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Slowly Progressive Apraxia in Alzheimer's Disease

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Slowly Progressive Apraxia in Alzheimer's Disease 31232ournal ofNeurology, Neurosurgery, and Psychiatry 1995;59:312-315 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.3.312 on 1 September 1995. Downloaded from SHORT REPORT Slowly progressive apraxia in Alzheimer's disease R C Green, F C Goldstein, S S Mirra, N P Alazraki, J L Baxt, R A E Bakay Abstract left hand reach out and remove it. After six Slowly progressive apraxia due to months, he had problems performing mental Alzheimer's disease was encountered in arithmetic, writing script or numbers with his a 66 year old, right handed man whose right hand, and he experienced spatial confu- initial impairments included coordinated sion when driving or in a familiar environ- movements of the left hand and some ment without visual cues. He and his wife features of the alien hand syndrome. agreed that he still did not have problems Over four years, the patient developed with gait, word finding, comprehension, or progressively worsening deficits of mem- memory. There was no personal or family ory and language. A biopsy of his right history of neuropsychiatric problems or sub- temporal lobe showed numerous plaques stance misuse, except for a maternal aunt and neurofibrillary tangles. Pronounced clinically diagnosed as having Alzheimer's right parietal lobe hypoperfusion on ser- disease. Detailed neurological examination ial SPECT suggests involvement of this was normal, including motor tone and pri- region in contralateral praxis. mary sensory and motor function in the limbs, except for moderately severe ( Neurol Neurosurg Psychiatry 1995;59:312-315) agraphaesthesia and astereognosis in the left hand and difficulties with left hand praxis. Neuropsychological evaluations were con- Keywords: Alzheimer's disease; subtypes; apraxia; ducted in July 1990, January 1991, and July movements 1992 (table). Apraxia was evaluated by hav- ing the patient carry out gestures and imag- ined use of objects. When seen in July 1990, Apraxia is a syndrome of impaired execution imitation was performed poorly by each hand of learned skilled movements that cannot be and the execution of transitive and intransi- explained by weakness, incoordination, sen- tive movements to command was worse with sory loss, lack of comprehension, or inatten- the left hand. Responses were slow, and body tion. Apraxia is common in Alzheimer's part as the object and spatial displacements http://jnnp.bmj.com/ disease, usually appearing after impairments were common. Performance did not appre- Neurobehavioral Program, Wesley of memory and language are established. A ciably improve when the patient was tested Woods Center and few cases of slowly progressive apraxia with- with actual objects. At his six month follow Department of out deficits in memory and language have up, there was a noticeable deterioration of Neurology, VA been Medical Center described but the underlying pathology transitive and intransitive movements with R C Green was not established.l1 Our patient showed the right hand. Imitation was still poor bilat- F C Goldstein progressive apraxic symptoms early in the erally, and had become even worse with the Gwinnett Neurology course of his illness. left hand. By July 1992 he could only perform on September 29, 2021 by guest. Protected copyright. Clinic simple right handed movement to command J L Baxt and imitation, and he could not execute any Department of Patient history Pathology and movements with his left hand. Laboratory Medicine A 66 year old, right handed, college educated In July 1990 he could not determine how S S Mirra chemical engineer experienced the insidious to hold a pencil with his left hand and he Department of onset of finger and hand incoordination in could not write a sentence correctly to dicta- Neurosurgery, Emery association with complex movements of the tion with his right hand, although he could University Hospital left hand at the age of 60. Initially, he had R A E Bakay copy. By January 1991, his writing with his minor problems driving his car and shuffling Department of right hand both to dictation and from copy Nuclear Medicine, playing cards, but by the end of 1988 he had deteriorated and by July 1992 he was unable Emergy University stopped working and could not easily tie his to write his name. His drawings were tremu- School ofMedicine, shoelaces, button his shirt, or eat with his left lous and and he was Atlanta, Georgia, USA micrographic, impaired N P Alazraki hand. He and his family denied any language in copying simple geometric designs on the or memory difficulties. When initially exam- Correspondence to: Benton visual retention test. By contrast with Dr Robert C Green, Wesley ined in June 1990 the patient was aware of his dyspraxic and dysgraphic symptoms, his Woods Center, 1841 Clifton his that his left hand "seems Road, NE Atlanta, GA deficits, stating language abilities were relatively preserved in 30329, USA. to have a life of its own." His wife confirmed July 1990 and January 1991. By July 1992, Received 21 March 1994 extraneous behaviours reminiscent of alien however, he displayed severe dysnomia, with and in final revised form hand such as a in 2 May 1995 syndromes, placing napkin many perceptual misidentifications and Accepted 9 May 1995 his lap with his right hand, only to have his semantic paraphasias. Generation of words Slowly progressive apraxia in Alzheimer's disease 313 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.3.312 on 1 September 1995. Downloaded from Neuropsychological test results propyline amineoxime with the patient supine July 1990 January 1991 July 1992 in a low stimulation environment in August 1990 and February 1991. These studies Intellectual functioning: (WAIS-R: mean = 100, SD = 15) showed hypoperfusion in the right frontal, Verbal IQ 109 110 69 parietal, temporal, and occipital regions Performance IQ 63 60 60 Full Scale IQ 88 87 63 (fig 1). In February 1991, biopsies of the middle Verbal age scaled scores: (mean = 10, SD = 3) and inferior temporal cortices on the right Information 14 14 1 side were performed. Neuropathological Digit span 8 5 3 Vocabulary 14 14 11 examination with Bielschowsky silver staining Arithmetic 4 4 2 of the paraffin embedded material showed Comprehension 12 16 8 Similarities 16 16 3 numerous senile plaques (up to 50 per low Performance age scaled scores: power field) (fig 2). The plaques were pre- Picture completion 3 4 2 Picture arrangement 6 2 2 dominantly neuritic in nature, some contain- Block design 2 2 3 ing amyloid cores; a few diffuse plaques were Digit symbol 2 1 1 also seen. The density of neuritic plaques met Language: conventional neuropathological criteria for Apraxia (No correct) Limb intransitive (12 total): diagnosis of Alzheimer's disease.5 Numerous RH: commands 8 6 1 neurofibrillary tangles were also noted, partic- Imitation 9 9 0 LH: commands 2 3 0 ularly in the deeper layers of cortex, along Imitation 8 4 0 with neuropil threads. Limb transitive (7 total): RH: commands 5 2 0 Imitation 4 3 0 LH: commands 2 2 0 imitation 6 4 0 Discussion Writing (No correct/6 words) Apraxia is common in dementia, occurring in Dictation (RH, LH) 2, CND 3, CND CND, CND Copy (RH, LH) 6, CND 4, CND CND, CND Huntington's disease, corticobasal ganglionic Naming (No correct/60 pictures) 55 55 10 degeneration, and occasionally in Parkinson's Verbal fluency (No correct) Three letters 26 20 0 disease. Apraxia is also common in Three categories 46 33 9 Alzheimer's disease, but is rarely described as Tactile naming (14 objects) RH (No correct) 12 10 NG a presenting symptom. Three cases of slowly LH (No correct) 8 10 NG progressive apraxia without tissue diagnoses Memory: have been reported, but two of these seemed Picture recognition to represent visual agnosias in which the (No correct/100) 78 67 CND Story recall patients made errors imitating gestures but (No units/50) 8 0 0 performed correctly to command.6 The third Story recognition (No units/20) 14 13 6/13 case, a patient with an insidious loss of abili- ties to perform limb and axial movements to CND = could not do; NG = not given; LH = left hand; RH = right hand. both imitation and command, more closely resembles our patient, but there was no fol- low up or tissue diagnosis. beginning with specific letters was in the low Patients with Alzheimer's disease estab- to normal range at his initial session, and lished by biopsy have presented with progres- deteriorated further in subsequent examina- sive left sided clumsiness, astereognosis, http://jnnp.bmj.com/ tions. agraphaesthesia, and choreoathetoid move- When initially tested, the patient's visual ments7 as well as with alien left hand and recognition memory was in the low average myclonus.8 Several other recent clinical case range. Six months later, there was a notice- reports of slowly progressive apraxia have able decline, and by July 1992 he could not been associated with unilateral or bilateral perform the task. Over the two years he was cortical atrophy and diminished SPECT followed up he exhibited a steady decline in activity in parietal regionslA but none of these his verbal intellectual abilities. The table reports included a tissue diagnosis. Published on September 29, 2021 by guest. Protected copyright. shows a pronounced difference between his reports of SPECT findings in patients with average verbal IQ and severely impaired per- Alzheimer's disease indicate that parietal lobe formance IQ in July 1990 and January 1991. hypoperfusion is often seen, but it usually By July 1992 the verbal IQ had dropped 40 presents bilaterally. Unilateral apraxia has points, and he had lost the insight that had often been described in patients with focal characterised his earlier testing sessions. lesions, particularly strokes, but is also Diagnostic testing in the summer of 1990 unusual in Alzheimer's disease.
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