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| Year : 1999 | Volume
: 47
| Issue : 2 | Page : 139-41 |
Primary progressive aphasia : a case report.
Arora A, Sawhney IM, Verma SK, Lal V, Prabhakar S
Departments of Neurology and Clinical Psychology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Correspondence Address:
Departments of Neurology and Clinical Psychology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Primary progressive aphasia is due to focal left perisylvian degeneration and manifests with progressive decline in language function for two or more years. There is preservation of cognitive functions and activities of daily living continue to be normal. We report a case of progressive aphasia in a 65 year old lady.
How to cite this article:
Arora A, Sawhney I M, Verma S K, Lal V, Prabhakar S. Primary progressive aphasia : a case report. Neurol India 1999;47:139 |
Primary progressive aphasia (PPA) is a clinical syndrome of progressive decline in language function for two or more years with preservation of activities of daily living and general cognitive functions. There is no space occupying lesion, infarction or other identifiable structural disorder to explain the deficit.[1] It represents a relatively selective left perisylvian degeneration and needs to be differentiated from generalised degenerative disorders e.g. Alzheimer's disease (AD) which may present with aphasia.[2] The syndrome was brought to clinical /sup> and a number of cases have been reported in western literature since then.[4],[5] However, there is lack of reports from the Indian subcontinent. We describe a case of progressive transcortical expressive aphasia without dementia.
A 65 year old house wife presented with progressive difficulty in speaking for 3 years. Family members and relatives complained that they could not understand her speech clearly. There was a decrease in speech output and she had hesitancy in speaking. She used wrong grammer and sometimes used to pause for a long time for finding words. However, there were no problems in activities of daily living and functional abilities. There was no loss of memory or decline in cognition. She had hypertension for 5 years which was well controlled with treatment.
General physical examination and systemic examination were normal. She was subjected to a detailed language function testing and psychometry. There was a marked decrease in her word output with loss of fluency. She missed words and could not use proper verbs during spontaneous speech. There was evidence of agrammatism. When given a task to count the number of vegetables, she could count only 12 in one minute (normal 18-22). Similarly she could count only 12 animals in one minute (Normal 18-22). Patient could write both Hindi and Punjabi but would mix the two languages. She missed words in writing a sentence and ended the sentence abruptly instead of writing it completely. However, the content of speech was normal. She had normal comprehension even on complex commands and could repeat long sentences though she took a longer time. She could name simple objects as well as parts of objects pointed to.
A detailed examination of her cognitive functions revealed normal registration, recall and past memory. She had normal judgement, abstract thinking and intelligence. There was no evidence of agnosia or apraxia. A detailed neuropsychological assessment was done.[6] On verbal adult intelligence scale, she had a verbal IQ of 118. Testing with coloured progressive matrices,[7] she had a performance IQ of 93. Her mean IQ was 106. But she showed some percepto-motor disturbance (poor depth perception, partial rotation, fragmentation) when tested with the help of Bender Gestalt and Nahar and Benson scales. On detailed memory testing, she performed very well on recognition but had perseveration on visual retention subtest. She had no motor sensory dysfunction. There was no other neurologic deficit. Haemogram and routine biochemistry were normal. A contrast CT scan revealed predominantly frontal atrophy. MRI scan confirmed the finding of frontal atrophy. There was no evidence of infarction, demyelination or space occupying lesion.
The syndrome of PPA is a classical example of a focal cortical degeneration and has been classified as asymmetrical cortical degeneration syndrome.[8] Though initially thought to be affecting people younger than those affected by AD, it has been reported in older individuals as well.[9] Clinical profile is that of a gradually progressive deficit in language function with a relative sparing of other intellectual functions.[2] The language deficits described in these patients include anomia, mild agrammatism and decreased fluency. In typical cases verbal comprehension and non verbal cognition were spared.[9],[10] Mesulam has described logopenia and long word finding pauses as typical of the disorder.[3] Weintraub et al investigated aphasia profiles in four such patients and described similar findings.[11] However, one patient in a study by Green et al[9] had a deficit in comprehension as well.
The most important differential diagnosis remains aphasic form of AD. In fact aphasia may be the presenting feature of AD.[12],[13] However, in AD aphasia is soon followed by major disturbances of memory and other cognitive functions. Judgement, insight and independence in daily living deteriorate in AD, while they may remain intact until the terminal stages in patients of PPA. The language dysfunction in patients of PPA is characterised by anomia, agrammatism and nonfluent features, while this kind of language deficit is rare in AD.[2] Weintraub et al emphasised that one type of aphasia that clearly differentiates PPA from AD is transcortical motor aphasia which has not been reported in AD, but is quite frequent in PPA.[11] Poeck and Luzatti extensively analysed three patients and denied the existence of entity called PPA.[14] But all three patients described by them were having fluent aphasia and one of them also had a marked deficit in comprehension. Thus patients having a nonfluent aphasia with relative preservation of lexical/semantic features of language clearly differentiate PPA from aphasic form of AD.
The imaging studies showing left perisylvian atrophy were initially thought to be the hallmark of the syndrome. Later studies, however, showed that imaging studies performed early in the course of illness did not provide asymmetrical abnormalities in the left cerebral hemisphere.[11] Sinnatamby et al studied the neuroradiological findings in 13 patients of PPA and reported focal atrophy in 4 out of 10 patients on CT scan and in 10 out of 12 patients on MRI.[15] However, all patients who underwent SPECT showed unilateral temporal lobe perfusion defects. They concluded that MRI and SPECT are the imaging modalities of choice in PPA. Chawluck et al studied two such patients using positron emission tomography and found focal hypometabolism in the left temporal and/or parietal lobes.[16] There are few reports of electrophysiological studies in such patients. Onfrj et al reported P300 studies in patients of PPA.[17] In their study when P300 was recorded using average reference, it could differentiate patients from controls. A negativity was recorded in patients from affected hemisphere, instead of positivity recorded in controls.
Variable pathological changes have been described in left perisylvian region. Mehler et al described two patients with clinical features similar to PPA.[18] The pathological study in one of these patients revealed non specific degenerative changes. Kirshner et al studied two patients and found changes of focal spongiform degeneration from perisylvian region.[19] Lippa et al also studied pathological changes in two patients and found focal neuronal achromasia.[1] Kertesz et al described 3 cases which showed a pathology similar to Pick's disease.[5] However, the cases were different in the sense that there were no Pick bodies in the hippocampus. They proposed that PPA is a variant of Pick's disease and all focal cortical degeneration should be classified as `Pick complex.' The syndrome of PPA carries a more benign prognosis as compared to Alzheimer's disease. The language disorder is very slowly progressive and independent activities of daily living, insight and judgement are preserved till long. Even very aphasic patients with PPA seem to have ability to make themselves understood by writing and by using circumlocutions. They maintain motivation and acquire new hobbies.[2],[11]
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