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Migraine aura masquerading as Balint’s syndrome
  1. PARVAIZ A SHAH,
  2. A NAFEE
  1. Division of Neurology, Department of Medicine, Government Medical College and Associated SMHS Hospital, Srinagar, Kashmir, J and K 190001, India
  1. Dr Parvaiz A Shah, Firdousabad, Batmaloo, Srinager, Kashmir, J and K 190001, India. Telephone 0091 194 452379.

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Migraine is a common neurological disorder with a prevalence of 0.5% to 2% in the general population.1 In one fourth of total migraineurs, headache is preceded by an aura.2 We describe a patient with recurrent episodes of migraine in whom headache was preceded by a constellation of visual symptomatology which constituted salient components of Balint’s syndrome. This syndrome, consisting of a triad of simultagnosia, optic ataxia, and oculomotor apraxia, is seen with bilateral lesions of occipitoparietal cortices affecting connections between visual cortical regions and the frontal eye field.3

A 29 year old female teacher presented with an 8 year history of paroxysmal alternating hemicranial and throbbing headache which was often associated with nausea and photophobia. Patients fulfilled the requisite criteria for establishing the diagnosis of migraine with aura as devised by the International Headache Society (1988).4She used to have six to eight episodes of headache a month. There was no history of status migranosus during these years. On several occasions, headache was preceded by a peculiar constellation of visual symptomatology comprising distortion of visual images followed by inability to perceive simultaneously objects in the visual field and touch an object under direct visual guidance. However, she could see the component parts of objects during the episode. These visual symptoms lasted for about 10–25 minutes and were followed by a hemicranial, throbbing headache which was often associated with nausea, photophobia, and occasionally vomiting. Headache used to last for about 4 to 18 hours and would respond to either ergot drugs or sumatriptan, especially if taken at the beginning of the episode. Occasionally these visual symptoms were not followed by headache. The patient would not lose contact with the environment during or after the visual symptoms. Her mother and two younger sisters were also having paroxysmal episodes of common migraine.

Her general physical and neurological examination in between the episodes was unremarkable. Neurological examination during the aura symptoms disclosed that she was unable to see simultaneously all the objects in the visual field (simultagnosia). She did omit several words while reading a paragraph. However, she could comprehend and read each and every word individually. On being shown a complex picture comprising multiple subunits she was not able to comprehend and perceive the entire picture but was able to perceive all the components of the picture individually (seeing in piecemeal). These aforementioned features were consistent with simultagnosia. Besides simultagnosia, she had optic ataxia as evidenced by her inability to coordinate hand and eye movements. Optic ataxia was tested as follows: each eye was tested separately and the hand ipsilateral to the eye being tested was used. The target stimulus was a 5 mm long pin with a white head placed at preselected locations. The patient was asked to touch this pin with her index finger without shifting her gaze from the fixation point. The patient had difficulty in performing this test but had no problems in reaching out to her own body parts or an auditory stimulus with her eyes closed. These features were consistent with optic ataxia. Moreover, gaze apraxia was evident by her inability to look at an object on command. However, she could do it spontaneously. In addition, she had impaired smooth pursuit and voluntary saccades in all directions. Reflex eye movements were normal. Visual acuity during the episode was 6/6 bilaterally. Visual fields were also normal during the episode as demonstrated by the confrontation method. Ophthalmological examination, including perimetry performed during a symptom free period, was normal. There was no clinical evidence of Gerstmann syndrome, prosopognosia, object agnosia, or colour agnosia. Her cranial CT and magnetic resonance angiography were unremarkable.

Electroencephalography was also non-contributory. The frequency of visual aura symptoms and headache decreased considerably after the patient was started on flunarizine at a daily dosage of 10 mg at bed time.

The visual impulses, after being received by the primary visual cortex (Brodmann area 17), are interpreted and integrated in visual association areas 18 and 19. Brodmann area 19, in turn, is connected with the angular gyrus and frontal eye field by virtue of association fibres. Any lesion in the visual association areas or their connections would result in impaired integration of visual impulses despite normal visual acuity.

The visual symptom complex in this case possibly represents an aura of migraine. The pathogenesis of migraine aura has been a debatable issue.5 in this case it is suggested that the pathophysiological process of migraine aura results in a disconnection syndrome by involving visual association areas and their association pathways. Optic ataxia, gaze apraxia, and simultagnosia seem to represent a dissociation of visual information from the frontal eye field and dorsal parietal regions.

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