Eclamptogenic Gerstmann's syndrome in combination with cortical agnosia and cortical diplopia.
Cortical blindness is defined as a loss of vision due to bilateral retrogeniculate lesions (geniculocalcarine blindness). Gerstmann's syndrome is a combination of disorientation for left and right, finger agnosia, and profound agraphia, alexia, and acalculia. It is due to a lesion in the left angular gyrus, situated at the confluence of the temporal, parietal, and occipital lobes. We report on a patient who suffered from severe underdiagnosed eclampsia and who developed bilateral extensive medial temporal, parietal, and calcarine ischemic infarctions during an eclamptic fit. In addition, ischemia destroyed the left angular gyrus. The combination of these lesions led to Gerstmann's syndrome with additional cortical agnosia and cortical diplopia. For the first few months following the ischemic insult, the patient had been cortically blind. Thereafter, the patient slowly regained a visual acuity of 0.1 in both eyes. She then experienced monocular and binocular diplopia. Her ocular motility was normal; there was no phoria or tropia. Monocular and binocular diplopia slowly became less severe over the following year. Now, 2 years after the incident, the patient has a visual acuity of 0.2 in both eyes and no double vision. However, the handicapping symptoms of Gerstmann's syndrome, which make leading a normal life impossible, have persisted--the patient still cannot cope alone, mainly due to the severe disorientation for left and right. The picture of cortical agnosia, cortical diplopia, and Gerstmann's syndrome is a very rare combination. Visual recovery and rehabilitation in cortical blindness are severely affected and made difficult by the symptoms of Gerstmann's syndrome. In our case the reason for such a dramatic clinical picture was eclampsia, whose prodomes had not been diagnosed in time.
D000328:Adult; D000381:Agraphia / Q000209:etiology; D001072:Apraxias / Q000209:etiology; D001766:Blindness / Q000209:etiology*; D002545:Brain Ischemia / Q000150:complications; D002540:Cerebral Cortex / Q000473:pathology*; D002544:Cerebral Infarction / Q000150:complications; D004172:Diplopia / Q000209:etiology*; D004411:Dyslexia, Acquired / Q000209:etiology; D004461:Eclampsia / Q000150:complications*; D005260:Female; D005862:Gerstmann Syndrome / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D011247:Pregnancy; D014792:Visual Acuity