See discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/15489779 Cerebral achromatopsia as a presentation of Trousseau's syndrome Article in Postgraduate Medical Journal · February 1995 DOI: 10.1136/pgmj.71.831.44 · Source: PubMed CITATIONS READS 14 59 4 authors , including: Richard W Orrell Martin N Rossor University College London University College London 180 PUBLICATIONS 10,022 CITATIONS 946 PUBLICATIONS 75,131 CITATIONS SEE PROFILE SEE PROFILE All content following this page was uploaded by Martin N Rossor on 15 May 2014. The user has requested enhancement of the downloaded file.
Downloaded from pmj.bmj.com on July 16, 2011 - Published by group.bmj.com 44 Orrell, James-Galton, Stevens, Rossor Cerebral achromatopsia as a presentation of Trousseau's syndrome Richard W Orrell, Merle James-Galton, John M Stevens, Martin N Rossor Summary four years previously, and had been taking A 67-year-old man developed a sudden 300 mg aspirin daily. onset of achromatopsia. Magnetic reson- On examination he appeared well, with a ance imaging showed occipital lobe regular pulse, blood pressure 160/100 mmHg infarction. Repeated episodes of neuro- normal heart sounds, and no carotid bruits. logical deficit referable to the posterior Neurological examination was normal, but for circulation initially suggested an embolic absent colour vision and a partial right source, but subsequently proved to be due hemianopia. Visual acuity was 6/18 bilaterally. to a coagulopathy related to a carcinoma Two weeks after the onset of visual distur- of the bladder. This has implications for bance he developed a sudden onset of upper the management of patients presenting motor neurone weakness in the right arm and with achromatopsia, and progressive or face, with reduced sensation in the right arm, recurrent neurological episodes, and in following a bout of heavy coughing. Investiga- particular the use of anticoagulation in tion at this time demonstrated normal full this situation. blood count, erythrocyte sedimentation rate, prothrombin time, serum urea and electrolytes, Keywords: cerebral achromatopsia, Trousseau's syn- and glucose. Treponemal serology was drome, bladder carcinoma, disseminated intravascular negative. Chest radiograph was normal. Com- coagulation puted tomographic (CT) scan of the brain showed an area of presumed recent infarction involving the left occipital lobe and posterior Introduction part of the temporal lobe. Magnetic resonance (MR) scan the next day showed bilateral 'Cerebral achromatopsia is a syndrome in which the patient loses the ability to see colours occipital lobe infarction, mainly below the calcarine fissure (see figures). MR angiography after cortical damage. This loss may be com- plete or partial, and it may or may not be showed the posterior circulation to be normal, accompanied by other visual defects'."2 We with no evidence of basilar artery thrombosis. describe a case of this unusual but readily Doppler examination of the carotid arteries showed a 30% internal carotid stenosis on the recognisable clinical syndrome, and its associa- tion with a paraneoplastic coagulopathy. right, and 55 % on the left. There was no clinical or electrocardiographic evidence of an arrhythmia, and an echocardiogram showed no Case report evidence of a cardiac source of emboli. A 67-year-old man was travelling as a front seat St Mary's Hospital, passenger in a car when he experienced a Praed Street, London, sudden brilliant flash of white throughout his NEUROPSYCHOLOGICAL ASSESSMENT UK vision, followed by a kaleidoscope effect, with A detailed neuropsychological assessment was Department of carried out at the time of the original visual criss-cross lines and bright colours. The Neurology RW Orrell experience lasted about 20 seconds, and he was deficit. On the Wechsler Adult Intelligence MN Rossor Scale (Revised) he obtained a verbal IQ of 128.3 left with some fogginess of vision, and was only Department of able to see images in black and white. Three There was no evidence of generalised intellec- Neuroradiology weeks previously he had experienced an epi- tual impairment. Recognition memory for ver- JM Stevens sode of weakness in the right hand, lasting for bal material was excellent, he scored in the days, but otherwise had no previous superior range on the Warrington Recognition Department of two Clinical neurological symptoms. Over a period of two Memory for words.4 Neuropsychology, The weeks there was some improvement in his Test of primary visual function were per- National Hospital, formed. Reading acuity on the Ffookes sym- vision, but persistence of the loss of colour Queen Square, bols test was 6/18. Shape discrimination was vision. London, UK within normal limits.5 Shape detection on the Eighteen months previously he had present- M James-Galton VOSP (Visual Object and Space Perception) ed with haematuria. Cystoscopy demonstrated figure-ground was unimpaired.6 Colour dis- Correspondence to a multifocal transitional cell carcinoma on the Dr Richard W Orrell, left wall of the bladder which was in the early crimination was severely impaired. He was Academic Unit of invasive stage. This was being controlled by unable to pick out any numbers on the Ishihara Neuroscience, Charing Cross test. He had great difficulty on the Farnsworth repeated cystoscopy and fulguration. He had Hospital, Fulham Palace Road, London W6 8RF, UK 100-Hue test, error score 784.78 There was no ischaemic heart disease, with occasional evidence of visual disorientation; he was able to angina, had coronary artery by-pass grafting Accepted 11 August 1994
Downloaded from pmj.bmj.com on July 16, 2011 - Published by group.bmj.com Cerebral achromatopsia 45 MRI, 1.5 Tesla, with Gadolinium enhance- Figure 1 ment, showing features of bilateral occipital lobe infarc- Figure 2 MRI, coronal section of the brain, showing tion, more marked on the left features of bilateral occipital lobe infarction, mainly below the calcarine fissure count scattered dots accurately, missing only those which fell within his visual field defect. Tests of higher visual processing from the period of weeks had some recovery of vision in VOSP6 were performed. He was completely left homonymous fields, but remained the unable to see any form of a letter on the drowsy, and the heparin was discontinued. Incomplete Letter task. His performance on Within two weeks he developed a deep vein the Silhouettes test was very poor (4/30). On thrombosis of the right leg, with gangrenous the Progressive Silhouettes test he was slow to areas in the toes, and as there was some identify the objects even when complete. He recovery in his conscious level, heparin was also made errors of identification of simple line recommenced. Within three months of the drawings of objects. He showed some degree of initial presentation of visual disturbance he prosopagnosias, being able to identify only 1/12 developed significant haematuria, with further Famous Faces and he complained of difficulty evidence of disseminated intravascular coagu- in recognising people. lation (DIC). Over a period of one week the The interpretation of his neuropsychological coagulopathy progressed. This failed to re- assessment is that the only elements of cortical spond to treatment with platelets and fresh blindness present were a severe achromatopsia frozen plasma and he died peacefully. and a mild impairment of acuity. In addition he Post-mortem examination showed the cause had an aperceptive agnosia. The degree of of death to be a pulmonary embolus, with a impairment of his primary visual processing thrombosed right external iliac vein. There was was insufficient to account for his poor perfor- a stage 4 bladder carcinoma, invading through mance on tests of visual object processing. the bladder wall into the perivesical fat, with a right hydronephrosis and hydroureter. His- tology showed an ulcerated poorly differenti- PROGRESS In view of the recurrent episodes of neuro- ated transitional cell carcinoma extending into the perivesicular fat, and lymphatic channel logical deficit, which were felt to be possibly embolic, he was started on an infusion of invasion. There was no evidence of metastatic spread. On examining the brain, there were heparin at 10 000 units over 24 hours, with bilateral basal occipital infarcts and bilateral caution because of the haemorrhagic nature of the infarct, and his previous haematuria. The parietal infarcts, all being recent, with signs of colliquative necrosis. Additional infarcts were right arm weakness resolved over three days, with a residual mild right facial weakness. He present in the kidney and spleen, with gang- renous infarction of the right forefoot and toes was commenced on warfarin anticoagulation with careful control. of the left foot. There was no evidence of an embolic source in the heart or great vessels. Two weeks later, having developed mild haematuria and having discontinued the war- farin himself, he was found wandering at night, Discussion aphasic and agitated. He had a left upper motor facial weakness, and a progressive left hemi- Cerebral achromatopsia has been recognised plegia. He had complete cortical blindness, for many years, with debate over the existence with small pupils, absent gag reflex, and brisk of a specific cortical colour centre. Clinical jaw jerk. CT scan at this stage showed extensive evidence links the anterior inferior part of the bilateral occipital infarction. He was again occipital lobe with colour perception in man. commenced on a heparin infusion, and over a Bilateral lesions at this site may cause achroma-
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