57-year-old Woman with Unilateral Ptosis and Blurred Vision
Jeeyoung Oh, MD
Department of Neurology, Konkuk University School of Medicine
Case – History
F/57
10
일 전 아침 기상 시 오른쪽 윗 눈꺼풀이 부은 느낌과 함께 사물이 뿌옇 게 보임.
거울을 보니 오른 눈꺼풀이 쳐져 있어 개인 안과 의원을 다니면서 치료를받았으나 증상은 호전되지 않음.
5
일 전에는 오른 손의 저린감이 생겨 한의원에서 침을 맞았고 이후 저린감은 호전됨.
증상은 처음 발생 시와 큰 차이는 없으며 일중 변동은 없다고 함.Case - History
Past History
Diabetes mellitus : 2 months (no medication) Hypertension : 4 years (amlodipine)
Dysthyroidism (-) Previous trauma (-)
Previous operation (+): HIVD, 3 months ago
Family history : none
Review of System
Headache (-)
General weakness (-)
Blurred vision (+)
Diplopia (+)
Ocular pain (-)
Tinnitus (-)
Dizziness (+)
Dysarthria (-)
Dysphagia (-)
Dyspnea (-)
Palpitation (-)
Abdominal discomfort (-)
Arthralgia (-)
Myalgia (-)
Skin lesion (-)
Tingling sense (-)
Dysuria (-)
Bowel dysfunction (-)
Physical Examination
Vital Sign: BP 133/77 mmHg, PR 72 bpm, BT 36.3
‘C
Ptosis (OD)
Orbital swelling (-)
Proptosis (-)
Tenderness on eyeball pressure (-)
Conjunctival injection (-)
Orbital bruit (-)
Carotid bruit (-/-)
Neurological Examination
Mental status: alert Cranial function test
Isocoric pupil
Prompt direct and consensual light reflex (OU)
RAPD (-)
Color vision: normal
Gross confrontation: no visual field defect
Ptosis (+), interpalpebral fissure 7mm/11 mmNeurological Examination
Cranial function test
Hypesthesia, right forehead
Mastication force: normal
Equivocal nasolabial fold flattening, right
Weber test: midline
Rinne test: positive (AU)
Dysarthria (-)
Gag reflex (+)
Tongue protrusion with wiggling: normal range and speedNeurological Examination
Motor system
Muscle tone: normal
Muscle strength: all MRC grade 5Somatosensory system
Normal and symmetric in pinprick and vibration DTR: normoactive
Babinski (-/-)
Cerebellar function test: normal
Problem List
Right eye ptosis with lateral gaze limitation
Sudden onset
Painless
Pupil-sparing
Static course (no diurnal variation)
No other visual symptom
Ipsilateral facial hypesthesia
Transient right arm paresthesia
Differential Diagnosis
1. Cavernous sinus lesion
Tumor
Infection
Aneurysm
Carotico-cavernous fistula 2. Nonspecific orbit lesion
3. Ocular myasthenia
Diagnostic Plan
Brain MRI with angiography
Routine laboratory examination
Thyroid function testConnective tissue disease
Serum acetylcholine antibody
Repetitive nerve stimulation test
Serum Chemistry
list result unit references WBC 6.06 X 103/㎕ 4-10
Hb 15.0 g/dL 13-17
Hct 43.0 % 39-52
PLT 225 X 103/㎕ 140-400
ESR 4 mm/h 0-20
AST 20 IU/L 7-38
ALT 25 IU/L 4-43
BUN 13.7 mg/dL 8.0-22.0
Cr 0.9 mg/dL 0.7-1.4
CK 32 IU/L 58-348
LDH 333 IU/L 263-450
VDRL <0.1 R.U 0.1-0.5
list result unit references
RA 41 IU/mL 3-18
FBS 98 mg/dL 70-110
HbA1C 6.3 % 4.7-6.4
FANA Negative
ENA Negative
Free T4 1.44 ng/dL 0.8-1.86
TSH 1.29 uIU/mL 0.4-4.1
CSF study
RBC 0 0
WBC 0 < 3
Protein 46.8 mg/dL 15-45 Glucose 76.0 mg/dL
Repetitive Nerve Stimulation Test
Brain MRI
Brain MRI
TFCA
Coil Embolization
Progress
Subjective improvement of facial discomfort but no change of ptosis and blurred vision after coil embolization
Acetylcholine receptor antibody: 6.334 nmol/L
Pyridostigmine test – improved ptosis and objective visual symptoms
Chest CT
Follow up
Thymectomy
- Thymoma, type B2 (2.8 x 2.5 x 1.2 cm)
Complete remission with prednisolone and azathioprine
No generalization for a year
Check Point
1. 임상적으로 ocular myasthenia를 의심할 수 있었던 sign은 없었는가?
2. Aneurysm으로 환자의 증상을 설명할 수 있었는가?
3. 진단 algorithm에 문제는 없었는가?
Ocular manifestation of MG
Brief review
Ocular manifestation of MG
Ocular involvement eventually occurs in 90% of all myasthenic patietns.
75% of MG patients present with visual complaints of droopy eyelids or double vision.
80% of ocular MG patients will generalize within 2 years.
Ocular Signs of Myasthenia Gravis
Signs of fatigue Levator palpebrae Lid twitch, ptosis after sustained upgaze Orbicularis oculi Afternoon ectropion, peek sign
Extraocular muscles Intrasaccadic fatigue, rapid small saccade Signs of variability Levator palpebrae Lid hopping
Extraocular muscles Saccadic jitter Signs of adaptation Levator palpebrae Enhanced ptosis
Lid retraction contralateral to ptotic eye Extraocular muscles Hypermetric small saccade
Dissociated gaze-evoked jerk nystagmus
Signs of combined Levator palpebrae Lid retraction with Cogan’s lid twitch
Weakness and adaptation Extraocular muscles Saccadic hypermetria after edrophonium
Ptosis in Horner syndrome and MG
Ophthalmoparesis in MG
Double vision, dizziness, gait instability, visual blurring
Medial rectus, inferior rectus, and superior oblique may be more commonly affected.
Eye movement abnormalities of MG mimic any central and peripheral eye movement abnormalities.
Ptosis with ophthalmoparesis + orbicularis oculi weakness
Dynamic Eye Movement Abnormalities
after 15-minute-sleep after edrophonium
Bedside Ocular Examination in Myasthenia
Cogan’s lid twitch sign
Curtaining sign or enhanced ptosis
Peek sign
Ice pack test
Fatigue and Recovery bedside test
Toyka, K. V. Neurology 2006;67:1524
Cogan’s Sign
Transient lid retraction, then twitch or droop back to a ptotic
position when the patient first look down for a short period and then make a saccade back to primary position.
Although Cogan’s lid twitch is not pathognomonic sign, it is only
rarely associated with other cause of ptosis.
Enhanced Ptosis
Owing to Hering’s law of equal innervation, central compensation for unilateral ptosis leads to hyper-retraction of a less-affected lid.
When ptotic eyelid is manually elevated, the retracted normal eyelid droops.
Gorelick P, et al. Arch Neurol 1981;38:531.
Peek Sign
After complete initial apposition of the lid margins, they quickly (within 30 seconds) start to separate and the sclera starts to show (ie, a positive peek
sign).
Simel DL, JAMA 2005;193:1906-1924
