Neuro Ophth Patel Mc Review Jan28-11

Front 1

What is the most common cause of CNIV palsy in children and if >10 degrees of fusional capacity, what does it indicate?

Back 1

congenital
>10 = bilateral

Front 2

How to distinguish congenital vs acquired horners

Back 2

congenital: heterochromia b/c of lack of sympathetic innervation, leading to blue iris

Front 3

Define palinopsia, localize and name 2 causes

Back 3

Temporal after images caused by increased cortical activity in the parietal-occipital region

Migraines, hallucinogens

Front 4

Define cerebral polyopia

Back 4

Preseveration of visual images in space (stacked up).

Can be monocular cause of diploplia/triploplia, etc.

Front 5

Describe progression, type of nystagmus, tests required, and expected resolution of disease in spasmus nutans

Back 5

Infancty - 5 yrs of age
disconjugate asymmetrial multiplanar nystagmus
neuroimaging to rule out monocular nystagmus of childhood (neuroblastoma, chiasmal glioma)

Front 6

Neuroblastoma causes which eye movement disorders (3)

Back 6

ocular flutter (horizontal) or opsoclonus or nystagmoid movements

Front 7

Downbeat nystagmus causes (4)

Back 7

1. etoh
2. lithium
3. arnold chiari (most common structural)
4. spinocerebellar deg

Front 8

Name 3 variants of spinocerebellar degeneration and identify the one that has ocular changes (name them)

Back 8

spinocerebellar (SCA3, SCA6 – *SCA7* )
*optic atrophy & retinal pigm. changes

Front 9

Name causes of bitemporal hemianopia besides a chiasmal lesion

Back 9

1. Tilted disc (pseudo hemianopia: nasal tilt)
2. Dermatochalasia (temporal hooding)
3. Sectoral RP
4. Interotemporal location of SCHISIS
5. Enlarged blind spot with MEWDS

Front 10

Name several causes of impaired vertical gaze

Back 10

Juvenile Gaucher syndrome (storage disorder), like Nieman Pick
PSP (mostly downgaze)
Sylvian aqueduct

Front 11

What causes a WEBINO?

Back 11

Affect convergence pathway that is more anterior (midbrain) rather than more posterior + bilateral MLF lesions causing bilateral INO

Front 12

Name the ocular findings of Bassen Kornzweig and what is the cause

Back 12

Bassen-Kornzweig syndrome is caused by deficiency in beta-lipoprotein resulting in intestinal malabsorption.

1. Inheritance is AR.
2. Signs. Spinocerebellar ataxia, ptosis and progressive external ophthalmoplegia.
3. Diagnostic tests. The blood film shows acanthocytosis
4. RP develops towards the end of the first decade; the pigment clumps are often larger than in classic RP and are not confined to the equatorial region. Peripheral white dots are also common.
5. Treatment with vitamin E, if instituted early, may be beneficial for neurological disability.

Front 13

What causes Refsum's. What are the ocular findings?

Back 13

Inborn error of metabolism due to a deficiency in the enzyme phytanic acid 2-hydroxylase resulting in the accumulation phytanic acid in the blood and body tissues.

1. Inheritance is AR.
2. Signs include polyneuropathy, cerebellar ataxia, deafness, anosmia, cardiomyopathy and ichthyosis.
3. Diagnostic tests. Lumbar puncture shows elevated CSF protein in the absence of pleocytosis (cytoalbuminous inversion).
4. RP develops in the second decade and is characterized by generalized 'salt-and-pepper' changes.
5. Other ocular features include cataract, miosis and prominent corneal nerves.
6. Treatment, initially with plasmapheresis and later with a phytanic-acid-free diet

Front 14

Infant has tonic downward deviation of eyes (sunsetting). What must you rule out

Back 14

Compression on midbrain/PC and intraventricular Hg

Front 15

Tonic deviation of the eyes to the right. 3 causes

Back 15

1. lesion in right frontal eye fields
2. seizure in left frontal eye fields
3. lesion of left PPRF/CNVI causing deviation to contra side

Front 16

Patient you suspect has IIH but CSF protein is high and ventricles enlarged. How does this change your diagnosis?

Back 16

IIH has normal/small ventricles.
Cannot have protein in CSF as this will osmotically raise intracranial pressure

Front 17

Patient with bilateral macular exudates and bilateral CNVI palsy. Diagnosis?

Back 17

IIH with chronic optic nerve swelling causing mac exudates and increased intracranial pressure causing CNVI palsies

Front 18

Name 4 causes of optic nerve hypoplasia in newborn

Back 18

Maternal DM – superior segmental hypoplasia
Maternal ETOH
Hypopituitarism – Demorsier: get endo asap
Maternal use of Dilantin

Front 19

5 classes of diseases causing bilateral cecocentral scotoma

Back 19

1. Nutritional/toxic: ethambutol, Isoniozid, azothiaprine, MTX
2. LHON, dominant optic atrophy
3. Neuroretinitis (pap mac bundle)
4. Infiltrative: leukemia, lymphoma
5. MS

Front 20

Suspect nutritional/toxic cause of bilateral cecocentral scotoma. Tests?

Back 20

Can measure: folate/B12 (B1 and B6 less commonly), LFTs for etoh and toxic exposures, Cu/Zinc (penicillamine chelates copper)

Front 21

NAION: common presentation of fundus, risk factors, % risk for other eye, and causes.

Back 21

Optic disc edema common
Visual less severe than AION
Small cup to disc is risk factor
15% to next eye within 5 years
can be due to anemia or hypotension or chemo agents

Front 22

What EOM movements would you specifically look at for abberrant regeneration

Back 22

1. adduction for miosis
2. downgaze for lid retraction

Front 23

What types of CNIII palsy require imaging

Back 23

1. progression to pupillary involvement
2. incomplete (partial)
3. aberrant regeneration

Front 24

Name 1 cause each where extraocular muscles will affect IOP

Back 24

1. thyroid - tight muscles cause increase
2. CNIII palsy where lack of tone causes decreased IOP

Front 25

Basilar tip aneurysm can cause this pupillary abnormality

Back 25

Parasympathetic dysfunction with mydriasis only

Front 26

For optic neuritis, vision gets worse within what time period, how many improve within 1 month, what % have fundus findings, and which eye movements cause the most pain?

Back 26

Usually progress over a few days to one week
Within 1 month, 80% improve
EOM worse on adduction and elevation (MR and SR blend with nerve sheath)
Swollen disc 1/3 (ONTT)

Front 27

Patient with MG is complaining of vertical diploplia and experiences a worsening of symptoms (muscular) on steroids. Check of reflexes reveals abnormal reflexes (hypo or areflexic). What is in your differential now?

Back 27

Lambert Eaton
MFS

Front 28

What is Cogan's dictum?

Back 28

Asymmetrical OKN response on IPSI side of parietal lesion allows one to distinguish parietal vs occipital lesion.

Front 29

What is postfixation loss?

Back 29

In complete bitemporal hemianopia, when you fixate on an object that is close, it falls on temporal fields and you can see it; once it is far enough away, it falls on nasal fields (chiasmal lesion) which are affected and is now within a blind field, causing loss of fixation

Front 30

Patient has proposagnosia. Where is lesion

Back 30

bilateral occipital temporal lobes

Front 31

What is the Riddoch phenomenon?

Back 31

Can perceive objects within the blind field only if they are moving.

Front 32

What is Anton's syndrome?

Back 32

denial of blindness in someone with occipital infarct

Front 33

What is Benedikt's syndrome

Back 33

IPSI CNIII
Contra tremor
Contra sensory loss (medial lemniscus)

Front 34

Young man with central scotoma and hyperemic disc with telangiectatic vessels. What does FA show? Name 3 mutations and identify which is most common, which has worst prognosis/best prognosis

Back 34

FA: no leaking from optic nerve

11778 - most common
14484 - best prognosis
3460 - worst/rarest

Front 35

What component of gaze do the vermis and flocculus control?

Back 35

Vermis - metricity (e.g hypermetric saccades)

Flocculus - cause of gaze evoked nystagmus

Front 36

Patient presents with acute CNVI palsy. What must you do on exam?

Back 36

1. CNVII - check for hemifacial spasms
2. Check taste (superior salivatory nucleus)
3. Check Check lacrimation (salivary nucleus?)
4. CNvIII - check hearing.

Front 37

Name 2 other disease associations connected with IIH

Back 37

1. Thyroid dysfunction
2. Sleep apnea

Front 38

Name 4 brain structures that physiologically calcify

Back 38

1. pineal gland
2. vascular structures
3. dura (e.g falx)
4. choroidal plexus

Front 39

What must you measure for anyone that shows up with bilaterally swollen optic discs?

Back 39

Blood pressure!!!!

Front 40

If something is brighter on CT, MRI with and without gado, how do you describe it?

Back 40

1. CT: hyperdense
2. MR without gado: hyperintense
3. MR with gado: contrast enhancing

Front 41

To differentiate PSP from Parkinson's name ocular findings

Back 41

PD: hypometric saccades, blepharospasm, accommodative insufficiency, square wave jerks, UPGAZE defecits

PSP: DOWNGAZE deficits (dirty tie syndrome), accommodative insufficiency

Front 42

Maneuver to differentiate CNVI versus gaze palsy (PPRF)

Back 42

If can overcome palsy with doll's head, then PPRF lesion. If CANNOT, then CNVI palsy.
* VOR can bypass PPRF but no CNVI

Front 43

What is the Pulfrich phenomenom?

Back 43

In patients with MS, optic nerve conduction is delayed and results in a stereoillusion whereby a swinging pendulum is seen as an ellipse rather than a linearly moving target

Front 44

Where is the lesion in oculopalatal myoclonus and describe the neuroanatomy?

Back 44

Malloret triangle.

dentato-rubro-olivary pathway connecting the dentate nucleus (DN) of the cerebellum of one side with the red nucleus (RN) and the inferior olivary nucleus (ION) on the other side, via the superior cerebellar peduncle, the central tegmental tract and the inferior cerebellar peduncle, respectively

Front 45

Patient with sudden onset unilateral NLP. Main causes?

Back 45

1. PION
2. GCA/ NMO
3. vasculitis
4. LHON (later age)
5. Infectious optic neuropathy
6. Infiltrative optic neuropathy
7. Autoimmune/paraneoplastic optic neuropathy (CAR, MAR, autoimmune)

Front 46

on MRI you see more subarachnoid fluid around one optic nerve. Why?

Back 46

Atrophic nerve will have larger subarachnoid fluid cuff

Front 47

You suspect a paraneoplastic/autoimmune optic neuropathy in NLP patient losing vision in the other eye. Investigations and tx?

Back 47

Tx with high dose prednisone

LP for autoimmune/paraneoplastic antibodies: if positive treat with IVIG or Rituxan

Front 48

What is pituitary apoplexy after pregnancy called?

Back 48

Sheehan's

Front 49

What is the triad of pituitary apoplexy

Back 49

VF loss (bitemporal), diploplia (CNIII or VI or complete), headache

Front 50

Name
1. what pituitary lies in
2. what covers pituitary
3. 2 structures anterior to pit
4. 2 structures posterior to pit

Back 50

1. sella turcica
2. diaphragm sella
3. anterior clinoid, planum sphenoidale
4. posterior clinoid, petrous apex

Front 51

Increased ICP causes which false localizing signs (3 most common cranial nerves)

Back 51

6 > 4 > 7

Front 52

Most common false localizing sign of decreased ICP

Back 52

6th nerve palsy/diploplia

Front 53

Name several causes of decreased ICP (4)

Back 53

1. medication (mannitol)
2. surgical (LP, shunt)
3. traumatic (CSF leak)
4. spontaneous erosion of dural shunt