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At least 55 records · Page 3Linked to original sources
PubMed1998

A case of marked cerebellar atrophy in a woman with anorexia nervosa and cerebral atrophy and a review of the literature.

While cerebral atrophy has been shown in patients with anorexia nervosa, cerebellar atrophy has never been reported in these patients. We report a case of cerebral atrophy with marked cerebellar atrophy in a 20-year-old woman with anorexia nervosa admitted to our hospital for severe weight loss. Neuroradiological examinations of the patient showed morphological brain alteration without focal parenchymal lesions. Cranial computerized tomography (CCT) showed an enlargement of the external cerebrospinal fluid spaces, especially those close to the cerebellar cistern. A brain magnetic resonance imaging (MRI) study confirmed the results of the CCT and dynamic single-photon emission tomography (d-SPECT) showed a reduced perfusion of the left brain areas. The reported case shows that some forms of anorexia nervosa have a concomitant presence of cerebral and cerebellar morphological anomalies. At present, it is not possible to demonstrate the whole correlation between the imaging reports and the clinical or neurological symptomatology. Some forms of brain alteration could be secondary to undernutrition; on the other hand, cerebral and cerebellar atrophy and eating disorders are far from clear and may also be an expression of an unknown common denominator.

Adult↗
PubMed1988

Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome). Autopsy case report of two brothers.

Autopsy cases of two brothers with bulbo-spinal muscular atrophy associated with gynecomastia, testicular atrophy and sensory neuropathy are reported. The disease started with finger tremor, proximal muscle weakness and facial muscle twitching at the second and fourth decades, accompanied by bulbar signs and glove-stocking type sensory disturbance. Systemic neurogenic patterns and diminished sensory nerve action potential amplitudes were recorded by electrophysiological studies. A marked loss of myelinated fibers was noticed upon sural nerve biopsy. Gonadal hormone values were normal, except for elevated urinary estrogen. Postmortem examinations revealed a remarkable degeneration of the facial and hypoglossal nuclei, and the spinal cord motoneurons. The skeletal muscles and the tongue showed neurogenic muscular atrophy with fatty replacement. Testicular atrophy was prominent showing hyalinized seminiferous tubuli with nodular and diffuse Leydig cell hyperplasia, containing estrogen immunoreactive substance. These clinical and histological features seemed to be highly compatible with those of Kennedy-Alter-Sung type bulbo-spinal muscular atrophy. The involvement of sensory peripheral nerves, however, was a distinct feature of this family.

Aged↗
PubMed1990

Olivopontocerebellar atrophy: MR diagnosis and relationship to multisystem atrophy.

Clinical diagnosis of olivopontocerebellar atrophy (OPCA) must be confirmed by radiologic demonstration of atrophy in an appropriate distribution. OPCA may be associated with degeneration of other systems in multisystem atrophy (MSA). The authors report 23 cases of OPCA, eight of which were associated with MSA. Atrophy involved the cerebellum, pons, and middle cerebellar peduncles in all cases. On intermediate and T2-weighted magnetic resonance (MR) images, abnormal signal intensity was always observed in the transverse pontine fibers, middle cerebellar peduncles, and cerebellum, structures known from pathologic study to degenerate in OPCA. Pyramidal tracts and superior cerebellar peduncles stood out because of their normal signal intensity. Of the eight patients with MSA, four also had variable abnormal signal intensities in the putamen. The authors believe that the combination of atrophy and abnormal signal intensity in the appropriate distribution strongly supports the diagnosis of OPCA. In some cases, MR imaging may demonstrate involvement of different systems, thus confirming the diagnosis of MSA.

Adult↗
PubMed1997

[A case of post-poliomyelitis muscular atrophy with cranial nerve signs and widespread muscular atrophy of the extremities].

Here we report a case of a 56-year-old male with post-poliomyelitis muscular atrophy (PPMA), who presented with cranial nerve signs and widespread atrophy of the extremities. He had suffered from poliomyelitis at the age of 2 years. After recovery from the acute stage, the paralysis remained in his left arm. He noticed muscle weakness of the right upper and lower extremities at the age of 45 years and the muscle atrophy progressed to his arms, hip and thigh at the age of 55 years. Neurological examination revealed muscle atrophy of the neck and disturbance of left V, VIII, IX, X and bilateral XI cranial nerves. We diagnosed this case as PPMA from his history and electromyographic and muscle biopsy findings which suggested chronic denervation. Among the 21 PPMA cases in the past in which the acute poliomyelitis had resulted in paralysis of the only one limb, ours was the only case that had muscle atrophy of all the limbs. Cranial nerve involvement is known to occur in acute poliomyelitis; therefore, there is a possibility that the involvement of the cranial nerves in our case might be a delayed progressive symptoms.

Cranial Nerves↗
PubMed2006

Cortical atrophy in the cerebellar variant of multiple system atrophy: a voxel-based morphometry study.

This study aimed to determine in vivo the atrophy patterns in clinically established cerebellar variant of multiple-system atrophy (MSA-C) using voxel-based morphometry (VBM). Thirteen patients with MSA-C (12 probable, 1 possible) and 13 healthy controls matched for age and sex were included. High-resolution MR images were acquired with a 1.5 T scanner. Images were normalized onto a study-specific template, segmented into the tissue compartments, modulated with the Jacobian determinants, and finally smoothed with a Gaussian kernel filter of 10 mm. The general linear model was used to assess statistical differences in gray and white matter. Infratentorial atrophy was observed in the cerebellar hemispheres, vermis, mesencephalon, and pons of MSA-C patients. Supratentorial volume loss was found in orbitofrontal and mid-frontal regions as well as in temporomesial and insular areas of both hemispheres. A negative correlation was observed between a cerebellar ataxia score and the volume of cerebellar hemispheres, peduncles, and pons. To compare this atrophy pattern to that of spinocerebellar ataxia (SCA2), which was previously reported by our group, a conjunction analysis was assessed. We observed a volume loss shared by both disorders comprising the cerebellum, vermis, pons, mesencephalon, orbitofrontal, mid-frontal, and temporomesial cortex of both hemispheres as well as the left insular cortex.

Adult↗
PubMed2000

Evolution of sporadic olivopontocerebellar atrophy into multiple system atrophy.

OBJECTIVE: To determine the percentage of sporadic olivopontocerebellar atrophy (sOPCA) patients who later develop multiple system atrophy (MSA). METHODS: Observations of the course of 51 sOPCA patients 20 years of age or older initially evaluated in an ataxia clinic over 14 years and followed at 3- to 6-month intervals for 3 months to 10 years (median 2.5 years, interquartile range 5 months to 4 years). RESULTS: Seventeen patients evolved to develop MSA, whereas the remaining 34 manifested only progressively worsening cerebellar ataxia. The features of the MSA cases included autonomic failure and parkinsonism in 10 patients, autonomic failure without parkinsonism in six, and parkinsonism without autonomic failure in one. Using survival analysis methods, the authors estimated that 24% of subjects in this population will evolve to MSA within 5 years of the onset of sOPCA symptoms (95% CI 10% to 36%). An older age at onset of symptoms and a shorter time from onset of symptoms to first presentation in a neurology specialty clinic were both highly predictive of evolution to MSA. Six of the 17 patients who evolved to MSA died 4 months to 5 years after they had met diagnostic criteria for MSA. The estimated median survival time from time of transition was 3.5 years. In contrast, death occurred in only one of the 34 patients with sOPCA who did not evolve to MSA. Autopsy examination of all six patients with MSA who died confirmed the diagnosis. CONCLUSIONS: Approximately one-fourth of sporadic olivopontocerebellar atrophy patients will evolve to multiple system atrophy within 5 years, and this transition carries a poor prognosis for survival. Older age at onset of ataxia and earlier presentation in a neurologic specialty clinic predicted transition to MSA.

Adult↗
PubMed1990

[An autopsied case of idiopathic late cortical cerebellar atrophy--comparison with other cortical cerebellar atrophy].

A 68-year-old man without familial history developed ataxic gait and sensory disturbance in the lower extremities. At the age of 74, neurological examination revealed marked cerebellar ataxia of all limbs, dysarthria, sensory disturbance of glove and stocking type in the extremities, and slight neurogenic muscular atrophy. There were no mental deterioration and dysautonomia. He died of pneumonia at the age of 74. Neuropathological findings. The cerebellum was decreased in size. Microscopically, there were severe disappearance of Purkinje cells in the cerebellar vermis and hemispheres. The molecular layer, granular cell layer, and cerebellar white matter were preserved. Neurons of the inferior olivary nuclei were also spared. In the spinal cord, there was myelin pallor in the posterior column predominant in Goll's fascicule and moderate atrophy of neurons in the anterior horn. Degeneration of the posterior roots was greater than that of the anterior roots. No abnormal findings were found in the extrapyramidal system, cranial nerves, and cerebrum. We compared this case clinicopathologically with other diseases with cortical cerebellar atrophy; alcoholic cerebellar degeneration, phenytoin intoxication, neuroleptic malignant syndrome, and subacute paraneoplastic cerebellar degeneration. In conclusion, idiopathic late cerebellar cortical atrophy (LCCA) was different clinicopathologically from the other diseases. Especially, LCCA showed the characteristic topography of Purkinje cells loss sparing the molecular and granule cell layers.

Aged↗
PubMed1994

[A case of facioscapulohumeral muscular atrophy presenting unusual squatting gait, associated with tongue atrophy and sensorineural hearing loss].

A 16-year-old girl has presented speaking disturbance since early in life. Difficulty in running was noted at the age of 7. Her mother (46 years old) had moderate facial weakness and mild proximal weakness of the upper and lower limbs. Neurological examination at age 9 revealed bilateral facial weakness, tongue atrophy and weakness of the shoulder girdle, upper arms, and thighs. Deep tendon reflexes were absent in the four limbs. Gait was waddling. Over the next several years, she developed wasting and weakness around the shoulder girdle, upper arms, thighs, and increased lordosis. She lost the ability to walk by age 13. Since then, she has moved with difficulty in a squatting position. Serum CK was moderately increased and needle EMG in the extremities revealed myopathic changes. Muscle CT demonstrated marked atrophy of the proximal muscles in the left arm and lower limbs and mild atrophy of the calves. Audiogram showed bilateral mild sensorineural hearing loss. Muscle biopsy of the quadriceps at age 9 showed nondiagnostic findings. This case could be diagnosed as congenital facioscapulohumeral muscular dystrophy. Tongue atrophy and sensorineural hearing loss are rarely associated with this syndrome, as well as unusual squatting gait.

Adolescent↗
PubMed1999

Multiple system atrophy with remarkable frontal lobe atrophy.

The autopsy findings of a multiple system atrophy (MSA) patient with remarkable frontal lobe atrophy are described. The patient was a 65-year-old woman with a 13-year history of untreatable parkinsonism, dysautonomia and progressive motor aphasia. The brain weight was 810 g, and there was remarkable atrophy of the cerebrum predominantly in the frontal lobe, striatum, pons and cerebellum. 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 second layer of the cortices of these regions were also revealed to be in a spongy state, and mild cell loss was seen in the fifth and six layers. The frontal lobe white matter showed a mild loss of myelinated fibers and axons, 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 was severe degeneration in the olivopontocerebellar and striatonigral systems, and GCIs in widespread regions of the brain. No Pick bodies, Lewy bodies, ballooned neurons, senile plaques, or significant amounts of neurofibrillary tangles were detected. 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↗
PubMed2002

Progressive cerebral atrophy in multiple system atrophy.

Nine patients with multiple system atrophy (MSA) were studied based on MRI findings of cerebral hemispheric involvement. The age at onset was 56.4+/-8.6 (mean+/-S.D.) years, duration of illness at the first MRI study 2.1+/-1.1 years, duration of illness at the last study 9.7+/-2.6 years, and the follow-up duration 7.6+/-2.3 years. Controls were 85 neurologically intact persons (60.2+/-11.1 years age). In the MRI study, measurements of the ratio of each area to the intracranial area were performed for the cerebral hemisphere, frontal, temporal and parietal-occipital lobes. A significant progression of atrophy to under the normal limit was observed in the cerebrum, frontal and temporal lobes. Besides the typical pathological lesions in MSA, five autopsied patients revealed frontal lobe atrophy with mild gliosis, mild demyelination and glial cytoplasmic inclusions (GCIs). One of these patients showed remarkable frontal lobe atrophy with degenerative changes in the cerebral cortex. We observed the involvement of the cerebral hemisphere, especially the frontal lobe.

Age of Onset↗
PubMed1979

Gyrate atrophy of the choroid and retina with hyperornithinemia: tubular aggregates and type 2 fiber atrophy in muscle.

We studied 21 patients with gyrate atrophy of the choroid and retina and hyperornithinemia. Although the patients were not weak, type 2 muscle fibers were almost universally atrophic and had tubular aggregates. Gyrate atrophy is the first disease in which females are shown to have tubular aggregates; the sexes were affected equally. In gyrate atrophy the number of type 2 fibers decreases with age. The muscle and eye changes are probably related to abnormal creatine synthesis, caused, in gyrate atrophy, by the increased body pool of ornithine; muscle abnormalities may also be present in other tapetoretinal dystrophies.

Adolescent↗
PubMed1995

Medial temporal lobe atrophy in an open population of very old persons: cognitive, brain atrophy, and sociomedical correlates.

Medial temporal lobe structures may be important for memory. We examined the cognitive, brain atrophy, and sociomedical correlates of medial temporal lobe atrophy (MTA) in 59 individuals (79.2 +/- 4.6 years old) randomly selected from a population-based study within strata of age and severity of clinically assessed DSM-III-R dementia (45 clinically normal and 14 mild/severely demented). MTA was qualitatively assessed on coronal T-1-weighted MRI. Thirty-three percent of the sample showed MTA, which was associated with dementia severity (p < 0.01), and cortical and white matter atrophy. MTA was not associated with age, education, sex, depressive symptoms, or presence of infarction. Controlling for age, education, and associated brain atrophy, those with MTA performed more poorly on a general test of cognitive function (the neuropsychological test component of the Cambridge Examination for Mental Disorders of the Elderly; p < 0.04) and its subtests of memory function (p < 0.02) and memory-related functions, including perception, fluency, and orientation (p < 0.05). In the clinically normal subsample, those with MTA performed more poorly on the memory function (p < 0.05) subtests. We conclude that MTA is common among very old persons, is associated with other brain abnormalities implicated in cognitive function, but may specifically contribute to memory dysfunction in the general population of very old persons.

Aged↗
PubMedDate not supplied

[Comparison between MRI and 3D-SSP in olivopontocerebellar atrophy and cortical cerebellar atrophy].

We compared images of three-dimensional stereotactic surface projections (3D-SSP) of SPECT with MRI images in spinocerebellar degeneration patients (13 olivopontocerebellar atrophy (OPCA) and 7 cortical cerebellar atrophy (CCA)). We analyzed a brain blood flow pattern with an image of statistics by 123I-IMP SPECT. In OPCA patients, a blood flow reduction was more remarkable in 3D-SSP than a degree of cerebellar atrophy in MRI. In patients with CCA, the cerebellum showed little blood flow reduction in 3D-SSP despite of apparent atrophy in MRI. Simultaneous examination both MRI and 3D-SSP might be useful for differential diagnosis of spinocerebellar degenerations.

Cerebellar Cortex↗
PubMed2005

[Cerebral atrophy and crossed cerebellar atrophy following acute subdural hematoma in infant].

Crossed cerebellar atrophy (CCA) on imaging study reflects secondary degeneration of the cerebellar hemisphere caused by neuronal disconnection with the contralateral cerebral hemisphere. We report an 11-month-old child with traumatic left acute subdural hematoma, who showed crossed cerebellar atrophy on MR images. Eighteen months after the trauma, MR images showed brain atrophy not only in the left cerebral hemisphere but also in the right cerebellar hemisphere. The cerebellar atrophy was prominent in the lateral part. This finding is consistent with CCA and may support neuronal interactions between the cerebral hemisphere and the lateral part of the contralateral cerebellar hemisphere.

Atrophy↗
PubMed1987

[An autopsy case of dentatorubropallidoluysian atrophy showing marked atrophy of the brain stem].

An autopsy case of a 66 year-old woman is reported. She developed personality change and psychotic symptoms at the age of 58. She began to show gait disturbance and forgetfullness at the age of 60. She was admitted to Okayama University Hospital at the age of 61, when she showed personality change, dementia, cerebellar sings and chorea like involuntary movement. The illness progressed slowly and she died of septicemia at the age of 66. At autopsy brain weighed 990 g. Macroscopically, the atrophy of the brain stem was severe, and the cerebellum was slightly atrophic. Microscopically, the globus pallidus was almost intact, but the degeneration involved dentate nuclei, their projections, red nucleus and the subthalamic nuclei, so this case was considered to be a case of pseudo-Huntington form of dentatorubropallidoluysian atrophy, proposed by Hirayama. The most striking feature of this case was marked atrophy of the brain stem and her intense familial history. Investigation of her familial history revealed that there were 18 affected cases in 5 successive generations. Their onset of the disease varied from the age of 10 to 60 years old. Cases of juvenile onset showed myoclonus and convulsion as the initial symptoms, and convulsion as the initial symptoms, and those of presenile onset showed dementia, cerebellar ataxia and chorea like involuntary movement. And in some of these cases it was proved by NMR-CT that their brain stem were small. We discussed the meaning of the atrophy of the brain stem in these cases and the difference of the symptoms between the cases of juvenile onset and the cases of presenile onset.

Age Factors↗
PubMed1983

[Total villous atrophy, mesenteric lymph-node cavitation, splenic atrophy. An unusual form of celiac disease in adults, apropos of a new case].

The authors report a new case concerning the syndrome "total villous atrophy, mesenteric lymph-node cavity formation and splenic atrophy" in a 41 year old woman. This pathologic association is characterized by: a) a clinical and biological malabsorption syndrome; b) proximal small bowel alterations as observed in coeliac disease, with, especially subtotal or total villous atrophy, abnormal enterocytic epithelium, hyperplastic crypts and lymph-plasmacytic infiltrate in the lamina propria; c) lymph node mesenteric cavity formation with an heterogeneous necrotic, sometimes liquefied, substance, without germ or parasite. Rare cortical lymphoid follicles are still persistent; 3) a splenic atrophy. A temporary improvement with gluten-diet (G.F.D.) was followed by a one-year period of total resistance. A treatment including corticotherapy was then successful and since the 14 past months her health remained satisfactory. Clinical, pathological data and the evolution about this patient are compared with those of the six published cases. The aetiology of this syndrome only observed in adults at the present time is unknown; the fact that patient's child presents with a coeliac disease, allows to authenticate, for the first time, this syndrome as a special form of adult coeliac disease.

Adult↗
PubMed1980

Studies on acid protease fractions and gastric mucosal atrophy. 2. Relationship of acid proteases in gastric juice separately determined by alkali treatment and gastric mucosal atrophy.

The relationships of two acid protease groups, alkali labile pepsin (ALP) and alkali stable pepsin (ASP), and gastric mucosal atrophy, were studied. ALP and ASP were determined separately by alkali treatment. The extent of fundic gland atrophy was detected by endoscopic congo red test. The subjects of the study were 10 normal subjects and 64 patients with gastroduodenal disease. In all subjects, both ALP and ASP outputs were increased by tetragastrin administration while ASP:ALP ratio was decreased by the provocation. ALP output was reduced in parallel with the extent of fundic gland atrophy whereas ASP output was kept until the atrophy became severe.

Adult↗
PubMed1989

Distal infantile spinal muscular atrophy associated with paralysis of the diaphragm: a variant of infantile spinal muscular atrophy.

We report the clinical, electrophysiological, and morphological observations of five infants with an unusual form of spinal muscular atrophy (SMA). In these infants muscular weakness and atrophy were initially restricted to the distal limbs and this pattern was associated with paralysis of the diaphragm. The difference between the clinical manifestations of this syndrome and the classical form of infantile spinal muscular atrophy (SMA type 1) as well as other congenital hereditary neuropathies is discussed.

Atrophy↗