Teaching Video NeuroImages: Minimal anomalies of dorsal midbrain syndrome (Parinaud syndrome)

Teaching Video NeuroImages: Minimal anomalies of dorsal midbrain syndrome (Parinaud syndrome)
Pilar Rojas, Philippe Maeder and François-Xavier Borruat.
Neurology. January 03, 2017; 88 (1) RESIDENT AND FELLOW SECTION

Parinaud syndrome results from posterior commissure dysfunction, and is associated with 4 major signs: limitation of upgaze, pupillary light-near dissociation, convergence abnormalities, and Collier sign.1,2

A 46-year-old man complained of vertical diplopia due to a subtle left skew deviation. Upgaze pursuit was normal, but upward saccades were slowed, without convergence abnormalities or Collier sign (video). Pupillary light-near dissociation was present (video). MRI revealed a tectal mesencephalic lesion (figure).

Figure Brain MRI
T2 axial and coronal cuts (A, C) and T1 sagittal cut with gadolinium (B) revealed a tumor originating from the walls of the third ventricle posteriorly, with an invasion of the posterior commissure (white arrow). The lesion was later biopsied and pathology revealed a pilocytic astrocytoma.

Video Ocular motility and pupils examination.
Horizontal pursuit and saccades, and both upward and downward vertical pursuit were normal in both eyes, namely without any limitation of upgaze. The speed of downward vertical saccades was normal, whereas upward saccades from primary gaze were slowed. The pupils were of intermediate and equal size, and pupillary light-near dissociation was present in both eyes.

Slowed upward saccades and pupillary light-near dissociation represent an early stage of posterior commissure dysfunction, before frank upgaze palsy, Collier sign, or convergence abnormalities.

AUTHOR CONTRIBUTIONS
Pilar Rojas is an author and contributed to drafting and revising the manuscript. Philippe Maeder is an author, contributed to data acquisition, and revised the manuscript. François-Xavier Borruat is an author, contributed to data acquisition, and revised the manuscript.

STUDY FUNDING No targeted funding reported.

DISCLOSURE The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

Footnotes
  Supplemental data at Neurology.org
  Download teaching slides: Neurology.org

REFERENCES
1. Leigh RJ, Zee DS. The Neurology of Eye Movements, 3rd ed. part II, ch 10. Oxford: Oxford University Press; 1999:517–519.
2. Miller NR, Newman NJ. Clinical Neuro-Ophthalmology, 5th ed. vol 1, ch 29. Baltimore: Williams & Wilkins; 1998:1304–1311.

 

 

Neuro-ophthalmology questions of the week: NOI13 Diplopia 9.3 – Internuclear & Supranuclear Lesions

Questions:
25. Where are the critical supranuclear structures mediating vertical gaze located?
26. What are the 4 most important pretectal areas related to vertical eye movement?
27. Where are the burst neurons responsible for vertical saccades located?
28. What serves as the neural integrator for vertical gaze and torsion?
29. For downgaze, where do the axons from the interstitial nucleus of Cajal (INC) project?
30. What are the 4 findings of the Dorsal Midbrain Syndrome?
31. What are 2 other names for the Dorsal Midbrain Syndrome?
32. What is a common cause of the Dorsal Midbrain Syndrome?
33. What are the characteristics of Skew Deviation?
34. What test can help differentiate a 4th nerve palsy from skew deviation?
35. What is the ocular tilt reaction?
36. What transmission pathway is disrupted in skew deviation and the ocular tilt reaction?
37. What infectious condition can cause a vertical gaze palsy?

Neuro-ophthalmology questions of the week: NOI13 Diplopia 9.2 – Internuclear & Supranuclear Lesions

Questions:
15. What is ocular apraxia?
16. What is convergence spasm?
17. What is the cause of convergence spasm?
18. What test will usually differentiate convergence spasm from a bilateral 6th nerve palsy?
19. What is convergence insufficiency?
20. How is convergence insufficiency treated?
21. What are causes of convergence insufficiency?
22. What is divergence insufficiency?
23. What causes divergence insufficiency?
24. What lesion location is likely in the presence of horizontal conjugate deviation of the eyes to one side?

Neuro-ophthalmology questions of the week: NOI13 Diplopia 9.1 – Internuclear & Supranuclear Lesions

Questions:
1. Where is the horizontal gaze center located?
2. What is the location of the burst neurons responsible for horizontal saccades?
3. What are the findings of a lesion of the 6th nerve nucleus?
4. What are the findings of a lesion of the PPRF?
5. What are 4 findings of a unilateral internuclear ophthalmoplegia?
6. Where is the lesion located that produces a unilateral internuclear ophthalmoplegia?
7. What are the findings of a bilateral internuclear ophthalmoplegia?
8. What are the findings of a walleyed bilateral internuclear ophthalmoplegia (WEBINO)?
9. Where is the lesion located that produces a wall-eyed bilateral internuclear ophthalmoplegia (WEBINO)?
10. What are the findings of the One-and-a-Half Syndrome?
11. Where is the lesion located that produces the One-and-a-Half Syndrome?
12. What are 2 common causes of isolated internuclear ophthalmoplegia?
13. What conditions may mimic an isolated internuclear ophthalmoplegia?
14. What conditions may mimic the one-and-a-half syndrome?

Neuro-ophthalmology Question of the week: NOI13  Diplopia 8 Multiple Cranial Nerve Involvement

Questions:
1. What associated signs with lesions causing unilateral or bilateral ophthalmoplegia may help localize the lesion
2. What are the findings of the orbital apex syndrome?

3. What are the findings of the cavernous sinus syndrome?
4. Do brainstem lesions commonly produce multiple cranial nerve palsies?
5. What are 7 systemic disorders that affect the cranial nerves?
6. What condition should be considered in patients with unilateral or bilateral ophthalmoplegia and normal pupils presumed to result from multiple cranial neuropathies?
7.What should be presumed to be the cause of a painful orbital apex syndrome in a diabetic patient?
8. Do normal orbital imaging studies rule out an orbital apex lesion?
9. Does the orbital apex syndrome involve V2?
10. Which cranial nerves are enclosed in the lateral wall of the cavernous sinus?
11. Why do cavernous carotid artery aneurysms often present with an isolated sixth nerve palsy?
12. What diagnoses should be considered in all cases of new onset constant or transient ptosis and/or diplopia
13. What syndromes have ataxia, areflexia and ophthalmoplegia?
14. What is the Guillain-Barré syndrome?
15. What is the Miller Fisher syndrome?
16. What is the difference between the Miller Fisher and the Guillain-Barré syndromes?
17. When should the Wernicke encephalopathy be considered and what are its symptoms?
18. What is the cause and treatment of Wernicke encephalopathy?
19. What does botulism do to the pupils and what are its other symptoms?
20. What is the infectious agent of botulism?
21. What is the mechanism of the botulism toxin?
22. What is the treatment for botulism?

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

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

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

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

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?

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

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?