Showing papers in "JAMA Neurology in 1987"

The clinical diagnosis of Alzheimer's disease

Abstract: • Clinical and pathologic diagnoses are compared in 65 patients who had dementia and who had been studied longitudinally during life. The sensitivity of diagnosis for dementia of the Alzheimer type (DAT) without any other diagnosis was 87%, and the specificity was 78%. The ischemic scale score did not discriminate well between patients with pure multi-infarct dementia and those with both DAT and multi-infarct dementia. However, 35 of 38 cases of pure DAT had a score of 4 or less on the ischemic scale.

Right Cerebral Dominance in Spatial Attention: Further Evidence Based on Ipsilateral Neglect

Abstract: • Tasks based on visuomotor scanning and tactile exploration were used to quantitate neglect behavior in patients with unilateral brain damage and in normal control subjects. The results confirm previous observations that contralateral neglect is markedly more severe following right-hemisphere injury and that it is independent of the modality of sensory input or motor output. In addition, patients with right-hemisphere injury also showed multimodal neglect for targets in the hemisphere ipsilateral to the brain lesion. The emergence of both contralateral and ipsilateral neglect in these patients strongly supports a model of right-hemispheric dominance for the distribution of attention within the extrapersonal space.

Mood Disorder and Multiple Sclerosis

Abstract: A systematic psychiatric evaluation was performed on 100 consecutive patients attending a multiple sclerosis clinic. Forty-two percent of the patients had lifetime history of depression, and 13% fulfilled criteria for manic-depressive illness. Only 28% of the patients had no psychiatric diagnosis. The relationship of psychiatric disorder to neurologic dysfunction and other aspects of multiple sclerosis are presented. Clinical and theoretical implications of these findings are discussed.

Neuropsychiatric aspects of multi-infarct dementia and dementia of the Alzheimer type

Abstract: • Delusions, depression, and hallucinations were assessed in 30 patients with dementia of the Alzheimer type (DAT) and 15 with multi-infarct dementia (MID). The nature and prevalence of delusions did not distinguish DAT from MID: delusions were present at the time of examination in 30% of patients with DAT and in 40% of patients with MID, and had occurred at some time in the course of the illness in half the patients of each diagnostic group. Delusions were primarily paranoid in type and involved elementary misbeliefs concerning theft or infidelity. Depression was significantly more common in MID than DAT. Seventeen percent of patients with DAT had depressive symptoms; none with severe depression were identified. Four of 15 patients with MID exhibited major depressive episodes and 60% manifested depressive symptoms. Depression and delusions were not deducted in patients with severe dementia. Hallucinations occurred in both diagnostic groups but were not common: one patient with DAT and one with MID had auditory hallucinations, and three patients with MID had visual hallucinations.

The acquired immunodeficiency syndrome dementia complex as the presenting or sole manifestation of human immunodeficiency virus infection.

Abstract: • Twenty-nine patients at risk of developing acquired immunodeficiency syndrome (AIDS) presented with cognitive, motor, and behavioral dysfunctions characteristic of the AIDS dementia complex, either preceding or in the absence of major systemic opportunistic infections or neoplasms. Six of these patients were medically well, while the remainder suffered only milder manifestations of the AIDS-related complex at the time of their neurologic presentation. Over half of these patients either survived for five to 16 months or died without exhibiting systemic manifestations of AIDS. This experience indicates that the AIDS dementia complex may be the earliest, and, at times, the only evidence of human immunodeficiency infection, and that its development in this context may present a diagnostic challenge, particularly in individuals in whom risk for infection by the AIDS virus is cryptic.

Reflex Sympathetic Dystrophy: A Review

Abstract: • Reflex sympathetic dystrophy is a syndrome of burning pain, hyperesthesia, swelling, hyperhidrosis, and trophic changes in the skin and bone of the affected extremity. It is precipitated by a wide variety of factors in addition to nerve injury. It occurs outside of dermatomal distributions and can spread to involve other extremities without new injury. The diagnosis is primarily clinical, but roentgenography, scintigraphy, and sympathetic blockade can help to confirm the diagnosis. The most successful therapies are directed toward blocking the sympathetic innervation to the affected extremity, in conjunction with physical therapy. The theories proposed to explain the pathophysiology of reflex sympathetic dystrophy include "reverberating circuits" in the spinal cord that are triggered by intense pain, ephaptic transmission between sympathetic efferents and sensory afferents, and the presence of ectopic pacemakers in an injured nerve.

Ischemic stroke in adults younger than 30 years of age. Cause and prognosis.

Abstract: • Because the cause and prognosis of ischemic stroke in adults younger than 30 years of age are not known, we observed 41 such patients (26 females and 15 males) using a standard protocol of investigations, including cerebral angiography and echocardiography. Mitral valve prolapse and arterial dissection accounted for 51% of infarcts, migrainous infarction was the likely cause in 15% of infarcts, and uncommon causes accounted for 34% of infarcts. Atherosclerosis played a role in only two patients. Two thirds of the women were taking oral contraceptives, which may have been the primary cause of stroke in one woman who had recurrent venous thromboses followed by thrombosis of the aortic arch. Three patients (7.3%) died of acute causes. During follow-up (mean, 46 months), the annual incidence of death (0.7%) and recurrent stroke (0.7%) was low. Eighty-one percent of the survivors could return to work. We conclude that cerebral angiography and echocardiography are indicated in all adults younger than 30 years of age. After the acute phase of stroke, prognosis is reasonable.

Cognitive and neurologic findings in subjects with diffuse white matter lucencies on computed tomographic scan (leuko-araiosis).

Abstract: • As part of a prospective clinicopathologic study, a cohort of 105 "normal" elderly volunteers was investigated with computed tomographic scans, psychometric testing (Extended Scale for Dementia [ESD]) and neurologic examination. Computed tomographic scans were evaluated for the presence or absence of white matter lucencies, termed leuko-araiosis . These are defined as patchy or diffuse areas of decreased attenuation involving only white matter and with no change in adjacent ventricles or sulci. The nine controls with leuko-araiosis had lower scores on the ESD than the 96 controls without leuko-araiosis (mean ESD with leuko-araiosis, 227.1 ± 14; without leuko-araiosis, 237.1 ± 8), and the difference remains significant even after adjusting for the possible confounding effects of age, sex, education, and infarct detected on computed tomography. Significant differences were also found comparing subjects with leuko-araiosis and those without in respect to abnormal gait, limb power, plantar response, and the rooting and palmomental reflexes. Leuko-araiosis may represent a marker for early dementia. The pathophysiology of this finding remains uncertain. Our results suggest that white matter abnormalities play a role in the development of intellectual impairment in the elderly.

Spontaneous Carotid Dissection With Acute Stroke

Abstract: • Thirty (2.5%) of 1200 consecutive patients with a first stroke had a spontaneous dissection with occlusion of the cervical internal carotid artery (ICA). A suggestive picture with ipsilateral headache and oculosympathetic paresis was uncommon (17%), so that diagnosis was uncertain before angiography. Seven patients died within one week. During follow-up (mean, 3.2 years) with sequential Doppler ultrasonographic testing, 12 survivors had a good recovery and early reopening of the occluded ICA, and 11 had a poor recovery usually without reopening of the ICA. Recurrence of a dissection occurred in only one patient. Large infarcts causing death or a severe disability were associated with an ICA thrombus and distal emboli; the organization of this intraluminal thrombosis may explain the absence of reopening in these cases while resorption of the intramural hematoma developed. Early heparin sodium therapy may help prevent intraluminal clotting without carrying an important risk of extending the dissection, but its clinical benefit remains unproven. Contrary to current opinions, ICA dissection with occlusion causing cerebral infarction may often carry a severe prognosis.

Worsening of neurologic syndrome in patients with Wilson's disease with initial penicillamine therapy.

Abstract: • We describe a patient with Wilson's disease who presented with neurologic disease, was treated with D-penicillamine, and suffered sudden neurologic deterioration coincident with therapy. Replicate brain magnetic resonance imaging examinations after six weeks and 11 months of penicillamine therapy documented the development of new brain lesions during this period, while liver biopsy specimen data disclosed that excellent hepatic decoppering had occurred. To develop information on the relative rarity or frequency of neurologic worsening with the initiation of penicillamine therapy, we conducted a retrospective survey of 25 additional patients with Wilson's disease who met the criteria of presenting with neurologic disease and having been treated with penicillamine. The replies indicate that, at least from the patient's viewpoint, this syndrome occurs frequently. We suggest that the cause of this distressing syndrome, and ways to mitigate or circumvent it, must be discovered.

The Spectrum of Neurologic Disease Associated With Antiphospholipid Antibodies: Lupus Anticoagulants and Anticardiolipin Antibodies

Abstract: • Lupus anticoagulants and anticardiolipin antibodies are antiphospholipid immunoglobulins, predominantly of the IgG, IgM, or mixed class. Recently, these antiphospholipid autoantibodies have been associated with neurologic conditions including focal cerebral and ocular ischemia, the myelopathy of lupoid sclerosis and Degos' disease, Guillain-Barre syndrome, migraine, chorea, and seizures. We review the neurologic manifestations associated with this group of antibodies. Recognition of these conditions may lead to further insights into pathogenesis and therapy.

Fetal Methylmercury Poisoning: Relationship Between Concentration in Single Strands of Maternal Hair and Child Effects

Abstract: • Pregnant women consumed bread that was prepared from methylmercurytreated wheat. Single strands of maternal head hair were analyzed by x-ray fluorescence spectrometry. The index of fetal exposure was the maximum hair mercury concentration during gestation. Effects were measured by the frequency of psychomotor retardation, seizures, and neurological signs in the children. A dose-response relationship was demonstrated for fetal effects of methylmercury. Analysis of single hair strands provides a better index of acute or subacute fetal exposure than analysis of bundles of hair; the duration and degree of exposure are more accurately defined. A sex difference in response is discussed.

Vascular risk factors and leuko-araiosis.

Abstract: • Leuko-araiosis was found in 49 of 140 demented patients compared with 12 out of 110 control subjects. Thirty-one of 95 patients with dementia of the Alzheimer's type had leuko-araiosis. A history of stroke was four times more frequent in patients with leuko-araiosis than in those without leuko-araiosis (17.4% and 4.4%, respectively). It occurred in 25% of controls with leuko-araiosis compared with only 2% of those without leuko-araiosis. Mean systolic blood pressure was associated with leuko-araiosis. No association was found for diastolic blood pressure, myocardial infarction, angina, diabetes, or carotid bruits. On logistic regression analysis, the strong association between dementia and leuko-araiosis was mainly explained by a history of stroke. There are common factors in leuko-araiosis and stroke, but stroke alone does not account for leuko-araiosis.

Clinical Assessment of 31 Patients With Wilson's Disease: Correlations With Structural Changes on Magnetic Resonance Imaging

Abstract: • Thirty-one patients with Wilson's disease were evaluated with detailed neurologic and medical examinations. Mean age (±SD) at onset was 21 ± 5 years and at examination was 28 ± 6 years. Of the 90% of patients who were first treated with penicillamine, 31% deteriorated initially despite therapy, and half never recovered to pretherapy baseline. At the time of our evaluations, the most common neurologic findings were dysarthria (97%), dystonia (65%), dysdiadochokinesia (58%), rigidity (52%), gait and postural abnormalities (42%), and tremor (32%). Chorea and dementia were rare. Twentytwo patients underwent magnetic resonance imaging. All but one of the 19 symptomatic patients had abnormal scans. The three asymptomatic patients had normal scans. Most lesions were seen in the caudate, putamen, subcortical white matter, midbrain, and pons. Generalized brain atrophy was also common. Lesions were less common in the thalamus, cerebellar vermis, midbrain tegmentum, globus pallidus, red nucleus, and dentate nucleus. Dystonia and bradykinesia correlated with putamen lesions, and dysarthria correlated with both putamen and caudate lesions.

Cognitive and Neurologic Findings in Demented Patients With Diffuse White Matter Lucencies on Computed Tomographic Scan (Leuko-Araiosis)

Abstract: • A series of patients referred to the University of Western Ontario, London, Dementia Study for investigation of possible dementia underwent computed tomographic scans, psychometric testing (Extended Scale for Dementia [ESD]), and neurologic examination. Thirty-nine of the 113 patients studied (ischemic score, ≤4) were found to have leuko-araiosis, which we have defined as patchy or diffuse lucencies in the white matter. Patients with leuko-araiosis had significantly lower mean scores on the ESD, 109.7 ± 61.2, compared with mean scores of 148.5 ± 78.0 in those without. However, only a trend toward lower scores on the ESD was observed when age, sex, education, and infarct were taken into account in the analysis of covariance. Leuko-araiosis was found to be associated with increasing age, hypertension, abnormalities of power in the limbs, and extensor-plantar responses in this sample of patients. In patients with Alzheimer's disease (AD) alone, diagnosed clinically, 29 out of 91 demonstrated leuko-araiosis on computed tomography, but scores on the ESD in this group overall were not significantly different when those with and without leukoaraiosis were compared. In less advanced cases, however, a highly significant trend was evident for leuko-araiosis to be associated with increased dementia in AD. The results are consistent with the hypothesis that leuko-araiosis is associated with dementia in AD, and that this is either most marked or most easily identifiable before the dementia becomes very severe.

The diagnosis of dementia with single photon emission computed tomography.

Abstract: • Single photon emission computed tomography is a practical modality for the study of physiologic cerebral activity in vivo. We utilized single photon emission computed tomography andN-isopropyl-p-iodoamphetamine iodine 123 to evaluate regional cerebral blood flow in nine patients with Alzheimer's disease (AD), five healthy elderly control subjects, and two patients with multi-infarct dementia. We found that all subjects with AD demonstrated flow deficits in temporoparietal cortex bilaterally, and that the ratio of activity in bilateral temporoparietal cortex to activity in the whole slice allowed the differentiation of all patients with AD from both the controls and from the patients with multi-infarct dementia. Furthermore, this ratio showed a strong correlation with disease severity in the AD group. Single photon emission computed tomography appears to be useful in the differential diagnosis of dementia and reflects clinical features of the disease.

Pregnancy-Related Primary Brain and Spinal Tumors

Abstract: • We reviewed the literature concerning primary brain and spinal tumors with first manifestation or acceleration of symptoms during pregnancy or within the first postpartum week and encountered four new cases in our center. The incidence of brain tumors that become symptomatic during pregnancy appears to be decreased compared with that in age-matched women. The relative frequency of the different primary brain tumor types is not changed by pregnancy. The number of meningiomas gradually tends to increase during pregnancy, with gliomas and spinal vascular tumors accumulating in the first and third trimesters, respectively. Postpartum amelioration of symptoms has especially been described for meningiomas and spinal vascular tumors. We conclude that different types of tumors are influenced at different stages of pregnancy. Although progesterone receptors predominate compared with estrogen receptors, no definite causal relationship with progesterone has been established.

Multivariate Analysis of Improvement and Outcome Following Stroke Rehabilitation

Abstract: • This study documented the status of 432 patients and characteristics of functional improvements and outcomes achieved by 163 patients who participated in comprehensive stroke rehabilitation Scores on the 100-point Activities of Daily Living Index improved from hospital admission to discharge and declined slightly at follow-up An average Activities of Daily Living Index point gain of 06 per day was found that was unrelated to age, sex, side of hemiparesis, or admission functional status Seventy-nine percent of the patients were discharged home; 85% were home at follow-up Eleven percent of the patients were working at follow-up Patients traveled outside their homes an average of 246 days during the three months immediately following discharge A significant number of patients achieved favorable functional housing, employment, and social outcomes This study supported referral for rehabilitation services regardless of age, side of hemiparesis, or degree of impairment

Magnetic resonance imaging correlates of dementia in multiple sclerosis.

Abstract: • Thirty-two patients with clinically definite multiple sclerosis were evaluated with neuropsychological procedures and magnetic resonance imaging (MRI). Neuropsychological evaluation included assessment of language, memory, cognition, visuospatial skills, and depression. Significant impairment in any three areas, compatible with diagnosis of a dementia syndrome, was observed in 28% of these patients, and lesser or no cognitive impairment characterized the remaining patients. Magnetic resonance imaging was used to evaluate the number and distribution of lesions as well as the presence of cerebral atrophy and atrophy of specific anatomic structures such as the corpus callosum. Results suggest that neither the number of lesions, the distribution of lesions, nor the extent of generalized cerebral atrophy was significantly greater in demented compared with non-demented patients. The primary finding was that atrophy of the corpus callosum was significantly more extensive on MRI scans in demented patients. Although the callosum itself may not be implicated directly in the pathogenesis of dementia, the presence of callosal atrophy on MRI scans should alert the physician to the possible occurrence of dementia in patients with multiple sclerosis.

Cognitive impairment in patients with multiple sclerosis and mild physical disability.

Abstract: • Forty mildly disabled and clinically stable patients with multiple sclerosis (MS), representative of the corresponding population in Northern Holland, with disability Status Scale scores evenly distributed within the 1 to 4 range, were compared with 40 age-, sex-, and education-matched normal controls on a battery of neuropsychological tests. Apart from impairments in perceptual-motor functioning, generally mild deficiencies in intelligence and, specifically, in memory were displayed in the MS group. Attentional processes appeared uncompromised. Increasing fatigue during testing could not account for poor performance. The memory deficits could be attributed to poor initial learning, although there was also evidence suggesting that accelerated forgetting of what had been learned may appear with the progression of MS. Seven patients (17.5%), as compared with none of the controls, were classified by blind clinical judgment of test performance as definitely impaired.

Electroencephalographic Activity After Brain Death

Abstract: • Fifty-six consecutive patients clinically diagnosed as brain dead were seen at Loyola University Medical Center, May-wood, III, from January 1984 through May 1986. Eleven (19.6%) of the 56 patients had electroencephalographic (EEG) activity following the diagnosis of brain death. The mean duration of the observed EEG activity was 36.6 hours (range, two to 168 hours). Three patterns of EEG activity were observed: (1) low-voltage (4 to 20 μV) theta or beta activity was recorded in nine (16.1%) patients as long as 72 hours following brain death. Neuropathologic studies in one patient showed hypoxicischemic neuronal changes involving all cell layers of the cerebral cortex, basal ganglia, brain stem, and cerebellum; (2) sleep-like activity (a mixture of synchronous 30 to 40 μV theta and delta activity and 60 to 80 μV, 10 to 12 Hz spindle-like potentials) was noted in two (3.6%) patients for as long as 168 hours following brain death. Pathologic studies in both cases demonstrated ischemic necrosis of the brain stem with relative preservation of the cerebral cortex; and (3) alpha-like activity (monotonous, unreactive, anteriorly predominant, 25 to 40 μV, 9 to 12 Hz activity) was observed in one (1.8%) patient three hours following brain death. Regardless of activity on the EEG, none of the patients recovered. The occurrence of EEG activity following brain death would suggest reliance on the EEG to confirm brain death may be unwarranted. The use of the EEG as a confirmatory test of brain death may be of questionable value.

Cerebral perfusion imaging in Alzheimer's disease. Use of single photon emission computed tomography and iofetamine hydrochloride I 123.

Abstract: • We used single photon emission computed tomography (SPECT) to study 15 patients with Alzheimer's disease and nine controls. lofetamine hydrochloride I 123 uptake data were recorded from the entire brain using a rotating gamma camera. Activity ratios were measured for the frontal, posterior parietal, posterior, medial, and lateral cortical temporal regions and striate cortex and were normalized by the activity in the cerebellum. Abnormalities in iofetamine hydrochloride I 123 activity were similar to the abnormalities in glucose metabolism observed with positron emission tomography. Cortical tracer activity was globally depressed in patients with Alzheimer's disease, with the greatest reduction in the posterior parietal cortex.

Abnormal neuroanatomy in a nonretarded person with autism. Unusual findings with magnetic resonance imaging.

Abstract: • Recent studies of infantile autism using computed tomographic scanning emphasized the importance of studying cases of classic autism (Kanner's syndrome) without complicating conditions such as mental retardation. Computed tomographic scan studies of such patients reported no evidence of anatomical abnormalities of cerebral hemi-spheres or of subcortical structures, which are defined by landmarks such as the lateral ventricles and lentiform nuclei. Examination of the cerebellum was not mentioned. The most recent postmortem neuropathologic study reported significant cerebellar abnormality, but the study was of a severely retarded autistic individual. Using magnetic resonance imaging, we have found in vivo evidence of a significant and unusual cerebellar malformation in a person with the classic form of autism uncomplicated by mental retardation (current nonverbal IQ = 112), epilepsy, history of drug use, postnatal trauma, or disease. The finding showed hypoplasia of the declive, folium, and tuber in posterior vermis, but not of the anterior vermis, and hypoplasia of only the medial aspect of each cerebellar hemisphere. The right posterior cerebral hemisphere also showed pathologic findings.

Comparison of Two Screening Tests in Alzheimer's Disease: The Correlation and Reliability of the Mini-Mental State Examination and the Modified Blessed Test

Abstract: • The Mini-Mental State Examination (MMSE) and the Blessed Orientaion-Memory-Concentration test (BOMC), a sixitem derivative of the Blessed Information-Memory-Concentration Test, were each administered to 36 patients with a clinical diagnosis of Alzheimer's disease. In 24 patients, both tests were readministered a month later. The correlation between the MMSE and BOMC was -0.83 with a test-retest correlation of 0.89 (MMSE) and 0.77 (BOMC). Factor analysis indicated that the multiple MMSE cognitive components could be explained by two factors, which together accounted for 66% of the variance. These factors are conceptually similar to the components of the BOMC, and so may explain the substantial correlation between the two tests. Since these cognitive status tests seem to be equivalent for Alzheimer patients, the briefer measure (BOMC), which offers additional advantages, may be preferred.

Temporal lobe involvement in multiple sclerosis patients with psychiatric disorders.

Abstract: • Patients with multiple sclerosis (MS) frequently develop psychiatric disturbances. The clinical histories and magnetic resonance imaging results from eight patients with MS and psychiatric disorders were compared with those from eight control MS patients matched for age, sex, duration and severity of illness, and clinical course. Both groups had widespread lesions on the magnetic resonance imaging scans; however, the psychiatric group had more clinically defined sites of MS involvement, possibly indicating a difference in the biologic activity of the lesions in this group. The total lesion area was not different between the groups. However, the distribution of lesions did differ; the psychiatric group had significantly more temporal lobe involvement than the control group. These results indicate a role for the cerebral lesions themselves, particularly those in the temporal lobes, in the genesis of psychiatric disorders in MS.

Clinical investigation of Duchenne muscular dystrophy. Interesting results in a trial of prednisone.

Abstract: • We investigated the effect of highdose prednisone therapy in 33 boys with Duchenne muscular dystrophy. The drug was given daily in doses of 1.5 mg/kg of body weight (to a maximum of 80 mg) for six months. Muscle strength, joint contractures, timed functional tests, functional ability, and pulmonary function were measured at the beginning and end of the treatment period. The trial was designed using natural history controls, and the power of the study was 0.80 to detect a slowing of 50% in the rate of progression. During the period of the trial, muscle strength, functional grades, timed functional tests, and pulmonary function improved. Contractures followed the expected natural history of the illness.

Relationship between lesion extent in 'Wernicke's area' on computed tomographic scan and predicting recovery of comprehension in Wernicke's aphasia

Abstract: • This study investigated the relationship between severity of auditory comprehension in Wernicke's aphasia and amount of temporal lobe damage within Wernicke's area (posterior two thirds of superior temporal gyrus region) as well as the total temporoparietal lesion size. There was a highly significant correlation between comprehension and the amount of temporal lobe lesion in Wernicke's area. There was no significant correlation between comprehension and the total temporoparietal lesion size. Patients with damage in only half or less than half of Wernicke's area had good comprehension at six months after the onset of stroke. Patients with damage in more than half of Wernicke's area had poor comprehension even one year after the onset of stroke. Additional anterior-inferior temporal lobe lesion extension into the middle temporal gyrus area was associated with particularly poor recovery.

Cerebral hemiatrophy, hypoplasia of internal carotid artery, and intracranial aneurysm. A rare association occurring in an infant.

Abstract: • A 4-month-old child with a very rare association of primary (congenital) left cerebral hypoplasia, hypoplasia of the left internal carotid artery, and giant anterior communicating artery aneurysm was seen. The aneurysm was detected and treated before rupture. We briefly reviewed the literature on agenesis (hypoplasia) of the internal carotid artery, intracranial aneurysms in childhood, and primary cerebral hypoplasia. It is proposed that cerebral arteriography, cranial computed tomography, or magnetic resonance imaging be performed in all children with primary cerebral hemihypoplasia to establish an etiologic diagnosis and, more importantly, to detect and treat possible associated intracranial aneurysms before they become symptomatic.

Prednisone treatment in Duchenne muscular dystrophy. Long-term benefit.

Abstract: • We report the long-term results of a therapeutic trial of prednisone in the treatment of Duchenne muscular dystrophy, comparing the age at which 16 treated patients and 38 controls lost the ability to ambulate. Survival curve analysis and group mean comparisons indicate that ambulation was significantly prolonged, by approximately two years, in the prednisone-treated group compared with the control group. The results of this study indicate that prednisone therapy has a long-term beneficial effect in the treatment of Duchenne muscular dystrophy, in addition to the short-term palliation we previously reported. Further studies are needed to establish optimal treatment schedules using prednisone and to develop improved therapeutic agents.

The Electroencephalogram in Alzheimer-Type Dementia: A Sequential Study Correlating the Electroencephalogram With Psychometric and Quantitative Pathologic Data

Abstract: • As part of a longitudinal cohort study of dementia, 139 patients with Alzheimer's disease (dementia of the Alzheimer type, senile dementia of the Alzheimer type, and mixed type [ischemic score, 4 to 7]) and 148 age-matched control subjects were evaluated for electroencephalographic (EEG) abnormalities and their evolution. Electroencephalograms were significantly different in the two groups; EEGs worsened overall in the two groups during a period of one to four years, but most subjects showed no alteration in their EEGs. Some patients showed improvement in their EEG findings during the follow-up period. A strong correlation between EEG grade and psychometric scores was consistently found over sequential studies. In a subgroup of patients on whom autopsies were performed, morphometric neuron loss correlated significantly with EEG severity.