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Alexia Without Agraphia

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Alexia Without Agraphia Neuroradiology(1992) 34:210-214 Neuro radiology Springer-Verlag 1992 Alexia without agraphia D. J. Quint I and J. L. Gilmore 2 1Division of Neuroradiology,Department of Radiology,University of MichiganHospitals, Ann Arbor, Michigan,USA 2Department of Neurology,University of Rochester, Rochester, New York, USA Received: September 16, 1991 Summary. Two new cases of alexia without agraphia are mentation and right visual complaints. She had suffered presented. Pertinent clinical findings, anatomy, pathophy- from extensive atherosclerotic peripheralvascular disease siology and differential diagnoses are reviewed. The im- involving both the systemic and cerebral vasculature, portance of carefully examining the inferior portion of the necessitating carotid endarterectomy. The patient denied left side of the splenium of the corpus callosum on CT any "strokes", but did suffer from complications of scle- and/or MR scans in patients who present with this clinical roderma including gastritis, esophagitis and Raynaud's syndrome is stressed. phenomenon and had also had a cervical sympathectomy. The patient did report a vague episode of "loss of con- Key words: Alexia - Agraphia - Disconnection syndrome sciousness" 4-5 months before admission. - Magnetic resonance imaging - Computed tomography General physical examination showed some edema of the legs. Neurologic examination revealed the patient to be oriented to person and place, but not to time. She could Alexia without agraphia or pure word blindness is con- sidered one of the classic disconnection syndromes. Pa- identify letters, but could not read. She could write her name and spell simple words forwards and backwards. Cra- tients retain the ability to write, but are unable to read nial nerve (II-XII) examination demonstrated a right ho- (even words that they have just written) and often have monymous hemianopia. Cerebellar function tests demon- right homonymous hemianopia. This disorder results strated decreased rapid alternating movements on the left. from the loss of visual input to the language area [usually Laboratory studies, with the exception of mild elevation the dominant (left) angular gyrus] without involvement of of BUN and creatinine, were normal. The EEG demon- the language area or output from the language area to the strated slowing over the left posterior temporal and occipi- motor cortex. The ability to process visual input into lan- tal regions consistent with a structural disturbance. Mild guage (i. e., reading) is lost in these patients. The pathways right temporal slowing was also demonstrated. Head CT involved in this syndrome are believed to be localized to (Fig. 1) demonstrated a left occipitalinfarct. the inferior portion of the splenium of the corpus callosum Formal neuropsychometric testing confirmed alexia on the side of the dominant (usually left) cerebral hemis- without agraphia. phere. Therefore, head CT and MR examinations in pa- tients who present with this symptom complex should be scrutinized for abnormalities in this region. Case 2 This report of two additional cases of alexia without A 79-year-old right-handed man with a history of hyper- agraphia is presented to show what we believe to be the tension was otherwise well until 2 months prior to ad- first published example of this syndrome demonstrated on mission when he experienced an episode of "jamais vu": MRI and to stress the importance of understanding the he could recognize his surroundings, but they seemed un- underlying anatomy thought to be involved so that the ap- familiar to him. This episode lasted several days and re- propriate regions are studied. curred i month prior to admission. The patient experi- enced an additional episode of "feeling odd" which Case reports prompted his admission to hospital 2 days later. While the patient did report a 2-month history of intermittent loss of Case 1 right vision, diplopia, vertigo, nausea and problems with speech, he denied loss of consciousness, seizures, episodes A 77-year-old right-handed woman with a long history of of weakness or symptoms consistent with stroke. Apart atherosclerotic peripheral vascular disease and scleroder- from the history of elevated blood pressure, his past medi- ma presented to the psychiatry service with decreased cal history was unremarkable. 211 Fig.1. Case 1. 77-year-old right-handed woman with alexia without agraphia and right homonymous hemianopia. CT dem- onstrates decreased attenuation with mild mass effect in the left posterior cerebral artery territory consistent with subacute infarction. Curved arrow indicates infe- rior portion of left side of splenium of cor- pus callosum Fig.2a-c. Case 2. 7%year-old right- handed man with alexia without agraphia and right homonymous hemianopia. Con- trast-enhanced CT demonstrates homo- geneous contrast enhancement of a sub- acute left posterior cerebral artery territory infarct involving a the left side of the inferior portion of the splenium of the corpus callosum (curved arrow) and b the left parieto-occipital and left pos- teromedial temporal regions (asterisks). A smaller right occipital infarct is also seen (straight arrow), c Proton density weighted MRI (1.5 T, 2000/30) also shows the right occipital and left parieto-occipi- tal and medial temporal lesions The patient was normotensive. General physical exam- to-nose movements were decreased on the left and there ination was normal. Neurologic examination revealed the was mild gait ataxia. patient to be oriented to person, place and time. Speech Laboratory studies were normal. Head CT (Figs. 2 a, b) was normal without dysarthria. The patient was unable to demonstrated an area of pathologic contrast enhance- read, although neuropsychometric testing revealed that ment in the deep left parieto-occipital region most consis- he could identify individual letters. He was unable to tent with a subacute left posterior cerebral artery distribu- name objects and to perform simple (seventh grade edu- tion infarct. MRI (Fig. 2 c) confirmed this. cation) arithmetic. He could recognize colors and write Formal neuropsychometric testing interpretation con- and did not demonstrate left/right confusion. He was able firmed alexia without agraphia. Higher cortical function to follow simple commands. Cranial nerve (II-XII) exam- and memory testing suggested additional bilateral cere- ination demonstrated a right homonymous hemianopia, bral hemisphere dysfunction. decreased pin-prick and touch sensation in the distribu- tion of all three divisions of the left trigeminal nerve and a Discussion right central facial weakness. Sensory examination dem- onstrated decreased vibration sense in the lower limbs Disconnection syndromes are the result of interruption of bilaterally (left more than right) and decreased pin-prick inter- or intrahemispheric white matter tracts [1]. Alexia and touch sensation on the right side of the body. Finger- without agraphia is a rare but "classic" disconnection syn- 212 drome [2]. Ddjerine [3] was the first to perform a postmor- sal branches arising in the region of the quadrigeminal tem examination on a patient with this syndrome. He plate cistern) and the left visual cortex (via calcarine and found an infarct of the left posterior cerebral artery terri- parieto-occipital branches) [12]. tory involving the splenium of the corpus callosum and the medial portion of the left occipital lobe. He and others [4, 5] elaborated on the work of Wernicke [6], concluding that Pathology loss of visual input from both occipital lobes to the domi- In reported cases of alexia without agraphia, infarction in nant angular gyrus (language area) results in the inability the distribution of the left posterior cerebral artery (PCA) to read without affecting the language area itself or the (Fig. 3 a) it the most common pathologic process [13-15]. output from the language region to the motor region (the The left visual cortex is supplied directly by the left PCA ability to write and speak). and compromise of this artery therefore results in varying Over the past 100 years, alexia (the inability to compre- degrees of infarction of this region, destroying input from hend written material) has been classified in several dif- this area to the angular gyrus and causing the right ho- ferent ways, which essentially divide it into two major monymous hemianopia commonly associated with this groups: alexia associated with or unassociated with agra- syndrome. Infarction of a portion of the splenium of the phia [7-9]. Recently, the alexias have been further cate- corpus callosum affects the fiber tracts from the right vis- gorized by a neurolinguistic approach specifying the type ual cortex to the left angular gyrus without affecting the of reading disability [9]. One such subclassification right visual cortex itself. Therefore, the end result is that scheme, that of Marshall and Newcombe [10], has gained vision (the right visual cortex) and language in the form of widespread acceptance. It divides the alexias into three speech and writing (left angular gyrus and the motor cor- groups including "visual dyslexia" (misreading a word by tex and its projections) are spared, but the patient is un- transposing letter positions, e.g., "exit" read as "next"), able to read, as visual information cannot be transmitted "deep dyslexia" (replacing a word with a synonym, e.g., to (has been "disconnected" from) the language area. "car" read as "auto") and "surface dyslexia" (misinter- In the rare reported cases of alexia without agraphia preting the sound of
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