Nov 24, 1992 - and a 'magnetic' left hand whereby the hand followed the ... He was unable to copy hand postures ... the precise pathology remains uncertain.
Journal of the Royal Society of Medicine Volume 87 June 1994
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Section case reports
Corticobasal degeneration: a report of three cases
K A Muhiddin PhD MRCPl"3 R J Hardie MD MRCP2 V R Pearce FRCP"3 B J Kirby FRCP34 Departments of 'Medicine for The Elderly, 2Neurology and 3General Medicine, Royal Devon and Exeter Hospital, and 4Postgraduate Medical School, Exeter, UK
Keywords: corticobasal degeneration; progressive apraxic rigidity; alien hand syndrome
Introduction Corticobasal degeneration (CBD) is a degenerative neurological disorder with certain distinctive clinical features that has been recognized in the last 20 years, even though the precise pathology remains uncertain. It is probably under-reported as its clinical manifestations are readily confused by non-neurologists with commoner neurological conditions. We report three cases of this condition in which the diagnosis seems likely. The first case was initially thought to have sustained a cerebrovascular event, the second case was initially thought to be Parkinsonism, whilst the third one was thought to have had a cerebral vasculitis.
Case reports Case 1. A right-handed man aged 77 years presented with a 6 month history of progressive difficulty in using his left upper limb and deterioration in walking. Without looking at his left arm and leg he was unaware of their movements. His past medical history included hypertension treated with nifedipine, and a stroke causing a mild right hemiparesis 13 years earlier. On physical examination, tone was increased and moderate global weakness in the left upper and lower limbs with hyperreflexia and a left extensor plantar response. He was thought to have had another stroke but a cranial computerized tomography scan showed generalized atrophic changes only. Over the next few months gait problems gradually increased and he became totally unable to move his left arm. On neurological examination he was mentally slow, had mild supranuclear restriction of extraocular movements, adopted a 'hemiplegic' posture of the left sided limbs which were mildly rigid and akinetic, with a profound inability to execute simple movements. There were no cerebellar or sensory abnormalities.
Case 2. An 82-year-old right-handed man gave a 3 year history of increasing right hand tremor. He had been treated elsewhere with propranolol, Madopar (co-beneldopa) and clonazepam without benefit. Primidone was only effective initially. Six months later, as well as the tremor, he complained of weak legs and diplopia. He became progressively less mobile, more stiff and unable to rise from a chair unaided with the result he was admitted for Correspondence to: Dr Khulood A Muhiddin, Consultant Physician, Department of Medicine for the Elderly, Derby City General Hospital, Uttoxeter Road, Derby DE22 3NE, UK
further assessment. On examination, he was mentally slow and apathetic. He had impaired voluntary saccadic eye movements, mild hypophonia, slurring dysarthria and severe rigidity in all four limbs. He could perform limited voluntary movements of the left upper limb only with forced grasping and a 'magnetic' left hand whereby the hand followed the movement of a nearby object. He was unable to copy hand postures suggesting apraxia. He was unable to move his right upper limb or either lower limb voluntarily at all. He had cog-wheel rigidity and hyperreflexia in the right arm with a left extensor plantar response. A magnetic resonance brain scan was normal. Case 3. A 65-year-old right handed lady had a 3 year history of gradual onset of motor difficulty affecting the dominant right upper limb. In addition, there had been infrequent myoclonic jerks and marked speech disturbance with episodes of 'confusion'. Her hypertension was well controlled with atenolol and chlorthalidone. She had received steroids for a left ischaemic optic neuropathy thought to have been due to giant cell arteritis 2 years previously. There had been a clear history of progressive neurological deterioration especially in the last year and she was believed to have cerebrovascular disease. In view of her past history, a normal carotid duplex scan and erythrocyte sedimentation rate of 84, this was attributed to cerebral vasculitis and she received immunosuppressant therapy with prednisolone and azathioprine but her condition continued to deteriorate gradually. On examination it was difficult to communicate with her clearly because of expressive and receptive dysphasia. She had oro-lingual dyspraxia. The most obvious other abnormalities were confined to the right upper limb which was mildly rigid, akinetic, dyspraxic, had stimulussensitive myoclonic jerks and was hyperreflexic. Other limbs were normal. The clinical picture was of asymmetric cortical deficits with a mixture of pyramidal and extra-pyramidal features of insidious onset and gradually progressive.
Discussion Corticobasal degeneration (CBD) is a recently recognized condition that has some similarities to both progressive supranuclear palsy or Steele-Richardson-Olszewski syndrome and Pick's disease. Rebeiz and colleagues in 19671 and 19682 were the first to describe three patients presenting in the sixth or seventh decade of life, with variable combinations of an asymmetric akinetic-rigid syndrome, involuntary movements, apraxia, dysarthria, dysphagia, supranuclear gaze palsy and frontal lobe signs. They suggested the term corticodentatonigral degeneration with neuronal achromasia. There have only been a few case reports. It was not until 1985 that there was a case report of the condition from Massachusetts General Hospital3 and another brief report4. Gibb and co-workers (1989) studied in detail three similar cases5 and suggested the more general term of 'corticobasal degeneration'. Our three patients have clinical features which seem to fit this syndrome of CBD, namely asymmetric progressive akinetic rigidity in the first and third case and magnetic hand with forced grasping in the second. A complex motor abnormality typically restricted to one limb is the most important and striking feature ofthis progressive syndrome. It includes akinetic-rigid features and a whole range of involuntary motor activities, including rest and action tremor, chorea, dystonia, myoclonus and the alien limb phenomenon. Patients describe difficulty in controlling movements of the alien limb sometimes so bad that it has to be restrained with the help of a normal hand.
Presented to Section of Geriatrics & Gerontology, 24 November 1992
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Journal of the Royal Society of Medicine Volume 87 June 1994
This phenomenon of alien limb with magnetic behaviour of the apraxic hand, results from frontal lobe lesions affecting the cingulate gyrus, area 8 and the supplementary motor area which are responsible for avoidance responses. Damage to these regions results in exaggeration of positive exploratory behaviours subserved by the parietal lobe6. Other clinical features of CBD include constructional difficulty (dyspraxia) suggesting parietal lobe damage, dysphasia, dementia, dysarthria and impaired eye movements. The latter involves impairment of saccadic and pursuit eye movements in both horizontal and vertical planes or complete supranuclear gaze palsy. Blepharospasm can also be observed. Tendon reflexes tend to be brisk in the affected limb with stimulus-sensitive myoclonus and the emergence of grasp reflexes and extensor plantar responses. In more severe cases, impaired postural reflexes, truncal ataxia and falls are reported. These features suggest additional involvement of multiple sites including the cerebral cortex (especially posterior frontal and parietal regions), nigrostriatal pathways, supranuclear corticobulbar pathways, and perhaps the cerebellum. The macroscopic pathological features of CBD include cortical atrophy especially of the frontoparietal region and pallor of the substantia nigra. Microscopically, there is severe neuronal loss and gliosis in affected cortical areas, with parietal lobe emphasis, certain subcortical and brain stem regions with absence of Lewy and Pick's bodies5. In contrast to Pick's disease, the hippocampus seems to be spared. Abnormal cortical cells are seen indistinguishable from Pick cells which appear ballooned, swollen and achromatic. However, weakly basophilic inclusions with faint fibrillary attachments are also present, most commonly in the substantia nigra and are believed to be specific as they are not seen in other disorders5. Using positron emission tomography, Sawle et at7 were able to show that corticobasal degeneration has a unique pattern of asymmetric involvement of various brain tissues. Regional cortical oxygen metabolism was mostly depressed in the superior and posterior temporal, inferior parietal, and occipital associated cortices. The two conditions that resemble CBD most closely are Pick's disease and progressive supranuclear palsy. Pick's disease is not a well-defined clinical entity but classically presents with cognitive impairments associated mainly with frontal and temporal lobe disorder such as apathy, perseveration and agnosias. Pick's disease can usually be
Frantz' tumour: a rare pancreatic neoplasm
E M Japes BSc K S JonesMA Homerton Hospital, Hackney, UK
Keywords: pancreas; neoplasm; female; pathogenesis
Introduction Frantz' tumour is a papillary cystic neoplasm of the pancreas. It is usually of low grade malignancy and seems to be almost confined to young women particularly those of African origin.
Case report A 22-year-old African receptionist presented in February 1991 with an abdominal mass. She noticed the swelling Correspondence to: E M Japes & K S Jones, WSCR, St Bartholomew's Hospital, West Smithfield, London EClA 7BE, UK
differentiated pathologically from CBD by its characteristic circumscribed frontotemporal atrophy with sparing of the precentral convolution and an abundance of argyrophilic cortical Pick bodies. Parkinsonian clinical features with moderate nigral degeneration and atrophy of basal ganglia, are rarely seen in Pick's disease, although occasional nigral basophilic bodies resembling the corticobasal inclusions can be found. In progressive supranuclear palsy, the main clinical features are supranuclear gaze palsy, axial rigidity, unsteady gait and mental slowing. It is primarily a symmetric akinetic rigid syndrome without rest tremor with preservation of power and sensation. Brain stem atrophy is characteristic, with neuronal loss and gliosis in globus pallidus, substantia nigra, superior colliculi and pretectal area. Weakly argyrophilic, globose neurofibrillary tangles are typically found in progressive supranuclear palsy with more distinct fibrillary components than the inclusions seen in CBD. In conclusion, CBD is a relatively recently recognized syndrome with particular characteristics which shares some clinical features with progressive supranuclear palsy. In view of the existence of Pick's cells in CBD, it is possible that this syndrome is a variant of Pick's disease that has predilection to affect different neuroanatomical areas. References 1 Rebeiz JJ, Kolodny EH, Richardson EP. Corticodentatonigral degeneration with neuronal achromasia: a progressive disorder of late adult life. Trans Am Neurol Assoc 1967;92:23-6 2 Rebeiz JJ, Kolodny EH, Richardson EP. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 1968; 18:20-33 3 Scully RE, Mark EJ, McNeely BU. Case records ofMassachusetts General Hospital. N Engl J Med 1985;313:739-48 4 Watts RL, Williams RS, Growdon JD, Young RR, Haley EC, Beal MF. Corticobasal ganglionic degeneration. Neurology 1985;35(suppl 1):178 5 Gibb WRG, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain 1989;112:1171-92 6 Feinberg TE, Schindler RJ, Flanagan NG, Haber LD. Two alien hand syndromes. Neurology 1992;42:19-24 7 Sawle GV, Brooks DJ, Marsden CD, Frackowiak RSJ. Corticobasal degeneration - a unique pattern of regional cortical oxygen hypometabolism and striatal fluorodopa uptake demonstrated by positron emission tomography. Brain 1991;114:541-56
(Accepted 27 September 1993)
7 months previously while dressing and it had not changed in size. She experienced occasional discomfort and a feeling of 'bloatedness'. A mass was palpable in the left upper quadrant of the abdomen extending 4 cm below the left costal margin. Her serum amylase was 341 U/L (normal < 220 U/L) and she had a microcytic hypochromic anaemia. Ultrasound examination demonstrated a cystic mass containing multiple septae 11 x9x 12 cm, in the tail of the pancreas. The body and head of the pancreas appeared normal. Computerized tomography scan demonstrated a mass in the tail of the pancreas displacing the stomach and spleen (Figure 1). The tumour was excised by distal pancreatectomy with splenic conservation. She remains well 1 year following her surgery. Pathology The tumour was a discrete cream cyst weighing 729 g with a smooth serosal surface. It contained scattered papillary areas attached to the wall and blood-stained serous fluid. Histological examination showed fibrovascular stalks, pseudorosettes and myxoid degeneration with widespread haemorrhage and necrosis. The surrounding pancreatic
Case presented to Clinical Section, 13 March 1992
