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PubMed1989

[Correlation of clinical course with MRI findings in olivo-pontocerebellar atrophy and late-cortical cerebellar atrophy].

We quantitatively analyzed 1.5 T MRI in 36 cases of sporadic spinocerebellar degeneration (SCD) and 30 control cases without intracranial lesions, using graphic analyzer. SCD consisted of 21 olivo-ponto-cerebellar atrophy (OPCA) and 15 late cortical cerebellar atrophy (LCCA). There was negative correlation between vermian size and the duration of illness both in OPCA (r = 0.8960, p less than 0.001) and LCCA (r = 0.7756, p less than 0.01), but the progression rate in OPCA was three times greater than that in LCCA. LCCA was suggested the preclinical vermian atrophy by the statistical regression study. In OPCA, the duration of illness also revealed significant correlations with atrophy of ventral pons (r = 0.8308, p less than 0.001) and also cerebellar hemisphere (medial hemisphere; r = 0.7278, p less than 0.001. lateral hemisphere; r = 0.6039, p less than 0.01). OPCA showed diffuse atrophy of cerebellar hemisphere, whereas LCCA showed medial dominant atrophy. OPCA demonstrated significant correlation between the fourth ventricle dilatation and the duration of illness (r = 0.6005, p less than 0.01). A discriminant study significantly separated OPCA, LCCA and control each other by sizes of ventral pons and cerebellar vermis (p less than 0.001). In T2 weighted MRI, 10 cases out of 14 LCCA did not show hypointensity in dentate nucleus in spite of normal appearance in the other portions usually decreased intensity. The dentate nucleus of OPCA showed a significant atrophy. The incidence of putaminal hypointensity in OPCA was significantly greater than that of control group (chi-square = 6.476, p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1996

Decreased striatal monoaminergic terminals in olivopontocerebellar atrophy and multiple system atrophy demonstrated with positron emission tomography.

We used [11C]dihydrotetrabenazine, a new ligand for the type 2 vesicular monoamine transporter (VMAT2), with positron emission tomography to study striatal monoaminergic presynaptic terminals in 4 patients with multiple system atrophy, 8 with sporadic olivopontocerebellar atrophy, and 9 normal control subjects. Specific binding in the striatum was significantly reduced in the multiple system atrophy patients as compared with the normal control group, with average reductions of 61% in the caudate nucleus (p = 0.002) and 58% in the putamen (p = 0.009). Smaller reductions were found in the sporadic olivopontocerebellar atrophy group, averaging 26% in the caudate nucleus (p = 0.05) and 24% in the putamen (p = 0.11). Mean blood-to-brain [11C]dihydrotetrabenazine transport (K1) was significantly different between groups only in the cerebellum, with values for the sporadic olivopontocerebellar atrophy group diminished compared with the normal control group. Cerebellar K1 was not significantly decreased in the multiple system atrophy group. The finding of reduced striatal VMAT2 in sporadic olivopontocerebellar atrophy patients suggests nigrostriatal pathology, indicating that some may later develop symptomatic extrapyramidal disease.

Adult↗
PubMed2005

White matter lesions are associated with cortical atrophy more than entorhinal and hippocampal atrophy.

The goal of this study was to examine the relationship between subcortical vascular disease and brain atrophy in patients with Alzheimer's disease (AD) and mixed dementia (i.e., AD and subcortical vascular disease together). MRI was performed on 77 cognitively normal (CN) subjects, 50 AD and 13 mixed dementia patients. Subcortical vascular disease was determined by white matter hyperintensities (WMH) volume and presence of subcortical lacunes. Brain atrophy was measured using total brain cortical gray matter (CGM), entorhinal cortex (ERC) and hippocampal volumes. CGM volume, but not ERC or hippocampal volume was inversely related to WMH volume in patients and controls. In contrast, no relationship was detected between CGM, ERC, or hippocampal volumes and subcortical lacunes. Furthermore, no interaction was found between WMH and diagnosis on cortical atrophy, implying that WMH affect cortical atrophy indifferently of group. These results suggest that subcortical vascular disease, manifested as WMH, may affect cortical atrophy more than ERC and hippocampal atrophy. Further, AD pathology and subcortical vascular disease may independently affect cortical atrophy.

Aged↗
PubMed2006

Optic nerve atrophy and retinal nerve fibre layer thinning following optic neuritis: evidence that axonal loss is a substrate of MRI-detected atrophy.

Magnetic resonance imaging (MRI) measures of brain atrophy are often considered to be a marker of axonal loss in multiple sclerosis (MS) but evidence is limited. Optic neuritis is a common manifestation of MS and results in optic nerve atrophy. Retinal nerve fibre layer (RNFL) imaging is a non-invasive way of detecting axonal loss following optic neuritis. We hypothesise that if the optic nerve atrophy that develops following optic neuritis is contributed to by axonal loss, it will correlate with thinning of the RNFL. Twenty-five patients were studied at least 1 year after a single unilateral attack of optic neuritis without recurrence, with a selection bias towards incomplete recovery. They had MR quantification of optic nerve cross-sectional area and optic nerve lesion length, as well as optical coherence tomography (OCT) measurement of mean RNFL thickness and macular volume, quantitative visual testing, and visual evoked potentials (VEPs). Fifteen controls were also studied. Significant optic nerve atrophy (mean decrease 30% versus controls), RNFL thinning (mean decrease 33% versus controls), and macular volume loss occurred in patients' affected eyes when compared with patients' unaffected eyes and healthy controls. The optic nerve atrophy was correlated with the RNFL thinning, macular volume loss, visual acuity, visual field mean deviation, and whole field VEP amplitude but not latency. These findings suggest that axonal loss contributes to optic nerve atrophy following a single attack of optic neuritis. By inference, axonal loss due to other post-inflammatory brain lesions is likely to contribute to the global MRI measure of brain atrophy in multiple sclerosis.

Adult↗
PubMed2004

Foxo transcription factors induce the atrophy-related ubiquitin ligase atrogin-1 and cause skeletal muscle atrophy.

Skeletal muscle atrophy is a debilitating response to fasting, disuse, cancer, and other systemic diseases. In atrophying muscles, the ubiquitin ligase, atrogin-1 (MAFbx), is dramatically induced, and this response is necessary for rapid atrophy. Here, we show that in cultured myotubes undergoing atrophy, the activity of the PI3K/AKT pathway decreases, leading to activation of Foxo transcription factors and atrogin-1 induction. IGF-1 treatment or AKT overexpression inhibits Foxo and atrogin-1 expression. Moreover, constitutively active Foxo3 acts on the atrogin-1 promoter to cause atrogin-1 transcription and dramatic atrophy of myotubes and muscle fibers. When Foxo activation is blocked by a dominant-negative construct in myotubes or by RNAi in mouse muscles in vivo, atrogin-1 induction during starvation and atrophy of myotubes induced by glucocorticoids are prevented. Thus, forkhead factor(s) play a critical role in the development of muscle atrophy, and inhibition of Foxo factors is an attractive approach to combat muscle wasting.

Adenoviridae↗
PubMed1995

The correlates of cortical sulci atrophy and central atrophy in a cohort of schizophrenia and manic subjects in Nigeria.

The prevalence of brain cortical sulci atrophy and central (subcortical) atrophy among Nigerian psychiatric patients is highlighted and compared with findings from developed countries. The relationship between these indices of brain atrophy and clinical parameters is also examined. Visual ratings of cortical sulci atrophy and central (subcortical) atrophy, assessed on the computed tomography (CT) image console, were compared among 50 patients with schizophrenia, 14 patients with mania and 41 healthy control subjects. The patients with schizophrenia and the patients with mania had a significantly higher prevalence of brain atrophy than normal subjects. Among the patients with schizophrenia, indices of brain atrophy were not significantly associated with disease outcome, and the presence of negative symptoms. In view of the findings from a parallel study of the same patients that psychiatric patient groups showed other evidence of CT abnormalities, the findings of this study indicate that the so-called functional psychiatric states in developing countries--as in developed countries--are probably associated with some diffuse neuropathological process.

Adolescent↗
PubMed1981

Cerebellar atrophy: relationship to aging and cerebral atrophy.

We studied the incidence of computed tomography evidence of cerebellar atrophy in 20 elderly patients with dementia, 20 age-matched controls, and 40 younger normal subjects. Cerebellar vermian atrophy was present in 6 of 20 demented patients, 7 of 20 elderly controls, and 1 of 40 younger controls. There was no other atrophy of infratentorial structures except for occasional enlargement of the cisterna magna and cerebellopontine angle cisterns. Vermian atrophy did not correlate with cerebral atrophy (enlargement of either lateral ventricles or cortical sulci). None of these patients had clinical signs of cerebellar dysfunction. Therefore, atrophy of the cerebellar vermis may occur selectively with aging, without atrophy of the cerebral cortex, and without clinical manifestations.

Adult↗
PubMedDate not supplied

Childhood progressive spinal muscular atrophy with facioscapulo-humeral predominance, sensory and autonomic involvement and optic atrophy.

A female child of healthy parents developed rotary nystagmus at the age of 15 months. Ophthalmoscopy disclosed incomplete optic atrophy. Blood tests, EEG and CT scans were normal. At 20 months progressive muscular weakness and wasting with limb-girdle distribution commenced, followed later by disturbance of gait. From muscle and nerve biopsy the diagnosis of a peripheral neuropathy with neurogenic muscular atrophy was made. No mental change occurred. At 23 months she sustained cardiac arrest and was resuscitated; thereafter, she remained in a vegetative state and expired 9 months later. Her brain was markedly atrophic and firm. Diffuse old ischemic necroses and neuronal loss with gliosis were found in the cortex, the neostriatum, the thalamus, parts of the lower brainstem, and the cerebellum. Her optic nerves and tracts showed complete atrophy. The spinal cord exhibited degeneration and loss of motor neurons with cervical accentuation. The intermediolateral nuclei, the dorsal nuclei and the spinal ganglia were also involved. There was demyelination of the posterior funiculi, the pyramidal tracts, and the sciatic, peroneal, sural, and superior frontal nerve. The voluntary muscles exhibited large group atrophy with liposclerotic change and limb-girdle predominance. The neck, tongue and ocular muscles were also involved, as were, to a less extent, the lower limbs. Although the loss of motor neurons in the spinal cord and at the bulbar level with the typical pattern of neurogenic muscular atrophy, as well as its distribution, resemble the facioscapulo-humoral type of heredity motor neuropathy (HMN), early onset, rapid course, sensory and autonomic involvement, and atrophy of the optic nerve do not fit this or any one type of HMN.

Atrophy↗
PubMed2004

Characteristic retinal atrophy with secondary "inverse" optic atrophy identifies vigabatrin toxicity in children.

OBJECTIVE: To describe the clinical pattern of retinal atrophy in children caused by the anticonvulsant vigabatrin. DESIGN: An interventional case series report. PARTICIPANTS: One hundred thirty-eight patients, mainly infants, were evaluated regularly for evidence of possible vigabatrin toxicity in the Eye and Neurology clinics at the Hospital for Sick Children, Toronto. METHOD: Sequential clinical and electroretinographic (International Society for Clinical Electrophysiology of Vision standards) evaluations every 6 months. MAIN OUTCOME MEASURES: Presence of recognizable retinal and optic atrophy in the presence of abnormal electroretinogram (ERG) and other clinical findings. RESULTS: Three children being treated for seizures with vigabatrin showed definite clinical findings of peripheral retinal nerve fiber layer atrophy, with relative sparing of the central or macular portion of the retina and relative nasal optic nerve atrophic changes. Some macular wrinkling was evident in 1 case. Progressive ERG changes showing decreased responses, especially the 30-Hz flicker response, supported the presence of decreased retinal function. CONCLUSIONS: A recognizable and characteristic form of peripheral retinal atrophy and nasal or "inverse" optic disc atrophy can occur in a small number of children being treated with vigabatrin. The changes in superficial light reflexes of the retina in children facilitate the clinical recognition of nerve fiber layer atrophy. The macula is relatively spared, although superficial retinal light reflexes indicating wrinkling of the innermost retina suggest early macular toxicity as well. Because these changes are accompanied by electrophysiologic evidence of retinal dysfunction, discontinuation of vigabatrin should be strongly considered.

Adolescent↗
PubMedDate not supplied

[Olivopontocerebellar atrophy in the context of progressive cerebellar atrophies (a clinico-anatomical study)].

The study of 15 cases of progressive cerebellar atrophies, and especially of the olivopontocerebellar atrophy, that was investigated both clinically and anatomically, has attempted to evidence particularities and correlations existing between these two types of atrophy. Olivopontocerebellar atrophy appears to be an abiotrophy of the cerebellum, considered to be spontaneous, sporadic, and sometimes with a hereditary familial background. It is a systemic disease, predominantly of the neocerebellum and involving the cerebellopedal system. It is at the opposite end of Holmes-type atrophies, and of the cerebello-olivary atrophies of the young (I. T. Niculescu, Th. Hornet, 1936) which mainly involve the paleocerebellum. The disease has a polymorphous symptomatology, it has a slow, progressive evolution with mostly cerebellar signs, with extrapyramidal phenomena and psychical disturbances due to lesions of the telencephalic pathways, and sometimes of the spinal, cerebellar and bulbar proprioceptive afferences, with spinal and bulbar involvement (Cezar Ionel, 1949, 1972).

Adult↗
PubMed2006

Neuronal atrophy and synaptic alteration in a mouse model of dentatorubral-pallidoluysian atrophy.

Dentatorubral-pallidoluysian atrophy (DRPLA) is a hereditary spinocerebellar degeneration caused by expansion of a CAG repeat in the disease protein. Despite the restricted and stable brain lesions, DRPLA patients show a variety of clinical symptoms and the brain exhibits generalized atrophy. In previous studies of DRPLA, we proposed that intranuclear diffuse accumulation of the mutant protein is a significant pathological feature of neurons, and that the variable prevalence of this pathology may be relevant to the variation of symptoms observed in patients with different repeat sizes. In this study, to elucidate the pathogenesis of the brain atrophy in DRPLA, we conducted morphological and statistical analyses of neurons affected by the polyglutamine pathology in DRPLA transgenic (Tg) mice with 129 polyglutamine stretches. Golgi-impregnated pyramidal neurons in cerebral cortical layer V of 15-week-old Tg mice showed significant atrophy of the perikarya and dendrites. Dendritic spines were decreased in number and size, and showed a change in morphology resulting in dominance of stubby spines. Interestingly, dendritic arborization was preserved. Electron microscopy revealed that axons in the pyramis and corpus callosum were also atrophic. The number of axonal microtubules was preserved; however, the inter-microtubule spacing was significantly decreased. In the neuropil of cerebral cortical layers II and III, atrophy of the pre-synaptic areas and lengths of the post-synaptic density was detected, but synaptic vesicle diameter was preserved. These results suggest that neuronal atrophy is an essential feature of the cell pathology in DRPLA and that this is closely related to polyglutamine pathogenesis and development of the clinical phenotype.

Animals↗
PubMed1988

[An experimental study on denervated muscle atrophy--effect of electrostimulation and comparison with immobilization muscle atrophy].

The efficacy of causing muscle atrophy was compared among denervation, arthrodesis and tenotomy in rat anterior tibial muscle. Reduction of wet weight was most pronounced in denervated muscle and least in arthrodesed muscle. Histochemical investigation by ATPase stain revealed that atrophy of Type 1 and Type 2 fiber was more severe in denervated muscle group than in the other two groups. Type 2 fiber atrophy was dominant in denervated muscle and in arthrodesed muscle. Type 1 fiber atrophy was dominant in tenotomized muscle. The effect of electrostimulation on denervated muscle was investigated. Electrostimulation significantly reduced the degree of denervation atrophy. Four weeks after severance of peroneal nerve, tibial nerve-crossing was done and electrostimulation was continued for eight weeks. Recovery of wet weight of re-innervated muscle with electrostimulation was significantly better than that without electrostimulation. Electrostimulation applied to denervated muscle reduced the progress of atrophy and improved the recovery after nerve repair.

Animals↗
PubMed1979

[Classification of tubular testicular atrophies in the diagnosis of sterility. Significance of the so-called "bunte Atrophie"].

In the course of testicular biopsies from 2317 patients in the years 1969--1978 at the Institute of Pathology of Zurich University, 1165 cases with tubular atrophy were retrospectively examined and classified on the basis of distribution of atrophy on the one hand and degree of atrophy (progressive depopulation) on the other. In virtue of this histological classification, an attempt was made to provide an absolutely or relatively reliable statement respecting the fertility situation, which was possible in 25% of the cases. In the group of the so-called "bunte Atrophie" an alteration could be described which is of interest not only because it accounts for a quarter of all testicular atrophies but also because there is a difference between the slight histological alterations and the obviously serious clinical consequences. It was possible to prove that an increase in the atrophy grade characterized by loss of spermatids is already responsible for rapid shrinkage of tubulus diameter in this group.

Atrophy↗
PubMed2003

Fourteen novel OPA1 mutations in autosomal dominant optic atrophy including two de novo mutations in sporadic optic atrophy.

The OPA1 gene, encoding a dynamin-related GTPase that plays a role in mitochondrial biogenesis, is implicated in most cases of autosomal dominant optic atrophy (ADOA). Sixty-nine pathogenic OPA1 mutations have been reported so far. Most of these are truncating mutations located in the GTPase domain coding region (exons 8-16) and at the 3'-end (exons 27-28). We screened 44 patients with typical ADOA using PCR-sequencing. We also tested 20 sporadic cases of bilateral optic atrophy compatible with ADOA. Of the 18 OPA1 mutations found, 14 have never been previously reported. The novel mutations include one nonsense mutation, 3 missense mutations, 6 deletions, one insertion and 3 exon-skipping mutations. Two of these are de novo mutations, which were found in 2 patients with sporadic optic atrophy. The recurrent c.2708_2711delTTAG mutation was found in 2 patients with a severe congenital presentation of the disease. These results suggest that screening for OPA1 gene mutations may be useful for patients with optic atrophy who have no affected relatives, or when the presentation of the disease is atypical as in the case of early onset optic atrophy.

Alternative Splicing↗
PubMed2000

Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy.

This report concerns a rare association of asymmetrical temporal lobe atrophy with multiple system atrophy (MSA). A 53-year-old Japanese woman developed cerebellar ataxia and parkinsonism and was diagnosed as olivopontocerebellar atrophy (OPCA). This patient showed forgetfulness and subsequent disorientation even in the early stage of the disease. She fell into a decorticate state at the age of 64, and died a year later. The autopsy showed MSA with asymmetrical atrophy of temporal lobes, intraneuronal globular inclusions mostly confined to the hippocampus, amygdaloid nucleus, and most abundant in the granule cells in the dentate fascia. These inclusions were intensely argyrophilic and expressed marked immunoreactivity to ubiquitin, but not to neurofilament (NF), tau and paired helical filaments (PHF). Ultrastructurally, they were composed of scattered short filamentous structures of 15 to 30 nm in diameter, ribosome-like granules, mitochondria and lipofuscin. The lack of immunoreactivity against tau, NF and PHF suggests that the inclusions are distinct from Pick bodies. To our knowledge, MSA in association with asymmetrical temporal lobe atrophy with the present neuronal inclusions has not been reported. This case is distinct from MSA combined with atypical Pick's disease in the distribution and immunohistochemical properties of neuronal inclusions, and may present a new variant of MSA since the neuronal inclusions are similar, in many respects, to those of neuronal inclusions reported in MSA. Globular inclusions are also discussed in variants of Pick's disease, amyotrophic lateral sclerosis and Alzheimer's disease.

Atrophy↗
PubMed2000

Cerebral atrophy in multiple system atrophy by MRI.

Cranial magnetic resonance images (MRI) of the cerebral areas of 40 patients with multiple system atrophy (MSA) and of 61 age-matched controls were analyzed. The cerebral area of MSA patients was 131. 95+/-15.89 cm(2) (mean+/-S.D.), which was significantly smaller than that of normal controls at 149.01+/-10.93 cm(2) (P<0.0001). All 23 MSA cases subjected to the MRI study over a 1-year period showed progressive cerebral atrophy, and the atrophy rate was 2.46+/-1. 66%/year. There were no significant differences within the MSA subtypes or between gender. The progression of cerebral atrophy in MSA correlated more with duration (r=-0.634) than age (r=-0.421). We conclude that MRI findings throughout the course of MSA suggest progressive cerebral atrophy, which is common in all subtypes and reflects duration of the disease rather than age.

Aged↗
PubMed1990

[Cognitive function, cerebral blood flow and brain atrophy in olivo-ponto-cerebellar atrophy].

UNLABELLED: Eighteen cases of OPCA (9 males and 9 females, age 39-70, mean 57.8 +/- 8.1 years). Mean duration of illness was 65.7 +/- 36 months. Age-matched controls for Brain-CT were 34 cases (13 males and 21 females) (age 39-70, mean 56.8 +/- 9.4 years), in which CT was examined because of functional disorders, and 50 healthy volunteers (25 males and 25 females) (age 36-72, mean 56.6 +/- 7.9 years) for regional cerebral blood flow (rCBF) and mental function. Brain Atrophy Index (BAI) and Ventricular Atrophy Index (VAI) were calculated by digitizer (PCPAD) on CT films in the slice of lateral ventricle level, basal ganglia level and cerebello-pontine level. There were 10 patients of OPCA who examined CT prior to this study. Interval of the two examinations was 21-76 months, mean 53 months. Regional cerebral blood flow (rCBF) were measured by 133 Xe inhalation methods. Intelligence were evaluated with Okabe's Intelligence Scale (Shortened and modified Wechsler's Memory Scale for Japanese Aged) and Kohs' Block Design Test. Depressive state was evaluated with Zung's Self-rating Depression Scale (SDS). RESULTS: 1) Cerebello-pontine atrophy was significantly prominent but no cerebral atrophy was observed in OPCA. 2) The rCBF was mildly but significantly reduced in OPCA. 3) Performance and verbal intelligence were significantly low in OPCA. 4) OPCA patients showed significant depressive state. 5) Grade of ADL did not show significant participation in the rCBF, intelligence and depressive state. 6) There was no significant correlation between duration of illness and rCBF or mental functions. 7) Cerebello-pontine atrophy significantly progressed in OPCA patients followed-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1998

[An autopsied case of multiple system atrophy with remarkable cerebral atrophy].

In this paper, we report the pathological findings of a multiple system atrophy (MSA) patient with remarkable frontal lobe atrophy. The patient was a 65-year-old woman with a 13-year history of untreatable parkinsonism, dysautonomia and progressive motor aphasia. The cranial imaging study disclosed progressive atrophy of the frontal lobe, striatum and pontocerebellar system. She died of pneumonia. The brain weight was 810 g, and there was remarkable atrophy of the cerebrum predominantly in the frontal lobe, striatum, pons and cerebellum. The microscopic examination revealed a preserved cortical structure with laminar gliosis in the sixth layer of the precentral and superior frontal gyri of the frontal lobe, and postcentral gyrus and inferior parietal lobule of the parietal lobe. The cortices of these regions also revealed to be in a spongy state in the second layer, and mild cell loss was seen in the fifth and six layers. The frontal lobe white matter showed a slight pallor, a mild loss of myelinated fiber and axon, and mild gliosis. Glial cytoplasmic inclusions (GCIs) were abundantly observed in the deep layer of the cortex in the regions mentioned above, and were more abundant in the white matter of the frontal and parietal lobes, callosal body, and internal, external and extreme capsules. There were severe degenerations in the olivopontocerebellar and striatonigral systems, and GCIs in widespread regions of the brain. We could not detect any Pick bodies, Lewy bodies, ballooned neurons, senile plaques, or any significant amounts of neurofibrillary tangles. There were no vascular changes. Thus, this was a verified MSA patient with progressive aphasia and remarkable frontal lobe atrophy. We indicate a possible involvement of the cerebral lobes in MSA.

Aged↗