Progressive Supranuclear Palsy Presenting with Dynamic Aphasia 407

J7ournal ofNeurology, Neurosurgery, and Psychiatry 1996;60:403-410 403 Progressive supranuclear palsy presenting with J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.60.4.403 on 1 April 1996. Downloaded from dynamic aphasia Thomas Esmonde, Elaine Giles, John Xuereb, John Hodges Abstract have been noted in some patients with PSP Background-Progressive supranuclear during the course of their illness,2-6 descrip- palsy (PSP) is an akinetic-rigid syndrome tions of a presentation with a disorder of spo- of unknown aetiology which usually pre- ken language production are virtually absent sents with a combination of unsteadiness, from the medical literature; Perkin et al8 bradykinesia, and disordered eye move- reported five patients with atypical presenta- ment. Speech often becomes dysarthric tions, two of whom had severe dysphasia in but language disorders are not well recog- the context of mild global dementia, one made nised. naming errors and later developed unintelligi- Methods-Three patients with PSP ble speech, and the other was described as a (pathologically confirmed in two) are non-fluent dysphasic. The lack of awareness of reported in which the presenting symp- this aspect is illustrated by the fact that two toms were those of difficulty with lan- recent books devoted entirely to PSP,9 10 guage output. although mentioning mild word finding diffi- Results-Neuropsychological testing culty and reduction in speech output during showed considerable impairment on a the course of the disease, do not refer to this range of single word tasks which require presenting feature. We have seen three active initiation and search strategies patients with PSP in whom the initial presen- (letter and category fluency, sentence tation was one of a verbal adynamia, resem- completion), and on tests of narrative bling the phenomenon described by Luria," language production. By contrast, nam- also sometimes termed dynamic aphasia.'2 All ing from pictures and from verbal three underwent detailed neuropsychological descriptions, and word and sentence and linguistic assessment, and in two the diag- comprehension were largely intact. The nosis was confirmed pathologically. degree of semantic memory impairment was also slight. Conclusions-Relatively selective involve- Patient 1 ment of cognitive processes critical for A 62 year old gardener presented in 1990 to a planning and initiating language output psychiatrist after a two year history of a change may occur in some patients with PSP. in personality. Initially, he had been disinhib- http://jnnp.bmj.com/ This presentation resembles the phenome- ited and aggressive, but had subsequently non of "verbal adynamia" or "dynamic become anergic and apathetic. Over this aphasia" seen in patients with frontal lobe period it was noticed by the patient, and his damage. Although definite cortical family, that his expressive output in both changes were present at postmortem speech and writing had diminished greatly. examination, it is likely that the neu- Practical abilities around the home seemed ropsychological deficits reflect functional preserved. Examination in 1991 showed him on September 24, 2021 by guest. Protected copyright. Department of Neurology, Royal frontal deafferentation secondary to to be very non-fluent with pronounced word Victoria Hospital, interruption of frontostriatal feedback finding difficulties. Speech was limited to sin- Belfast, UK loops. gle words or short phrases which were gram- T Esmonde matically correct without phonological or The University (3 Neurol Neurosurg Psychiatry 1996;60:403-4 10) semantic paraphasias. By contrast, confronta- Neurology Unit, Addenbrooke's tion naming, word and sentence repetition, Hospital, Cambridge, and comprehension of simple and complex UK Keywords: progressive supranuclear palsy; dynamic commands were strikingly normal. Physical E Giles aphasia J Hodges examination showed a mild degree of supranuclear vertical gaze limitation and poor horizon- Department of Pathology, Progressive supranuclear palsy (PSP) as origi- tal saccadic movements. His gait was wide Addenbrooke's nally described by Richardson et al in the based and slightly unsteady, but Romberg's Hospital, Cambridge, 1960s' characteristically presents as a parkin- test was negative. Other extrapyramidal signs UK J Xuereb sonian syndrome with axial rigidity, bradyki- were absent. Correspondence to: nesia, postural instability, and paralysis of Brain CT showed a mild degree of frontal Dr J R Hodges, Department conjugate gaze for voluntarily guided eye atrophy. Examination of CSF was normal. of Neurology, Addenbrooke's Hospital, movements. Dysarthria and swallowing diffi- The diagnosis at this stage was unclear, but Hills Road, Cambridge, UK. culties occur as a result of involvement of the PSP or dementia of frontal lobe type were Received 18 May 1995 muscles of articulation by extrapyramidal considered the most likely possibilities. and in final revised form 8 December 1995 rigidity. Although dysphasia (of a generally Over the ensuing 12 months, anxiety and a Accepted 15 December 1995 unspecified type) and word finding difficulties preoccupation with death became prominent 404 Esmonde, Giles, Xuereb, Hodges features. Axial rigidity became pronounced, died after a total duration of illness of five J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.60.4.403 on 1 April 1996. Downloaded from and there was a loss of all voluntary eye move- years. ments. He became increasingly immobile and often fell. Speech was restricted to single word POSTMORTEM EXAMINATION repetitive utterances before mutism super- Macroscopically, the brain showed mild gen- vened in the terminal stages of his illness. He eralised gyral atrophy and pallor of the sub- died four years after the onset of the first stantia nigra. Microscopical examination symptoms. showed a few neurofibrillary tangles in the frontal cortex and an occasional tangle in the POSTMORTEM BRAIN EXAMINATION temporal cortex. Senile plaques were absent. Macroscopical examination of the brain Nerve cell loss and gliosis were present in the showed a degree of cerebral gyral atrophy, subthalamic nucleus. Neurofibrillary tangles more pronounced in the lateral frontal regions, were numerous in the globus pallidus, and pallor of the substantia nigra and locus Meynert's nucleus, the thalamus, the hypo- coeruleus in the brain stem. Microscopically, thalamus, and the subthalamic nucleus. severe nerve cell loss and gliosis were evident Tangles were also prominent in the red in the subthalamic nucleus and the central nucleus, substantia nigra, the nucleus of mesencephalic grey. Less severe neuronal loss Darkshevich, central mesencephalic grey, was present in the brain stem, pigmented brain stem pigmented nuclei, reticular forma- nuclei, and the dentate nucleus. Surviving tion, pontine nuclei, accessory dorsal olivary nerve cells in these subcortical and brain stem nucleus, and the dentate nucleus. As in the areas contained tau positive neurofibrillary previous patient, the neuropathological find- tangles; tangles were also present in the globus ings were diagnostic of PSP. pallidus, the red nucleus, the brain stem reticular formation, the pontine nuclei, and particu- larly in the nucleus of Darkshevich in the Patient 3 rostral midbrain. Neurofibrillary tangles were A right handed housewife and exwarden of a numerous in the frontal cortex but there were nursing home presented at the age of 65 with a very few in the temporal cortex; senile plaques one year history of word finding difficulty in were sparse. The type and distribution of speech and very impaired conversational abili- lesions confirmed the diagnosis of PSP. ties. Her ability to use her right hand in writing had slowed up and there was a tendency to overbalance easily. Despite the reduced verbal Patient 2 output and clumsiness, she continued to com- A retired dispatch manager presented at the plete her daily crossword puzzle. age of 67 when his wife noticed that over the She had somewhat immobile facies, mild previous two years there had been a reduction axial rigidity, and a hesitant gait. Voluntary in his speech, which was now reduced to single upward saccades and pursuit movements were words or short phrases. His comprehension abnormal. Her speech was mildly dysarthric seemed intact and he was only mildly forget- and slow, with reduced phrase length. ful. On further enquiry, it became apparent Paraphasic errors were absent. Naming, word that more recently his personality had changed and sentence repetition, and comprehension http://jnnp.bmj.com/ to the extent that he had become rigid, irrita- were normal. ble, and had lost his sense of humour, but he Brain CT, chest radiograph, thyroid func- did not complain of any of these difficulties tion, syphilis serology, and CSF examination spontaneously. His general abilities around the were all normal. home seemed normal, and he had been able to She was treated with levodopa, lisuride, and cook and clean when left on his own while his amantadine, without noticeable response and wife was in hospital for a cataract operation. A her condition deteriorated. Five years from the on September 24, 2021 by guest. Protected copyright. degree of postural instability was evident from onset of first symptoms, she was chairbound the fact that he had had several falls. with very dysarthric speech and minimal spon- He had a dyspraxic gait and postural insta- taneous utterances. Rigidity was pronounced. bility. Eye movements were abnormal with

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