Tag: hysteric blindness

Monocular blindness with normal pupillary reaction: mistery pediatric case

In EnglishEN: A case of September 2016. Normal pupillary reactions in a blind eye! Quite a difficult case, which was not resolved. A 8yo girl presented with a history of complete vision loss in left eye. She was not sure was it rapid or slow vision loss. It could be a slow vision loss during 2 previous weeks. The history was not clear. Vision of right eye 1,0. Left eye – no light perception (!).

Pupillary reactions are presented on video. No APD or RAPD!!! But one can also notice, that the kid does not simulate. She doesn’t blink at all, when there is a rapid movement of my hand close to her eye.

Her history was unremarkable: no heat traumas, no headaches, no general body symptoms. Two weeks ago we have treated stromal keratitis of the same left eye. It could have been herpes. Otherwise the eye looked completely intact, including retina and optic nerve!

Upon consultation with my peers I had a following list of differential diagnoses: monocular hysteric blindness? monocular cortical blindness? monocular retrobulbar neuritis? Moyamoya disease?

“Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage.  Moyamoya disease was first described in Japan and is found in individuals around the world; its incidence is higher in Asian countries than in Europe or North America. The disease primarily affects children but it can also occur in adults. In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks (TIA, commonly referred to as “mini-strokes”), frequently accompanied by muscular weakness or paralysis affecting one side of the body. Adults may also experience these symptoms that arise from blocked arteries, but more often experience a hemorrhagic…”

The girl showed positive optokinetic nystagmus test in both eyes!!!

My additional research showed the necessity of the following data:

Additional Hx: loss of consciousness?
epilepsy drugs? Head trauma? Normal delievery?
Epilepsy? perinatal hypoxia? cardiac problems?
Previous surgeries?
infection? TB?

Additional exam: near pupillary response?
Seeing dynamic objects?
Seeing far objects? Can move with other eye closed?

Additional tests: DCT, RPR, LFTs, CXR
Additional investigations: CT (cerebral atrophy?)
VFT (other eye field defects?)

DDx: cortical blindness? functional vision loss?
Prognosis: ~40% of acquired pediatric cortical blindness have fair or good visual prognosis. Interval for recovery: 2 weeks – 5 months.

The case remained a mystery, as I lost contact with the parents and a patient. I still hope to get an answer some time later. The parents gave consent on publishing the materials.

in Russian / по-русскиRU: Случай от сентября 2016 г. Зрачковые реакции на слепом глазу.
Девочка 8 лет. 4 недели назад потеряла зрение на левый глаз. Возможно, резко, возможно теряла в течение 2 недель – анамнез не ясный. Зрение правого глаза – единица. Левого глаза – ноль, отсутствие светоощущения.
Зрачковые реакции представлены на видео. Дефектов зрачковой реакции обоих глаз не выявлено.
Анамнез не блещет особенностями. Нет головных болей, нет травм в анамнезе, и вообще ничего не беспокоит. Две недели назад девочке излечен едва выраженный стромальный кератит этого же глаза. Стромальный кератит мог быть герпетической этиологии.. Но в остальном глаз в идеальном порядке, роговица была чистая, как стёклышко, а сетчатка и зрительный нерв без патологии.

Неделю думал, что девочка в школу не хочет. Но как-то для трёх недель многовато. И видно, как она даже моргнуть не моргнула на махание рукой перед глазом. Я пытался вылечить ее волшебными каплями. Не вылечилась…

Для неврита маловато клиники, история нетипичная, и зрачки нормальные. Истерическая односторонняя слепота у детей встречается. Консультация невролога не была возможной – его не было. Так же в стране не было доступно ЭФИ. В идеальной ситуации, разумеется, требовалась МРТ ангиография, ЭФИ, +/- допплерография.

Родители дали согласие на публикацию видео с консультативными целями.