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Frontal Lobe Dementiaand Motor Neuron Disease

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Frontal Lobe Dementiaand Motor Neuron Disease Journal ofNeurology, Neurosurgery, and Psychiatry 1990;53:23-32 23 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.53.1.23 on 1 January 1990. Downloaded from Frontal lobe dementia and motor neuron disease D Neary, J S Snowden, D M A Mann, B Northen, P J Goulding, N Macdermott Abstract with a 12 month history of personality change. Four patients are described, in whom a Formerly equable and conscientious, he profound and rapidly progressive became rude and verbally aggressive, and dementia occurred in association with progressively more withdrawn and clinical features ofmotor neuron disease. unmotivated, his sole remaining interest being The pattern of dementia indicated im- in food. He continued to carry out activities of paired frontal lobe function, confirmed daily living independently. by reduced tracer uptake in the frontal Physical examination General examination was lobes on single photon emission com- normal. Neurological examination revealed puted tomography (SPECT). Patho- slight weakness of the right side of the face, logical examination of the brains of two dysarthria, weak cough and gag reflex, wasting patients revealed frontal-lobe atrophy, and fasciculation of the tongue. The jaw jerk with mild gliosis and spongiform change. was exaggerated. Muscular power was normal The spinal cord changes were consistent but there was a mild spastic increase in tone in with motor neuron disease. The clinical the lower limbs. There were fasciculations of picture and pathological findings resem- the muscles of the shoulder girdle and the bled those of dementia of frontal-lobe biceps. Tendon jerks were brisk, the plantar type and were distinct from those of responses flexor and abdominal reflexes Alzheimer's disease. The findings have present. Sensation, coordination and gait were implications for the understanding ofthe normal. spectrum of non-Alzheimer forms of Mental examination He had a bland, un- primary degenerative dementia. concerned affect and he lacked insight. His behaviour was mildly disinhibited. Examina- tion of his conversational speech, comprehen- The association between dementia and motor sion, repetition, word finding, reading and neuron disease has become increasingly recog- writing revealed no linguistic impairment. He nised over recent years.' Formerly identified as performed normally on the abbreviated Token part of the Dementia-Parkinsonism complex test12 and Boston naming test.'3 However, he occurring commonly on the Pacific island of exhibited concreteness of thinking, with literal Guam,23 there have since been several reports interpretations of metaphors and proverbs. http://jnnp.bmj.com/ of the association from Japan' and from There was no evidence ofperceptual, spatial or Western countries.7-"1 However, the pattern of praxic disorder. He identified objects, line mental change and its relationship to other drawings of objects and faces of celebrities. He forms of dementia remains unclear. Four could locate objects in the room, track the patients, in whom the diagnosis of presenile Money road map'4 and reproduce non- dementia with motor neuron disease was made representational hand postures. His spontan- during life, have been studied longitudinally. eous drawing of a house and copies of a cube Comprehensive clinical analysis, including and abstract figures were normal, with on September 26, 2021 by guest. Protected copyright. both qualitative and quantitative assessment of preserved spatial configuration. He could mental function, has permitted the identifica- reproduce Koh's block figures. He had no tion of characteristic features of the dementia dressing difficulty. He could demonstrate The Department of and Neurology, accompanying motor neuron disease, which actions by gesture pantomime. Manchester Royal could be compared with characteristics ofother He was fully orientated for time and place Infirmary and the forms of primary degenerative dementia. The and gave a good account of day to day events. Department of He could summarise fable-like short stories Neuropathology*, brains of two patients have been studied at University of necropsy, permitting an examination of immediately after reading them aloud and Manchester clinico-pathological relationships. recalled them to a comparable level of D Neary proficiency after a one hour delay. On the J S Snowden D M A Mann* Case histories Warrington word and face recognition tests'5 B Northen In none of the four patients was there any he scored respectively 40/50 and 39/50, lying at P J Goulding the 10th percentile for his age group. On a N Macdermott previous medical or psychiatric history. There had been no occupational exposure to paired-associate task he failed to learn six pairs Correspondence to: words over six trials. Dr D Neary, Department of chemicals or heavy metals. They had never of unrelated learning Neurology, Manchester suffered a major head injury and there was no However, when provided with a visual image Royal Infirmary, Manchester he could learn a matched set of M13 9WL, United evidence of alcohol abuse. There was a family strategy Kingdom. history of dementia in patient 2 only. unrelated word pairs on the first trial. He Received 13 December 1988 recalled all word pairs after a one hour delay and in revised form 1 without additional 7 April 1989. Patient training. Accepted 12 April 1989 A 52 year old male security officer presented Assessment with the WAIS'6 yielded a 24 Neary, Snowden, Mann, Northen, Goulding, Macdermott J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.53.1.23 on 1 January 1990. Downloaded from Table 1 WAIS age-scaled scores (patient 4 not tials. Concentric needle electromyography of testable) muscles of the upper and lower limbs revealed abundant multifocal fasciculation, motor unit Patient potentials as great as 10 mV, and reduced 1 2 3 interference pattern, compatible with motor neuron disease. Verbal Information 7 4 1 Progression His condition deteriorated Digit Span 8 10 4 dramatically over the ensuing six months. Vocabulary 7 7 1 Arithmetic 8 4 2 However, for geographical reasons re-exami- Comprehension 5 6 1 nation was not possible. Similarities 2 4 2 Performance Picture completion 10 7 2 Picture arrangement 6 - 2 Block design 10 6 5 Patient 2 Object assembly 12 7 4 A 58 year old labourer presented with an 18 Digit symbol 6 7 2 month history of personality change and dis- verbal IQ of 77, performance IQ of 91 and full ordered conduct. Formerly sociable and scale IQ of 83. Disparities between subtest considerate he became thoughtless and callous. scores (table 1) are of relevance. The low He was restless and impatient and could no "comprehension" score arose since his longer apply himself constructively to tasks. responses reflected personal anecdotes rather Previously an occasional Bingo player he than generalisations. The poor scores on the became obsessed with the game. Yet, he would "similarities" sub-test reflect a consistent miss the numbers called because of poor con- tendency to identify differences rather than centration. He lost his former meticulous table similarities between pairs of objects. In the manners and would cram food into his mouth picture arrangement task he left pictures essen- and eat noisily. He had developed an abnormal tially in their original order. He could itemise fondness for chocolates. He was noted to feel elements within individual pictures but did not the cold more than before. One year after the attempt to integrate these into a coherent onset of his symptoms his behaviour was more whole. On the National Adult Reading Test'7 overtly disinhibited: he would shout and swear he achieved an error score of 30, which yields a in public and appeared oblivious to social predicted verbal IQ of 101 and performance IQ mores. Although previously little interested in of 104. Using a 5% cut-off the discrepancy sexual relations he became obsessed with sex between predicted and obtained IQ is highly and would proposition females indiscrimin- significant for the verbal scale but does not ately, including an aunt of 80 years. Approxi- reach significance for the performance scale. mately nine months after the onset of his He performed poorly on tasks sensitive to mental symptoms he developed weakness ofthe frontal lobe dysfunction, that make demands arms and six months later ofthe legs. In the two on abstraction, planning and mental flexibility. months before investigation he had developed On the modified version of the Wisconsin card slurring of speech and difficulty in swallowing. sorting test'8 he achieved two categories only. The patient denied mental or physical difficul- Despite reminders of the test instructions he ties and despite his weakness persisted in http://jnnp.bmj.com/ persistently sorted cards with those previously driving his car, professing himself competent placed, rather than according to the four to do so. stimulus cards. On the Weigl's block task'9 he The patient's mother had had a similar achieved one method of grouping only. When change in personality and conduct when aged blocks were grouped by the examiner he could 60 years. She had neglected personal hygiene identify the underlying sorting criterion in one and self-care and refused to eat. When her case only, on subsequent trials displaying per- family stocked her larder for her she had posted severation ofthis original sorting rule. In a task the food through neighbours' letter boxes. She on September 26, 2021 by guest. Protected copyright. based on the "20 questions" principle,20 which too had become obsessed with the game of requires the subject to identify a "target" Bingo. She had begun to drink heavily, result- picture presented amongst alternatives by ask- ing in falls. She had been admitted to an elderly ing questions yielding a yes/no response, per- persons home where she had been aggressive formance was concrete and lacking in strategy. and disinhibited. She had wandered inces- He pointed to individual pictures at random santly. In contrast to her mental difficulties she and made no attempt to generate questions that had remained physically well, and there was no would eliminate the greatest number of alter- report of weakness, difficulties in swallowing natives.
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