The lecture based on postgraduate students in Ophthalmology that is FCPS, MS DO. DCO

1. Neuro-Ophthalmology: Pupil
Dr. Md. Anisur Rahman (Anjum)
Professor & Head, Department of Ophthalmology
Dhaka Medical College, Dhaka

2. Adie pupil/tonic pupil/Adie syndrome
• An Adie pupil (tonic pupil, Adie syndrome) is caused by denervation of the
postganglionic parasympathetic supply to the sphincter pupillae and the ciliary
muscle, and may follow a viral illness.
• Sites of dysfunction are presumed to be the ciliary ganglion, and, in wider
Holmes–Adie syndrome, the dorsal root ganglion involved in reflex pathways.
• It typically affects young women and presents in one eye in 80%, though
involvement of the second eye typically develops within months or years.

3. Adie's tonic pupil
Adie's tonic pupil refers to a dilated, poorly reactive pupil, presumably from
dysfunction of the ciliary ganglion. The nerves responsible for pupil constriction
connect through the ciliary ganglion. When they are damaged the pupil dilates.
Sometimes, over time, the second eye becomes affected.

4. Adie syndrome
• Adie syndrome is a neurological disorder affecting the pupil of the eye and the
autonomic nervous system. It is characterized by one eye with a pupil that is
larger than normal that constricts slowly in bright light (tonic pupil), along with
the absence of deep tendon reflexes, usually in the Achilles tendon.

5. What is Adie's pupil?
With Adie's pupil, there is an abnormal pupillary response to light. In most cases, it
affects only one eye. The affected pupil is usually larger than normal and does not
constrict as it should in the presence of bright light

6. Diagnosis: Symptoms.
Patients may notice anisocoria, or may have blurring for near due to impaired
accommodation.

7. Signs
Pupil: Large, regular (irregularity sometimes reported).
The direct light reflex is absent or sluggish.
On slit lamp examination, vermiform movements of the pupillary border are
typically seen.
Constriction is also absent or sluggish in response to light stimulation of the
fellow eye (consensual light reflex)

8. The pupil responds slowly to near, following which re-dilatation is also slow.
Accommodation may manifest similar tonicity, with slowed and impaired
focusing for near and prolonged re-focusing in the distance.
In long-standing cases the pupil may become small (‘little old Adie’).

9. Large right pupil
A) Large right pupil B) absent or sluggish direct light reflex
C) consensual reflex is similar (D) diminished deep tendon reflex
Right
Adie
pupil
.

10. Pharmacological testing
 Instillation of 0.1–0.125% pilocarpine into both eyes leads to constriction of the
abnormal pupil due to denervation hypersensitivity, with the normal pupil
unaffected. Some diabetic patients may also show this response and very
occasionally both pupils constrict in normal individuals.
 Syphilis serology should usually be checked in patients with bilateral tonic pupils.

11. Argyll Robertson pupils
Argyll Robertson pupils are caused by neurosyphilis, and have been attributed to a
dorsal midbrain lesion that interrupts the pupillary light reflex pathway but spares
the more ventral pupillary near reflex pathway – light–near dissociation results.

12. Argyll Robertson pupils

13. Argyll Robertson pupils
In dim light both pupils are small and may be irregular. In bright light neither
pupil constricts, but on accommodation (near target) both constrict. The pupils do
not dilate well in the dark, but cocaine induces mydriasis unless marked iris
atrophy is present.

14. How to differentiate Argyll Robertson pupils and Adie tonic pupil?
After instillation of pilocarpine 0.1% into both eyes, neither pupil constricts,
distinguishing Argyll Robertson pupils from bilateral long-standing tonic pupils.

15. Unilateral
• Afferent conduction defect
• Adie pupil
• Herpes zoster ophthalmicus
• Aberrant regeneration of the third cranial
nerve
Bilateral
• Neurosyphilis
• Type 1 diabetes mellitus
• Myotonic dystrophy
• Parinaud (dorsal midbrain) syndr
• Familial amyloidosis
• Encephalitis
• Chronic alcoholism
Causes of light–near dissociation

16. Pituitary gland
The Sella turcica (Turkish saddle) is a deep saddle-shaped depression in the
superior surface of the body of the sphenoid bone in which the pituitary gland lies
The roof of the Sella is formed by a fold of dura mater, the diaphragma sellae,
which stretches from the anterior to the posterior clinoid process.

17. Upper nasal fiber
Chiasma
Lower nasal fiber
Pituitary Gland
Dorsum sellae
Diaphragma sellae

18. • The optic nerves and chiasm lie above the diaphragma sellae; posteriorly, the
chiasm is continuous with the optic tracts and forms the anterior wall of the third
ventricle. A visual field defect in a patient with a pituitary tumour therefore
generally indicates suprasellar extension.

19. • Tumours less than 10 mm in diameter (microadenomas) tend to remain confined to
the Sella, whereas those larger than10 mm (macroadenomas) often extend outside.

20. Pituitary adenomas
Tumour classification is based on the type of hormone secreted;
about 25% of primary pituitary tumours do not secrete any hormones and may be
asymptomatic, cause hypopituitarism and/or ophthalmic features.
An older classification system divided pituitary tumours into acidophilic, basophilic
and chromophobic types based on their histological staining characteristics, but is
now not commonly used.

21. Ophthalmic features of large adenomas
Large pituitary lesions may first present to ophthalmologists, often with vague
visual symptoms, and a low threshold should be adopted for visual field assessment
in chronic headache of any sort. It is also important to perform a careful visual field
assessment on both eyes in patients with unexplained unilateral central visual
impairment.

22. Symptoms
Headache may be prominent due to local effects but does not have the usual features
associated with raised ICP, and diagnostic delay is therefore common.
Visual symptoms may be vague; they usually have a gradual onset and may not be
noticed by the patient until well established.

23. Symptoms
Colour desaturation across the vertical midline of the uniocular visual field is an
early sign of chiasmal compression.
The patient is asked to compare the colour and intensity of a red pin or pen top as it
is moved from the nasal to the temporal visual field in each eye.
Another technique is to simultaneously present red targets in precisely symmetrical
parts of the temporal and nasal visual fields, and to ask if the colours appear the
same.

24. Symptoms
• Optic atrophy is present in approximately 50% of cases with field defects. When
optic atrophy is present the prognosis for visual recovery after treatment is
guarded.
• When nerve fiber loss is confined to fibres originating in the nasal retina (i.e. nasal
to the fovea) only the nasal and temporal aspects of the disc will be involved,
resulting in a band or ‘bow tie’-shaped atrophy.

25. Symptoms
• Papilloedema is rare.
• Visual field defects depend on the location and direction of enlargement of a
compressive lesion, as well as the anatomical relationship between the pituitary
and chiasm. Patients may not present until central vision is affected from pressure
on macular fibres.

26. Symptoms
• Extraocular muscle paresis due to disruption of the cranial nerves traversing the
cavernous sinus.
• See-saw nystagmus is a rare feature.

27. Anatomical variations in the position of the chiasm
Central 80%
Prefixed 10%
Post-fixed 10%

28. OS OD
HM
CF
HM
CF
Typical progression of bitemporal visual field defects caused by compression of the chiasm
from below by a pituitary adenoma. LE = left eye; RE = right eye

29. • Lower nasal optic nerve fibres traverse the chiasm inferiorly and anteriorly, hence
the upper temporal quadrants of both visual fields are affected first by most
expanding pituitary lesions, giving a bitemporal superior quadrantanopia
progressing to the classic chiasmal visual field lesion, a bitemporal hemianopia
(Fig) loss is commonly asymmetrical between the two eyes

30. Typically asymmetrical bitemporal hemianopia

31. Upper nasal fibres traverse the chiasm high and posteriorly and therefore are
involved first by a lesion such as a craniopharyngioma that arises above the
chiasm. If the lower temporal quadrants of the visual field are affected more
profoundly than the upper, a pituitary adenoma

32. Examples of optic nerve compression by meningioma; a junctional scotoma
resulting from a tuberculum sellae lesion is also shown
Olfactory groove
meningioma
Tuberculum sellae
meningioma
Sphenoidal ridge
meningioma

34. ‘Junctional scotoma’
Junctional scotoma is the visual field defects that arise from damage to the junction
of the optic nerve and the optic chiasm. Sellar masses including pituitary tumors are
the most common cause of these visual field defects.
Visual field shows ipsilateral central scotoma and contralateral superior temporal
quadrantanopia (junctional scotoma, JS)
It occurs mostly in postfixed chiasma.

35. Junctional scotoma: Etiology
Lesions that produce the junctional scotoma are typically extrinsic compressive
mass lesions at the junction of the optic nerve and the chiasm. Other
demyelinating, infectious, inflammatory, infiltrative, traumatic, and other
etiologies can occur in this location however.

36. Junctional scotoma: Etiology
Following are the common cause:
• Suprasellar tumors (commonly pituitary adenoma)
• Suprasellar meningioma
• Craniopharyngioma
• Aneurysms of the internal carotid or the anterior communicating artery

38. Sphenoid wing meningiomas
Sphenoid wing meningiomas are slow growing tumors that originate from outer
arachnoid meningeal epithelial cells.
Meningiomas can be multiple, particularly when they associated with
neurofibromatosis type 2 (NF2).

39. Sphenoid wing meningiomas
Sphenoid wing meningiomas are classified as either globoid tumors with a
nodular shape or an en plaque tumor which is flat and spreads along the entire
sphenoid ridge.
The globoid tumors include 3 groups depending on their location:
inner (medial),
middle, and
lateral (pterional)

40. Sphenoid wing meningiomas
Medial sphenoid wing meningiomas have a higher morbidity, mortality, and
recurrence rate compared to other meningiomas due to their involvement with
anterior visual pathways, anterior intracranial arteries, and the cavernous sinus

41. Clinical Features
Tumours compress the optic nerve early if the tumour is located medially and late
if the lateral aspect of the sphenoid bone and middle cranial fossa are involved. A
classic finding in the latter is fullness in the temporal fossa due to hyperostosis

43. Investigation
MR with gadolinium contrast utilizing multiple planes and thin sections
demonstrates the relationship between a mass lesion and the chiasm, and is usually
the preferred imaging modality. Adenomas are typically hypointense on T1 and
hyperintense on T2 images.
CT will demonstrate enlargement or erosion of the sella.
Endocrinological evaluation is complex, particularly as combined hormonal
over- and under-secretion may be present, and is usually undertaken by an
endocrine

44. Treatment of pituitary adenomas
Observation may be appropriate for incidentally discovered and clinically
silent tumours.
Medical therapy is usually the initial step and consists of the reduction in
tumour size and secretion using agents such as dopamine agonists (e.g.
cabergoline and the older bromocriptine) and somatostatin analogues such
as octreotide, with supplementary hormonal correction as appropriate.

45. Treatment of pituitary adenomas
Surgery consists of tumour debulking rather than complete excision
and is usually carried out endoscopically via a trans-sphenoidal
approach through a gum incision behind the upper lip. Indications
include the failure or intolerance of medical management and
sometimes decompression for acute visual loss. Visual field
improvement is fastest in the earliest weeks and months following
surgery.

46. Treatment of pituitary adenomas
Radiotherapy is rarely employed due to the risk of complications,
but is utilized in some circumstances. Newer techniques include
intensity-modulated radiation therapy and stereotactic radiosurgery.
Monitoring. Long-term ophthalmological review is required, with
serial assessment of visual function

47. Pituitary apoplexy (PA)/ Sheehan syndrome
• Pituitary apoplexy (PA) is caused by acute haemorrhage into or infarction of the
pituitary gland, and is usually associated with a previously undiagnosed adenoma;
Sheehan syndrome is infarction of the pituitary usually associated with childbirth
and is generally regarded as a form of PA.
• PA typically manifests with the sudden onset of a severe headache, nausea and
vomiting, sometimes with meningism and occasionally reduced consciousness or
stroke.

48. Pituitary apoplexy (PA)/ Sheehan syndrome
• There is often reduced visual acuity and/or a bitemporal hemianopia depending
on the anatomical effects of the lesion.
• Double vision due to compromise of the adjacent ocular motor nerves is common.
Acute hormonal insufficiency can lead to life-threatening complications such as an
Addisonian crisis.

49. Pituitary apoplexy (PA)/ Sheehan syndrome: Investigation and
treatment
Investigations include
• MR, urgent
• visual field testing, and
• hormonal assessment.
• Acute medical management, including hormone administration and surgical
decompression, may be necessary.

50. Craniopharyngioma: In children
Craniopharyngioma is a slow-growing tumour arising from vestigial remnants of
the Rathke pouch along the pituitary stalk.
Affected children frequently present with
• dwarfism,
• delayed sexual development and
• obesity due to interference with hypothalamic function.

51. Craniopharyngioma: In adults
 Adults usually present with
visual impairment.
Visual field defects are complex and may be due to involvement of the optic
nerves, chiasm or tracts

52. Progression of bitemporal visual field defects caused by compression of the chiasm from
above by a craniopharyngioma. LE = left eye; RE = right eye

53. • The initial defect frequently involves both inferotemporal fields because the
tumour compresses the chiasm from above and behind, damaging the upper nasal
fibres. MRI shows a solid tumour that appears isointense on T1 images. Cystic
components appear hyperintense on T1 images.
• Treatment is mainly surgical, but recurrences are common

54. • Magnetic resonance imaging (MRI) is one of the most commonly used tests in
neurology and neurosurgery. MRI provides exquisite detail of brain, spinal cord
and vascular anatomy, and has the advantage of being able to visualize anatomy in
all three planes: axial, sagittal and coronal (see the example image below).

55. Axial, Sagittal, Coronal
MRI has an advantage over CT in being able to detect flowing blood and cryptic vascular
malformations. It can also detect demyelinating disease, and has no beam-hardening artifacts
such as can be seen with CT. Thus, the posterior fossa is more easily visualized on MRI than
CT. Imaging is also performed without any ionizing radiation

56. In general, T1- and T2-weighted images can be easily differentiated by
looking the CSF. CSF is dark on T1-weighted imaging and bright on T2-
weighted imaging.