th Nerve Palsy

April 26, 2018 By

NOI13 Diplopia 6 – 4th Nerve Palsy
Questions:
63. What are 6 characteristics of a 4th nerve palsy?
64. When a patient has a 3rd nerve palsy and the eye cannot adduct, how can the function of the ipsilateral 4th nerve shown to be present?
65. What other conditions should be considered when the patient has the findings of a 4th nerve palsy?
66. A patient has a 4th nerve lesion associated with a contralateral Horner syndrome, where does this localize the lesion?
67. A microvascular 4th nerve palsy is suspected because a 60-year-old patient has diabetes, hypertension and hyperlipidemia what should be ordered?
68. A presumed microvascular 4th nerve palsy in a 60-year-old patient with diabetes, hypertension and hyperlipidemia has not resolved within 3-6 months, what test should be ordered?
69. A 60 year-old patient presents with intermittent vertical diplopia with the findings of a 4th nerve palsy on the 3-step-test. What in addition to an acquired 4th nerve palsy, restriction, myasthenia, Wernicke encephalopathy and cranial arteritis should be considered and what would help confirm this?
70. What are 3 findings characteristic of a congenital a decompensated congenital 4th nerve palsy?
71. What should one consider with a hypertropia not mapping to a 4th nerve palsy?
72. How can one differentiate a skew deviation from a 4th nerve palsy?

Teaching Video NeuroImages: Bilateral abducens ocular neuromyotonia

April 18, 2018 By

Teaching Video NeuroImages: Bilateral abducens ocular neuromyotonia
Kavin Vanikieti and Joseph F. Rizzo
Neurology September 05, 2017; 89 (10)
RESIDENT AND FELLOW SECTION http://n.neurology.org/content/89/10/e128

A 44-year-old woman with a history of resolved nasopharyngeal carcinoma presented with an 8-month history of transient binocular horizontal diplopia. She received complete concurrent chemoradiotherapy 7 years ago. Following prolonged eccentric gaze to the right, she developed involuntary contraction of right lateral rectus, which resulted in 60 seconds of right exotropia. These events also occurred following prolonged eccentric gaze to the left, which resulted in left exotropia (video 1). Treatment with carbamazepine improved her symptoms (video 2). Ocular neuromyotonia should be included as a differential diagnosis of transient diplopia, especially in those who have undergone prior radiotherapy to the parasellar region.1

Video 1 – Pretreatment
Following prolonged eccentric gaze to the right, the patient developed involuntary contraction of right lateral rectus, which resulted in right exotropia. Each episode lasted approximately 60 seconds and then the eyes returned to normal alignment. These events also occurred following prolonged eccentric gaze to the left, which resulted in left exotropia.

Video 2 – Post Treatment
Complete resolution with carbamazepine

Teaching Slides – PowerPoint Presentation

Reference: 1. Leigh RJ, Zee DS. The Neurology of Eye Movements, 5th ed. Oxford: Oxford University Press; 2015:615.

Questions of the week: NOI13 Diplopia 5 – 6th Nerve Palsy

April 18, 2018 By

NOI13  Diplopia 5 – 6th Nerve Palsy  

Questions: 
1. Where is the nucleus of the 6th cranial nerve located?
2. Which cranial nerve passes through theDorellocanal beneath the petroclynoid (Gruber) ligament?
3. What are the clinical findings of a unilateral 6th nerve nuclear lesion?
4. Why does a unilateral nuclear 6th nerve palsy cause an ipsilateral horizontal gaze palsy?
5. Why is a unilateral 6th nerve palsy often accompanied by an ipsilateral 7th nerve palsy?
6. Where does an ipsilateral Horner syndrome and 6th nerve palsy localize the lesion?
7. Where does an ipsilateral Horner syndrome and 6th nerve palsy associated with a third, fourth, V1 and V2 deficit localize?
8. A microvascular 6th nerve palsy is suspected because a 60-year-old patient has diabetes, hypertension and hyperlipidemia, what tests should be done?
9. A presumed microvascular 6th nerve palsy is suspected in a 60-year-old patient with diabetes, hypertension and hyperlipidemia has not resolved within 3-6 months, what test should be ordered?
10. What is Brown syndrome?
11. What explains the findings of Duane syndrome?
12. What are the findings of Duane syndrome?
13. Are systemic associations common in Duane syndrome?
14. What is the Möbius syndrome?
15.What should be done in a patient with bilateral  6th nerve palsies and a normal MRI?
16. What is best initial imaging study for an isolated 6th nerve palsy in  20-year-old?
17. What is the best initial treatment for a comitant small-angle 6th nerve palsy?
18. What is the likelihood that an isolated,vasculopathic6th nerve palsy will recover?
19. What condition does convergence spasm mimic?
20. What are the findings of convergence spasm?
21. What exam technique should be used in suspected bilateral or unilateral 6th nerve palsy to eliminate convergence spasm?
22. What may mimic a 6th nerve palsy?

Clinical Reasoning: A 22-year-old man with diplopia

April 15, 2018 By

Clinical Reasoning: A 22-year-old man with diplopia
Chelsea Meyer, DO, Don Raphael P. Wynn, MD, Stefan M. Pulst, MD, DrMed Ricky Chen, MD, Kathleen Digre, MD Correspondence to Dr. Meyer: chelsea.meyer@hsc.utah.edu
Neurology October 11, 2016; 87 (15)
RESIDENT & FELLOW SECTION Section Editor John J. Millichap, MD

SECTION 1
A 22-year-old previously healthy man presented to an ophthalmology clinic with binocular horizontal diplopia. He had recently traveled to the main island of Hawaii. About 2 weeks after returning home, he developed a severe headache with associated fever, emesis, photophobia, phonophobia, and neck stiffness. He also reported a sensation of pressure in his left eye and both ears but denied any pulsatile tinnitus or transient vision loss. Over the next 2 weeks, his headaches worsened, causing him to wake up frequently in the night. He then developed horizontal diplopia that was worse at a distance and was referred to the neuro-ophthalmology clinic. Examination. The patient had some limitation in neck flexion with associated pain. His visual acuity was 20/20 on the right and 20/25 on the left. Pupillary examination showed no relative afferent defect and visual fields were full. He was found to have bilateral abducens palsy with an esotropia of 30 D and a left hypertropia of 2 D with notable mild right head tilt. The hypertropia was thought to be a partial left 4th nerve palsy with a compensatory right head tilt, although a full Parks-Bielschowsky 3-step test was not preformed to confirm this. He had moderate to severe papilledema on funduscopic examination (figure, A). The remainder of his neurologic and ophthalmologic examination was within normal limits.


Figure (A) Funduscopic photographs of the optic disc of the left eye show papilledema with blurring of the disc margin, optic nerve hyperemia, and peripapillary hemorrhages.

Questions for consideration:
1. Given his papilledema and bilateral abducens nerve palsy, where would you localize this?
2. What is the differential diagnosis for bilateral abducens nerve palsy?

Questions of the Week: Diplopia 4 – Binocular Diplopia 2

April 12, 2018 By

Questions:
8. What tests should be done in all patients over age 50 with transient or permanent diplopia?
9. What is the Silent Sinus Syndrome?
10. What is the Kearns-Sayre syndrome?
11. What are 5 mitochondrial diseases that can have ophthalmoplegia?
12. What are the features of Myotonic Dystrophy?
13. What are the features of Oculopharyngeal Dystrophy?
14. What 3 conditions other than paresis or restriction should be considered in any adult with new-onset transient or permanent ocular misalignment?
15. What may induce the myasthenia gravis or make it worse?
16. What is the mechanism of action of edrophonium?
17. What are the side effects of the Tensilon test?
18. What does the development of lid retraction in a patient with Myasthenia suggest?
19. What percent of patients presenting with purely ocular myasthenia will progress to develop generalized disease?
20. Does myasthenia gravis ever affect the pupils?
21. Does myasthenia gravis ever cause pain?

Clinical Reasoning: A 20-year-old man with headache and double vision

April 4, 2018 By

Khaled Moussawi, Anoopum Gupta and Haatem Reda
Neurology October 11, 2016; 87 (15)
Correspondence to Dr. Moussawi: khaled.moussawi@nih.gov
RESIDENT AND FELLOW SECTION
Section Editor John J. Millichap, MD

From Harvard Medical School (K.M., A.G., H.R.), Boston; Department of Neurology (K.M., A.G., H.R.), Massachusetts General Hospital, Boston; and Department of Neurology (K.M., A.G.), Brigham and Women’s Hospital, Boston, MA. Dr. Moussawi is currently with the National Institute on Drug Abuse; and the Department of Psychiatry, Johns Hopkins Medicine, Baltimore, MD.

SECTION 1
A 20-year-old man presented to the emergency department with 1 week of headaches and double vision following 2 days of fever (1028 F), nausea, and vomiting. His headache was progressively worsening, throbbing behind his right eye, nonpositional, and associated with photophobia, blurry vision, and pain with eye movement. Occasionally, it was severe enough to wake him up from sleep. Horizontal double vision ensued soon after the headache. His diplopia was worse looking at a distance, improved on leftward gaze, worsened on rightward gaze, and resolved with closing one eye. He denied neck stiffness, focal weakness, numbness, or other neurologic symptoms. He denied recent rashes, infections, or tick bites. He lives on a farm in central Brazil. He arrived in Massachusetts 2 months before his presentation to visit family members. He had no significant medical history and took no medications. He did not smoke or use drugs.

Question for consideration:
1. What is the localization of his presenting symptom?

Questions of the Week: Diplopia 3 Diagnosis of Binocular Diplopia 1

April 4, 2018 By

Questions:
1. What is the most common cause of unilateral proptosis?
2. What is the most common cause of bilateral proptosis?
3. Which is the most commonly involved extraocular muscle in thyroid eye disease?
4. What conditions should be considered in a patient with enlarged extraocular muscles?
5. On CT or MRI, which condition spares the insertions of the extraocular muscles, thyroid eye disease or idiopathic orbital inflammation (orbital pseudotumor)?
6. What 4 features differentiate idiopathic orbital inflammation (orbital pseudotumor) from thyroid eye disease?
7. When should a biopsy be done in a patient with presumed “myositis”?