Archives for May 2018

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?

Teaching NeuroImage: Convergence spasm associated with midbrain compression by cerebral aneurysm

Teaching NeuroImage: Convergence spasm associated with midbrain compression by cerebral aneurysm
Konrad P. Weber, Matthew J. Thurtell, and G. Michael Halmagyi
Neurology. April 08, 2008; 70 (15) RESIDENT AND FELLOW SECTION

ARTICLE
A 72-year-old woman presented with intermittent diplopia. She had a history of vascular disease, but no history of stroke or psychiatric disturbance. On examination, she developed convergence spasm with associated miosis following fixation on near targets and during horizontal smooth pursuit (video, figure 1). The spasms were terminated with a blink. She had no ptosis, her eye movements were normal in range, and her pupils were equal in size with normal reactions to light. Neurologic examination was otherwise unremarkable. Five years prior to her current presentation, a left-sided terminal carotid artery aneurysm had been incidentally detected. The aneurysm gradually expanded to a diameter of 2.4 cm and compressed the midbrain (figure 2). The intermittent diplopia had developed in the months following endovascular coiling of the aneurysm.


Figure 1 (A–D) Convergence spasm precipitated by fixation on a near target and released after a blink (video, A).
(A) During fixation on a distant target, the patient has mid-range pupils.
(B) During fixation on a near target, the patient converges and has miotic pupils.
(C) After the near target is removed, convergence is maintained. The pupils remain miotic, which helps to differentiate from bilateral sixth nerve palsies.
(D) After a blink, the patient is able to re-fixate on the distant target and the pupils return to mid-range diameter.
(E) Binocular search coil recording during sinusoidal horizontal smooth pursuit, in which there is a period of convergence spasm that is released by a blink (video, B).
(F) The vergence angle progressively increases up to 50° during the period of convergence spasm (gray triangle).

 

Figure 2 T2-weighted axial MRI demonstrating progressive compression of the midbrain by an expanding aneurysm (arrowheads) arising from the terminal left carotid artery
(A) MRI 5 years prior to onset of symptoms.
(B) MRI at the time of presentation with convergence spasm (after endovascular coiling of the aneurysm).

Convergence spasm is characterized by the inappropriate appearance of the near triad, which consists of convergence, miosis, and accommodation.1 Most patients are young and the cause is psychiatric; a focal lesion is rarely found.1 Since the neural substrate for the near triad is located in the midbrain, convergence spasm could occur with a midbrain lesion. In our patient, isolated convergence spasm may have been a manifestation of midbrain compression. Thus, in patients presenting with convergence spasm, the presence of other focal neurologic signs or the absence of psychiatric history, especially in the elderly, should prompt neuroimaging. Furthermore, it is important not to mistake convergence spasm for bilateral sixth nerve palsies; miosis on attempted lateral gaze is diagnostic of convergence spasm.2

ACKNOWLEDGMENT The authors thank Dr. Geoffrey D. Parker for help with the interpretation of the MRI.

Footnotes
   Supplemental data at www.neurology.org
   
Supported by Garnett Passe and Rodney Williams Memorial Foundation.
   Disclosure: The authors report no conflicts of interest.
   Series editor: Mitchell S.V. Elkind MD, MS, Section Editor

REFERENCES
1. Goldstein JH, Schneekloth BB. Spasm of the near reflex: a spectrum of anomalies. Surv Ophthalmol. 1996;40:269–278
2. Griffin JF, Wray SH, Anderson DP. Misdiagnosis of spasm of the near reflex. Neurology. 1976;26:1018–1020

 

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?

Teaching Video NeuroImages: Pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia

Teaching Video NeuroImages: Pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia
Eleni Papageorgiou, Evangelia E. Tsironi, Sofia Androudi and Petros Koltsidopoulos
Neurology. April 24, 2018; 90 (17) RESIDENT & FELLOW SECTION

ARTICLE
A 65-year-old man with a history of hypertension presented with double vision. Examination revealed wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) (figure 1) and nonsymptomatic, pulse-synchronous, pulsatile proptosis of the left eye (video, links.lww.com/WNL/A389). According to the patient, there was a history of blunt head trauma in childhood and the pulsatile eye was present since then. CT revealed a meningocele into the left orbit due to a bony defect in the orbital roof (figure 2A). CT angiography ruled out a carotid-cavernous fistula. MRI showed an acute infarction at the midline of the midbrain tegmentum, which involved the medial longitudinal fasciculus bilaterally and caused the WEBINO (figure 2B).1 The pulsatile proptosis was attributed to the childhood head injury and due to the orbital roof fracture and associated meningocele pulsation of the brain vessels passed onto the CSF.2

Figure 1 Extraocular movements in 9 cardinal gaze positions
Extraocular eye movements demonstrate exotropia of both eyes in primary positions and bilateral internuclear ophthalmoplegia with impaired adduction on attempted lateral gaze. Upgaze is also mildly affected.

Figure 2 Coronal brain CT and axial brain MRI
(A) Coronal brain CT shows fracture of the left orbital roof with an associated meningocele.
(B) T2-weighted axial brain MRI demonstrates an acute infarction at the midline of the midbrain tegmentum, which involves the medial longitudinal fasciculus bilaterally.

Author contributions E.P.: study concept and design, acquisition of data, analysis and interpretation, writing of the manuscript. E.E.T. and S.A.: study supervision, critical revision of the manuscript for important intellectual content. P.K.: acquisition of data, analysis and interpretation.

Study funding No targeted funding reported.

Disclosure The authors report no disclosures relevant to the manuscript.

References:
1. Kim JS, Jeong SH, Oh YM, Yang YS, Kim SY. Teaching NeuroImage: wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) from midbrain infarction. Neurology 2008;70:e35.
2. Chousterman B, Gualino V, Dohan A, Payen D. A mysterious post-traumatic pulsatile exophthalmos. Intens Care Med 2014;40:102–103.


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?

Clinical Reasoning: A 64-year-old man with painful, unilateral external ophthalmoplegia

Clinical Reasoning: A 64-year-old man with painful, unilateral external ophthalmoplegia
M. Tariq Bhatti. Neurology. 2010; 75 (8)
RESIDENT AND FELLOW SECTION

SECTION 1
A 64-year-old man was referred for evaluation of double vision in March 2009. Four months prior, he experienced daily pain in the region of the right forehead and right eye. Two months later, he noticed constant binocular, vertical double vision. Evaluation by an outside ophthalmologist resulted in the diagnosis of a right 4th cranial nerve (CN) palsy, a normal cranial and orbital MRI study with contrast, and an unremarkable laboratory evaluation. One month later, his right eye began “turning in,” and within a few days he was unable to abduct the eye. Several weeks later, the right eyelid began to droop and progressively worsened over the next several days to complete closure of the eye.

His past medical history was notable for arterial hypertension, depression, rheumatoid arthritis, gastroesophageal reflux disease, nephrolithiasis, squamous cell carcinoma of the forehead, and a precancerous melanoma of the left ear.

When he was seen in the neuro-ophthalmology clinic, visual acuity was 20/20 in each eye. Color vision was intact in each eye. The right pupil was 2 mm larger than the left and was nonreactive to light or near effort. There was no relative afferent pupillary defect (RAPD). Dilated fundus examination was normal in each eye. Eye movements of the right eye were limited in all directions and there was complete right upper eyelid ptosis (figure 1). Corneal sensation of the right eye was absent and there was numbness over the right forehead. The remainder of the cranial nerve examination was normal.

Figure1

Figure 1 Nine cardinal positions of eye movements
There is limited movement of the right eye in all directions of gaze. There was no intorsion of the right eye on attempted downgaze. The right eyelid is manually elevated because of the complete right eyelid ptosis (center, middle panel). Permission obtained from patient.

Questions for consideration:
1. What is the clinical presentation?
2. Where does the lesion localize?

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?