Journal of the Royal Society of Medicine Volume 85 August 1992
449
Cerebellar ataxia in the elderly
A F Safe MSc MRCP
S Cooper mB MRcP
A C M Windsor FRCP
Department of Geriatric Medicine,
Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW Keywords: cerebellar ataxia; elderly patients; prognosis
Summary In a retrospective study of 624 elderly patients referred with falls and gait disorders, 45 patients were found to have ataxia. Cerebrovascular diseases were the most common underlying cause of ataxia (15 patients, 37%). Nine patients had hereditary/ degenerative cerebellar ataxia. History suggesting alcohol as an underlying cause was established in two patients with cerebellar ataxia. Three patients had normal pressure hydrocephalus and their condition improved remarkably after surgery. No definite cause was found in five patients. Cranial computed tomography (CT) showed cerebral atrophy in 16 patients and in three patients there was evidence of atrophy of the cerebellar vermis. Four patients had femoral neck fractures and three patients had other fractures. In a 5-year follow-up five patients died with bronchopneumonia (1 1% mortality) and patients with dementia showed rapid deterioration. All patients were referred to the day hospital for rehabilitation. The best treatment outcome was achieved in patients who had a single cerebrovascular accident with no cognitive impairment and in those whose ataxia was secondary to medication. Fourteen patients (44%) moved to residential care while 27 (66%) continued to live in their homes with community support. We concluded that there is no evidence of increased mortality in the elderly patients with cerebellar ataxia. CT scan is mainly helpful in diagnosing specific diseases such as tumours or hydrocephalus. A significant proportion of elderly patients with ataxia may have reversible or treatable conditions and can pursue an independent life.
Introduction Normal body posture and locomotor control depend on afferent influxes from three main sensory modes; the proprioceptive endings, vision and vestibulocerebellar connectionsl. These postural responses are slowed in older subjects2 and sway while standing increases with advancing age3. As a result gait disturbances are common in the elderly -and can be disabling. In one population based study, 15% of the subjects over 60 years of age had some abnormality of gait4. Gait disorders contribute to the risk of, falling5 which is a major cause of -morbidity among elderly persons. Non-ambulatory patients have excess morbidity and mortality and often require long term nursing care. Ataxia is a rare cause of gait disorders. In a survey study of 50 elderly patients, cerebellar ataxia accounted for 8% and sensory imbalance for 18% of gait disorders6. There. are few reports in the literature about ataxia in the elderly. The purposes of this retrospective study are to establish the causes
of cerebellar ataxia in the elderly, its response to treatment and its long term prognosis. Patients and methods The study population was composed of elderly patients >65 years who had been referred to the unit in the last 5 years with gait disturbances and found to have cerebellar ataxia. The patients were examined independently by at least two experienced physicians. Patients were defined as having cerebellar ataxia if they had truncal ataxia and an ataxic gait. Patients with hemiparesis or major motor defects from strokes had been excluded from the study. The diagnosis of stroke was made when cranial computed tomography (CT) showed clear evidence of cerebral infarction. Data collection Data were collected from the HAA system in the hospital, from the patients' case notes and by contacting the general practitioners and community services regarding long term care.
Results Forty-five patients were identified to have definite cerebellar ataxia out of 624 patients referred with falls and gait disorders. Four patients were,excluded as their full clinical details were not available or it was not possible to trace their long term care. Fortyone patients were included in the study (21 females and 20 males). Their mean age at presentation was 76 years (age range was 65-83 years). Four patients were diagnosed some years before referral; two with multiple sclerosis and two with hereditary ataxia. Causes of ataxia These are shown in Table 1. Cerebrovascular disease wasthe most common underlying cause accounting for 37% of cases. Three patients had cerebellar ataxia and peripheral neuropathy. History of heavy intake Table 1. Causes of cerebellar ataxia in 41 elderly patients
Diseases
Number of patients
Cerebrovascular diseases
15
Hereditary/degenerative
9 2 2 2 3 3 5 41
Multiple sclerosis Alcohol Diugs (phenytoin) Normal pressure hydrocephalus Tumours (primary & secondaries)
Unknowni Total
0141-0768/92/
080449-03/S02.00/0 © 1992 The Royal Society of Medicine
450
Journal of the Royal Society of Medicine Volume 85 August 1992
Table 2. Relevant physical findings in 41 patients with cerebellar ataxia
Physical signs
Number of patients
Truncal ataxia Limb ataxia Nystagmus Dysarthria Upward plantars Pes cavus
41 17 21 18 8 3
of alcohol was established in two patients. TIwo patients had cerebellar metastases from bronchial carcinoma, but in the review no cases of nonmetastatic carcinomatous cerebellar degeneration were identified. Physical findings
These are summarized inTable 2. Truncal ataxia-was l___ *3 AA in all11 tne aocumented patients witn ataxic gait, out limb ataxia was only found in 17 patients. -A
-A..!
-!.A
therapy and speech therapy. Twelve ofthe 15 patients with cerebrovascular diseases (80%) improved significantly with rehabilitation. ;Seven of these paients who had a single stroke attended the day hospital after discharge from the hospital for an average period of 4 weeks. They improved remarkably and pursued an independent life at home. The other five patients attendedl the ilay h6spital for -loinger period (average df(8 weeks). Three patients who had multiple strokes and cognitive impairment did not respond to treatment. Four of the nine patients with degenerative/ hereditary ataxia (44%) responded to the physical therapy while five patients showed little improvement. Patients with normal pressure hydrocephalus had surgery and their disability improved considerably.
Long term care Fourteen patients (34%) moved to residential care; eight in elderly people homes -and six in nursing homes. Twenty-seven patients continued to live in their own Qhomes with1 some community support.
1---_
CT scan finding Thirty-five patients had cranial CT scan. Sixteen patients showed cerebral atrophy. In three patients there was evidence of atrophy of the cerebelar verbnis. In the group of patients with cerebrovascular disease, CT scan showed multiple infarct disease in five patients, lacunar infaretions in two and periventricular white matter hypodensity in three patients. Two patients had cerebellar metastases from bronchial carcinoma. In three patients the diagnosis of normal pressure hydrocephalus was established from the clinical picture, the CT scan findings and the neurosurgical pressure studies. In one patient CT scan showed cerebral atrophy but magnetic resonance imaging showed a tumour arising from the uncus. Associated neurological conditions Peripheral neuropathy was fbund in three patients. Two of them had hereditary ataxia and the third patient had a history of alcoholism. Two patients had parkinsonism and one patient had-choreoathetosis. There was evidence of pyramidal'signs in two patients, with hereditary ataxia. Twelve patients had dementia.
Drug history Two patients were on phenytoin for epilepsy. One of them had phenytoin toxicity but the other patient had a normal serum level. The ataxia disappeared after stopping the phenytoin in both cases.
Complications and long term prognosis All patients were referred with falls and loss of balance. Four patientshad emoralneckfracturesand three had other fractures (two Colles' fracture and one ribs). In a 5-year follow-up five patients died with bronchopneumonia (11 % mortality) and the patients with bronchial carcinoma died shortly after the diagnosis., Two patients had postmortem examination which showed evidence of generaized cerebral atrophy. Treatment and its outcome All patients were referred to the day hospital and received someform of physiotherapy, occupational
Discussion In a retrospective study of 41 patients with cerebellar ataxia, cerebrovascular diseases were the most commoncause (15 patients,37%). These patients had a .history of neurological deficits of sudden onset gsugestive 6f: vascular causes Most ofithem; had cerebellar signs of limb and truncal ataxia with dysarthria, nystagmus and other brain stem signs. In 3 patients there was only evidence oftruncal ataxia and the CT scan showed periventricular white matter hypodensity and mild cerebral atrophy. The most probable cause for their gait disturbances was small vessel disease (Binswanger's disease)7. CT scan wa undertaken in 35 patients. In three patients it showed atrophy -of the cerebbllar vermis. Previousstudies hate shown that cerebellar vermal atrophy may occur selectivel with aging; and there is no correl-ation between the radiolgical findingsand the cluncal --nifestatio; The CT scwas inly helpful in diagnosing specific diseases such as tunlours and hydrocephalus.? Five patients died with bronDdopneuonoa (mortality rate 11%). Interestingly ,theses five patients had dementia and the underlying diagnosis was cerebrovascular disease. Several reports have shown increased mortality in patients with Alzheimer's disease and multi-infaret dementia where cognitive impairment is a major determinant factor regarding mortality andmorbidity_ in -the elderly9. -In fact the present study indicatedithat-patients with-dmentia showed rapid deterioration regardingg obility and functional abilities. Three patients had normal.pressure hy phalusz They presented with ataxia, falls; iucbntinence and some cognitive impairment T-hey all had a shunt operation and-their disabilitieshave improved remarkably. Several studies have shown that shunt produe aremarabletoration of-the opera nOurol6g functions4 Ovorl, about 50% improveiO, -btit resutli depend on the appropriate selection of procedure. Shuntinghowever, still remains a controversial procedure because rgery may result in conplications, such aswinfection obstruction, epilepy or subdural haematoma, in about 30* of casesi°. Reversible. cerebellar dysfunctions have long been recognized as an acute effect- f phekiytin intoxication.
Journal of the Royal Society of Medicine Volume 85 August 1992
Cerebellar degeneration has been reported in epileptic patients on long term phenytoin therapy even if the serum levels are within the considered normal level'1. The mechanism of this degeneration is uncertain. In our series two epileptic patients developed ataxia after they had been on phenytoin therapy for some years. In one patient the serum level was not toxic and both patients' ataxia resolved after withdrawing the drug. Although many of the patients had untreatable causes, they attended the day hospital for various periods and received some form of physiotherapy, occupational therapy and speech therapy. These treatment modalities were helpful in improw ing their mobility and quality of life and i nig their independence. The study showed that patients with cerebrovascular diseases responded"well t treatment especially those who had a single strokle with no cognitive impairment. On-the.otherJiahd patients with long-standing degenerative' d1soideri, multiple sclerosis or recurrent strokes had less satisfactory responses. All the patients who moved to residential care had no relatives or spouse to look after them, but 66% of the patients continued to live in their own home with community support. Out of 624 elderly patients referred to the unit with gait disorder and falls, 45 patients were identified to have cerebellar ataxia (7%). These findings are similar to previous results6 indicating that cerebellar ataxia is an uncommon cause of gait disorders in the elderly. The study showed -that in five patients ataxia was reversible (three with normal pressure hydrocephalus and two with phenytoin toxicity). In 16 patients it was treatable though irreversible (12 with CVA and four with degenerative conditions). Our data indicated that 21 patients responded to treatment and their ataxia resolved completely. We would like to emphasize that
a significant proportion of elderly patients with ataxia may have reversible or treatable conditions and can pursue an independent life. References 1 Era P. Posture and control in the elderly. Int J Technol Ageing 1988;1:166-79 2 Woollacot MH, Shumway-Cook A, Nashner LM. Aging and posture control. Changes in sensory organisation and muscular co-ordinations. Int J Aging Hum Dev 1986;23:93-114 3 Brockelhurst JC, Robertson D, James-Groom P. Clinical correlations of sway in old age - sensory modalities. Age Ageing 1982;11:1-10 4 Newman G, Dovenmuehle RH, Busse EW. Alterations in neurologic status with age. J Am Geriatr Soc 1960;8:915-17 5 Tinetti ME, Speechley M, Ginter SF. Risk factors for falls among elderly persons living in the community. N Engl J Med 1988;319:1701-7 6 Sudarsky L, Ronthal M. Gait disorders among elderly patients: a survey study of 50 patients. Arch Neurol 1983;40:740-3 7 Babikian V, Ropper AH. Binswanger's disease: a review. Stroke 1987;18:2-12 8 Koller WC, Glatt S, Perlik S, Huckman MS, Fox JH. Cerebellar atrophy demonstrated by computed tomography. Neurology (NY) 1981;31:405-13 9 Molsa P, Marttila R, Rinne U. Survival and cause of death in Alzheimer's disease and multi-infarct dementia. Acta- Neurol Scand 1986;74:103-7 10 Black P. Idiopathic normal pressure hydrocephalus. Results of shunting in 62 patients. J Neurosurg 1980;522:371-7 11 McLain LW, Martin JT, Allen JH. Cerebellar degeneration due to chronic phenytoin therapy. Ann Neurol 1980;7:18-23
(Acepted 30 October 1991)
451
449
Cerebellar ataxia in the elderly
A F Safe MSc MRCP
S Cooper mB MRcP
A C M Windsor FRCP
Department of Geriatric Medicine,
Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW Keywords: cerebellar ataxia; elderly patients; prognosis
Summary In a retrospective study of 624 elderly patients referred with falls and gait disorders, 45 patients were found to have ataxia. Cerebrovascular diseases were the most common underlying cause of ataxia (15 patients, 37%). Nine patients had hereditary/ degenerative cerebellar ataxia. History suggesting alcohol as an underlying cause was established in two patients with cerebellar ataxia. Three patients had normal pressure hydrocephalus and their condition improved remarkably after surgery. No definite cause was found in five patients. Cranial computed tomography (CT) showed cerebral atrophy in 16 patients and in three patients there was evidence of atrophy of the cerebellar vermis. Four patients had femoral neck fractures and three patients had other fractures. In a 5-year follow-up five patients died with bronchopneumonia (1 1% mortality) and patients with dementia showed rapid deterioration. All patients were referred to the day hospital for rehabilitation. The best treatment outcome was achieved in patients who had a single cerebrovascular accident with no cognitive impairment and in those whose ataxia was secondary to medication. Fourteen patients (44%) moved to residential care while 27 (66%) continued to live in their homes with community support. We concluded that there is no evidence of increased mortality in the elderly patients with cerebellar ataxia. CT scan is mainly helpful in diagnosing specific diseases such as tumours or hydrocephalus. A significant proportion of elderly patients with ataxia may have reversible or treatable conditions and can pursue an independent life.
Introduction Normal body posture and locomotor control depend on afferent influxes from three main sensory modes; the proprioceptive endings, vision and vestibulocerebellar connectionsl. These postural responses are slowed in older subjects2 and sway while standing increases with advancing age3. As a result gait disturbances are common in the elderly -and can be disabling. In one population based study, 15% of the subjects over 60 years of age had some abnormality of gait4. Gait disorders contribute to the risk of, falling5 which is a major cause of -morbidity among elderly persons. Non-ambulatory patients have excess morbidity and mortality and often require long term nursing care. Ataxia is a rare cause of gait disorders. In a survey study of 50 elderly patients, cerebellar ataxia accounted for 8% and sensory imbalance for 18% of gait disorders6. There. are few reports in the literature about ataxia in the elderly. The purposes of this retrospective study are to establish the causes
of cerebellar ataxia in the elderly, its response to treatment and its long term prognosis. Patients and methods The study population was composed of elderly patients >65 years who had been referred to the unit in the last 5 years with gait disturbances and found to have cerebellar ataxia. The patients were examined independently by at least two experienced physicians. Patients were defined as having cerebellar ataxia if they had truncal ataxia and an ataxic gait. Patients with hemiparesis or major motor defects from strokes had been excluded from the study. The diagnosis of stroke was made when cranial computed tomography (CT) showed clear evidence of cerebral infarction. Data collection Data were collected from the HAA system in the hospital, from the patients' case notes and by contacting the general practitioners and community services regarding long term care.
Results Forty-five patients were identified to have definite cerebellar ataxia out of 624 patients referred with falls and gait disorders. Four patients were,excluded as their full clinical details were not available or it was not possible to trace their long term care. Fortyone patients were included in the study (21 females and 20 males). Their mean age at presentation was 76 years (age range was 65-83 years). Four patients were diagnosed some years before referral; two with multiple sclerosis and two with hereditary ataxia. Causes of ataxia These are shown in Table 1. Cerebrovascular disease wasthe most common underlying cause accounting for 37% of cases. Three patients had cerebellar ataxia and peripheral neuropathy. History of heavy intake Table 1. Causes of cerebellar ataxia in 41 elderly patients
Diseases
Number of patients
Cerebrovascular diseases
15
Hereditary/degenerative
9 2 2 2 3 3 5 41
Multiple sclerosis Alcohol Diugs (phenytoin) Normal pressure hydrocephalus Tumours (primary & secondaries)
Unknowni Total
0141-0768/92/
080449-03/S02.00/0 © 1992 The Royal Society of Medicine
450
Journal of the Royal Society of Medicine Volume 85 August 1992
Table 2. Relevant physical findings in 41 patients with cerebellar ataxia
Physical signs
Number of patients
Truncal ataxia Limb ataxia Nystagmus Dysarthria Upward plantars Pes cavus
41 17 21 18 8 3
of alcohol was established in two patients. TIwo patients had cerebellar metastases from bronchial carcinoma, but in the review no cases of nonmetastatic carcinomatous cerebellar degeneration were identified. Physical findings
These are summarized inTable 2. Truncal ataxia-was l___ *3 AA in all11 tne aocumented patients witn ataxic gait, out limb ataxia was only found in 17 patients. -A
-A..!
-!.A
therapy and speech therapy. Twelve ofthe 15 patients with cerebrovascular diseases (80%) improved significantly with rehabilitation. ;Seven of these paients who had a single stroke attended the day hospital after discharge from the hospital for an average period of 4 weeks. They improved remarkably and pursued an independent life at home. The other five patients attendedl the ilay h6spital for -loinger period (average df(8 weeks). Three patients who had multiple strokes and cognitive impairment did not respond to treatment. Four of the nine patients with degenerative/ hereditary ataxia (44%) responded to the physical therapy while five patients showed little improvement. Patients with normal pressure hydrocephalus had surgery and their disability improved considerably.
Long term care Fourteen patients (34%) moved to residential care; eight in elderly people homes -and six in nursing homes. Twenty-seven patients continued to live in their own Qhomes with1 some community support.
1---_
CT scan finding Thirty-five patients had cranial CT scan. Sixteen patients showed cerebral atrophy. In three patients there was evidence of atrophy of the cerebelar verbnis. In the group of patients with cerebrovascular disease, CT scan showed multiple infarct disease in five patients, lacunar infaretions in two and periventricular white matter hypodensity in three patients. Two patients had cerebellar metastases from bronchial carcinoma. In three patients the diagnosis of normal pressure hydrocephalus was established from the clinical picture, the CT scan findings and the neurosurgical pressure studies. In one patient CT scan showed cerebral atrophy but magnetic resonance imaging showed a tumour arising from the uncus. Associated neurological conditions Peripheral neuropathy was fbund in three patients. Two of them had hereditary ataxia and the third patient had a history of alcoholism. Two patients had parkinsonism and one patient had-choreoathetosis. There was evidence of pyramidal'signs in two patients, with hereditary ataxia. Twelve patients had dementia.
Drug history Two patients were on phenytoin for epilepsy. One of them had phenytoin toxicity but the other patient had a normal serum level. The ataxia disappeared after stopping the phenytoin in both cases.
Complications and long term prognosis All patients were referred with falls and loss of balance. Four patientshad emoralneckfracturesand three had other fractures (two Colles' fracture and one ribs). In a 5-year follow-up five patients died with bronchopneumonia (11 % mortality) and the patients with bronchial carcinoma died shortly after the diagnosis., Two patients had postmortem examination which showed evidence of generaized cerebral atrophy. Treatment and its outcome All patients were referred to the day hospital and received someform of physiotherapy, occupational
Discussion In a retrospective study of 41 patients with cerebellar ataxia, cerebrovascular diseases were the most commoncause (15 patients,37%). These patients had a .history of neurological deficits of sudden onset gsugestive 6f: vascular causes Most ofithem; had cerebellar signs of limb and truncal ataxia with dysarthria, nystagmus and other brain stem signs. In 3 patients there was only evidence oftruncal ataxia and the CT scan showed periventricular white matter hypodensity and mild cerebral atrophy. The most probable cause for their gait disturbances was small vessel disease (Binswanger's disease)7. CT scan wa undertaken in 35 patients. In three patients it showed atrophy -of the cerebbllar vermis. Previousstudies hate shown that cerebellar vermal atrophy may occur selectivel with aging; and there is no correl-ation between the radiolgical findingsand the cluncal --nifestatio; The CT scwas inly helpful in diagnosing specific diseases such as tunlours and hydrocephalus.? Five patients died with bronDdopneuonoa (mortality rate 11%). Interestingly ,theses five patients had dementia and the underlying diagnosis was cerebrovascular disease. Several reports have shown increased mortality in patients with Alzheimer's disease and multi-infaret dementia where cognitive impairment is a major determinant factor regarding mortality andmorbidity_ in -the elderly9. -In fact the present study indicatedithat-patients with-dmentia showed rapid deterioration regardingg obility and functional abilities. Three patients had normal.pressure hy phalusz They presented with ataxia, falls; iucbntinence and some cognitive impairment T-hey all had a shunt operation and-their disabilitieshave improved remarkably. Several studies have shown that shunt produe aremarabletoration of-the opera nOurol6g functions4 Ovorl, about 50% improveiO, -btit resutli depend on the appropriate selection of procedure. Shuntinghowever, still remains a controversial procedure because rgery may result in conplications, such aswinfection obstruction, epilepy or subdural haematoma, in about 30* of casesi°. Reversible. cerebellar dysfunctions have long been recognized as an acute effect- f phekiytin intoxication.
Journal of the Royal Society of Medicine Volume 85 August 1992
Cerebellar degeneration has been reported in epileptic patients on long term phenytoin therapy even if the serum levels are within the considered normal level'1. The mechanism of this degeneration is uncertain. In our series two epileptic patients developed ataxia after they had been on phenytoin therapy for some years. In one patient the serum level was not toxic and both patients' ataxia resolved after withdrawing the drug. Although many of the patients had untreatable causes, they attended the day hospital for various periods and received some form of physiotherapy, occupational therapy and speech therapy. These treatment modalities were helpful in improw ing their mobility and quality of life and i nig their independence. The study showed that patients with cerebrovascular diseases responded"well t treatment especially those who had a single strokle with no cognitive impairment. On-the.otherJiahd patients with long-standing degenerative' d1soideri, multiple sclerosis or recurrent strokes had less satisfactory responses. All the patients who moved to residential care had no relatives or spouse to look after them, but 66% of the patients continued to live in their own home with community support. Out of 624 elderly patients referred to the unit with gait disorder and falls, 45 patients were identified to have cerebellar ataxia (7%). These findings are similar to previous results6 indicating that cerebellar ataxia is an uncommon cause of gait disorders in the elderly. The study showed -that in five patients ataxia was reversible (three with normal pressure hydrocephalus and two with phenytoin toxicity). In 16 patients it was treatable though irreversible (12 with CVA and four with degenerative conditions). Our data indicated that 21 patients responded to treatment and their ataxia resolved completely. We would like to emphasize that
a significant proportion of elderly patients with ataxia may have reversible or treatable conditions and can pursue an independent life. References 1 Era P. Posture and control in the elderly. Int J Technol Ageing 1988;1:166-79 2 Woollacot MH, Shumway-Cook A, Nashner LM. Aging and posture control. Changes in sensory organisation and muscular co-ordinations. Int J Aging Hum Dev 1986;23:93-114 3 Brockelhurst JC, Robertson D, James-Groom P. Clinical correlations of sway in old age - sensory modalities. Age Ageing 1982;11:1-10 4 Newman G, Dovenmuehle RH, Busse EW. Alterations in neurologic status with age. J Am Geriatr Soc 1960;8:915-17 5 Tinetti ME, Speechley M, Ginter SF. Risk factors for falls among elderly persons living in the community. N Engl J Med 1988;319:1701-7 6 Sudarsky L, Ronthal M. Gait disorders among elderly patients: a survey study of 50 patients. Arch Neurol 1983;40:740-3 7 Babikian V, Ropper AH. Binswanger's disease: a review. Stroke 1987;18:2-12 8 Koller WC, Glatt S, Perlik S, Huckman MS, Fox JH. Cerebellar atrophy demonstrated by computed tomography. Neurology (NY) 1981;31:405-13 9 Molsa P, Marttila R, Rinne U. Survival and cause of death in Alzheimer's disease and multi-infarct dementia. Acta- Neurol Scand 1986;74:103-7 10 Black P. Idiopathic normal pressure hydrocephalus. Results of shunting in 62 patients. J Neurosurg 1980;522:371-7 11 McLain LW, Martin JT, Allen JH. Cerebellar degeneration due to chronic phenytoin therapy. Ann Neurol 1980;7:18-23
(Acepted 30 October 1991)
451
