Blackwell Science, LtdOxford,
UKCHACephalalgia0333-1024Blackwell Science, 2005263345348Clinical CorrespondenceProsopagnosia as symptom of migraine with auraPS Sándor et al.
CLINICAL CORRESPONDENCE
Prosopagnosia as symptom of migraine with aura: a case report PS Sándor1,3, GP Morath1, K Hess1, H Kaube3, RM Agosti1,2 & M Regard1 1 3
Headache & Pain Unit, Neurology Department, University Hospital Zurich and 2Zurich Hirslanden Headache Centre, Zurich, Switzerland, and Headache Group, The National Hospital for Neurology and Neurosurgery, London, UK
Peter S Sándor MD, Headache & Pain Unit, Neurology Department, University Hospital Zurich, Frauenklinikstrasse 26, 8091 Zurich, Switzerland. Tel. + 41 1255 5514, fax + 41 1255 4507, e-mail
[email protected]
Prosopagnosia is a condition during which a face is recognized as such, but not as the face of a person familiar to the observer (1). Patients suffering from this condition recognize their fellow humans by means of their voice, movements, clothing, and so called paraphernalia (glasses, beard, earrings, etc.). Most likely, a dysfunction or lesion of the right fusiform/lingual gyrus is sufficient to cause prosopagnosia, at least transiently. The most common cause is ischaemic, but other causes for disturbed function in the respective region, e.g. tumours, trauma, intoxication, have been reported (2). Here we report the first patient with stereotyped prosopagnostic aura.
Case report A 58-year-old left-handed man, a tax consultant in his own small firm, was treated in our tertiary care headache centre at Zurich University Hospital. He had suffered from migraine without aura since the age of 20, with an attack frequency of about one per month, with several attack-free periods of a few years. He reported a transient increase in attack frequency around the age of 25, because of which he was put on prophylactic treatment for a few months (no further details remembered). Since the age of 52, all of his migraine attacks had become associated with stereotyped auras, starting with a propagation of ‘coloured rings’, extending from bottom to top in both visual fields, leading after about 10 min to a concentric restriction of the visual fields, sparing more than 45∞ of central vision. He was unable to specify the colours, but stated that the phenomenon was certainly coloured rather than black and white, without any predominant colour. At this stage the patient was unable to recognize faces of familiar persons, although he could see them as a whole, and described them as ‘whitish disks’ or ‘eggheads’. To recognize a familiar person he would © Blackwell Publishing Ltd Cephalalgia, 2006, 26, 345–348
have to use their voices, movements and clothing. If he was concentrating on a certain part of the face for a while, he was able to recognize it clearly as such (e.g. the nose or the mouth). He could clearly see other parts of the body, the environment, and objects, in colour and had no problem in recognizing them. The prosopagnostic condition lasted an additional 10–15 min, and faded away over about 5 min while the visual field defect normalized. Mental status during such an episode could not be obtained, but in the interval there was no deficit in his status; this included strictly normal face recognition. Without any interval, there followed strongly pulsating headache of moderate intensity, with a duration of about 12 h when untreated. Headache localization was unilateral in about 80%, and about equally often on each side. In the remaining cases headache was bilateral. It was aggravated by physical activity and accompanied by phono- and photophobia, but no nausea. His usual treatment consisted of analgesics (either 1000 mg of acetylsalicylic acid or a combination of 500 mg paracetamol, 50 mg caffeine and 4 mg chlorphenamine). He reported stress, particularly during work, and weather changes as typical triggers. Since the onset of migraine aura, migraine frequency was higher than before, with several attacks per month. In the 6 months before presentation, on a combination of a b-blocker (nadolol 30 mg/day) and a selective serotonin reuptake inhibitor (fluoxetine 20 mg/day) prescribed by the general practitioner, only three attacks had occurred. About 5 months before presentation to the headache clinic, the patient had suffered a single episode of a typical transient global amnesia, which was diagnosed during a brief hospitalization in our neurology department (normal medical and neurological examination, normal computed tomography). Family history was negative concerning migraine. His mother died from heart failure at the age of 74, 345
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his 49-year-old brother and his 86-year-old father did not have any relevant medical problems. In his past medical history the patient reported having an allergy to penicillin, a tendency to depressed mood during the winter months, an appendectomy and an operation for an inguinal hernia. Two documented neurological examinations had been normal (when he presented, 55 years old, in the headache unit as well as during his transient global amnesia); apart from slightly elevated blood pressure (about 140/90 mmHg), medical examination was normal, with normal cardiac auscultation and without any rumors over the carotid arteries. Magnetic resonance tomography showed a few small vascular lesions bilaterally in the frontal white matter as well as one small vascular lesion in the right cerebellar hemisphere. Mesiotemporal structures were normal. Neurovascular investigations (Doppler, duplex) were normal, without any signs of obstruction in the posterior circulation. The EEG did not show any abnormalities. Laboratory analyses showed slightly elevated liver enzymes [aspartate aminotransferase 84 U/l (
