There have been only few studies of brain magnetic resonance imaging (MRI) in spinocerebellar ataxia (SCA) type 2. We investigated 20 SCA2 patients, from 11 Sicilian families, and 20 age-matched control subjects using MRI. Our data confirm that olivopontocerebellar atrophy (OPCA) is the typical pattern in SCA2. We found no ...

A 6-year-old neutered female Boston Terrier had a slow onset of blindness and behavior changes. Neurologic abnormalities included bilateral visual loss with absent menace responses and visual tracking, mydriatic pupils, slow pupillary light responses and papilledema. On magnetic resonance imaging (MRI) there were multiple cyst-like structures found in the parenchyma ...

Molecular genetic assessments of 69 individuals in 44 families with hereditary cerebellar ataxia (HCA) were made to determine the relative frequencies of subtypes of HCA in Yamagata, Japan. Fifteen families (34%) had SCA1, none had SCA2, nine (20%) had MJD, five (11%) had SCA6 and nine (20%) had DRPLA. These ...

We report on a 3-year-old girl with respiratory syncytial virus (RSV) encephalitis manifested by disturbance of consciousness, conjugate eye deviation, anuria, truncal ataxia and intention tremor. T2-weighted magnetic resonance imaging (MRI) showed hyperintense areas in the cerebellar cortex. No lesion was detected in the cerebral cortex, pons or spinal cord. ...

The MRI findings in rostral vermian dysplasia are described for the first time. Defective foliation and abnormal fissuration of the rostral vermis can clearly be depicted on coronal images. The abnormalities are limited to the anterior lobe of the vermis and its hemisphere extension. A hypothesis is put forward to ...

The neuro-anatomical substrate of autism has been the subject of detailed investigation. Because previous studies have not demonstrated consistent and specific neuro-imaging findings in autism and most such studies have been performed in adults and school-aged children, we performed a retrospective review in young children in search of common functional ...

We report a patient with rhinocerebral mucormycosis whose initial central nervous system involvement was isolated pontine infarction due to basilar arteritis caused by the fungus. The patient was diagnosed and followed by MRI and CT and basilar arteritis was demonstrated well on MRI studies. Involvement of the skull base was ...

Central pontine myelinolysis (CPM) was initially associated with alcoholism. Subsequently other factors, including rapid reversal of hyponatraemia and extreme serum hypoosmolality associated with severe burns, have been identified as other important factors in its pathogenesis. Extra-pontine lesions have also been described. CPM may be found at autopsy, either having been ...

A 61-year-old woman had a 3-year history of imbalance. Eye movement studies revealed square-wave jerks, gaze paretic nystagmus, rebound nystagmus, impaired smooth pursuit, impaired optokinetic nystagmus, and abnormal fixation suppression of vestibular nystagmus. A brain magnetic resonance imaging study showed extensive areas of increased signal from the middle cerebellar peduncles ...

The authors report a 3-year-old male with glutaric aciduria type II, whose magnetic resonance imaging studies revealed agenesis of the cerebellar vermis and hypoplastic temporal lobes. Proton magnetic resonance spectroscopy in the parietal white matter revealed a markedly increased choline/creatine ratio, suggesting a demyelinating process. Gas chromatographic analysis of urinary ...

The authors investigated the feasibility of using T2-weighted, half-Fourier rapid acquisition with relaxation enhancement, or RARE, dynamic susceptibility contrast magnetic resonance (MR) imaging to depict crossed cerebellar diaschisis. In 10 patients after unilateral supratentorial stroke, crossed cerebellar diaschisis was demonstrated in the relative regional cerebellar blood volume maps obtained with ...

Surgical exposure of internal acoustic meatus via typical suboccipital retrosigmoid craniotomy is limited by inner ear structures that should remain intact if hearing preservation is attempted. Feasibility of supracerebellar-infratentorial approach to the meatus with more medial angle of exposure and with preservation of inner ear structures was studied on fresh ...

We encountered a 5-year-old girl with acute onset of alternating, comitant esotropia in the absence of diplopia and other neurologic findings. She did not have any refractive error and seen bilateral papilledemas in fundus examination, magnetic resonance imaging (MRI) of the head was performed. A large cerebellar astrocytoma and moderate ...

Autism is a neuropsychiatric disorder of social, cognitive, and language development. Cerebellar abnormality in autism has been shown consistently from autopsy and magnetic resonance image (MRI) studies. A new MRI study with careful methodologic designs identified two subgroups of autistic patients: hypoplasia and hyperplasia of cerebellar vermian lobules VI-VII. The ...

As isolated symptoms, vertigo, dizziness and imbalance are not regarded by neurologists as reflections of transient ischemia in the vertebrobasilar circulation. The purpose of this retrospective study was to demonstrate that these symptoms can and do occur in isolation. To this end, we analyzed the symptoms, stroke risk factors and ...

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 ...

We describe a patient showing an atypical phenotype of Huntington's disease (HD), including prominent generalized dystonia, peripheral amyotrophy of the legs with an inverted champagne bottle configuration and pes equinus. The patient also had congenital defects of the lower left leg. Chorea and psychiatric symptoms were not prominent. Polymerase chain ...

The authors observed a periodic downbeat nystagmus with a cycle of 3 minutes 30 seconds, beating downward for a period of 90 seconds every 2 minutes. It lasted 86 hours in a patient with severe hypomagnesemia associated with complications from scleroderma. There was no lesion on brain imaging, and an ...

Central pontine myelinolysis (CPM) is an uncommon complication in sick patients with severe underlying disorders such as chronic alcoholism, malignancy, malnutrition and hyponatraemia. We report two patients with advanced HIV infection who developed CPM. In one case the diagnosis was not suspected in life, in the other the diagnosis was ...

The underlying lesion in congenital saccade initiation failure (c-SIF) ('congenital ocular motor apraxia', 'Cogan's apraxia') is uncertain. Often no abnormality can be found, yet in others a midline cerebellar abnormality has often been reported. We examined this cerebellar association in a brother and sister. In addition to standard ophthalmological and ...

Power spectrum analysis of auditory brainstem responses (ABR) and magnetic resonance imaging (MRI) findings were obtained from 14 patients, 9 with olivopontocerebellar atrophy (OPCA; Dejerine-Thomas type) and 5 with Holmes-type cerebello-olivary degeneration, and compared to data from normal controls. The power spectrum was divided into three components according to frequency: ...

OBJECTIVE: To clarify the characteristic magnetic resonance imaging (MRI) findings in patients with spinocerebellar ataxia 6 (SCA6) diagnosed by genetic analysis. PATIENTS AND METHODS: Using MRI, we examined 10 patients genetically diagnosed as having SCA6 and 40 control subjects. RESULTS: The mean (+/-SD) CAG repeat length in 10 patients with ...

A few patients with ataxia telangiectasia survive into the 3rd decade. In the central nervous system, dilated meningeal veins have been noted in a few cases but as a rule the vasculature in both brain and spinal cord appears normal. We present the case of the longest reported surviving patient ...

Three-hundred pediatric patients were evaluated for pontine signal abnormalities with special reference to small nodular, central pontine lesions. The ages of the patients ranged from 1 year to 17 years (mean = 7.3 years). There were 157 males and 143 females. The magnetic resonance (MR) imaging examinations were performed at ...

We analysed six patients who showed spontaneous improvement among the 19 nonsurgically treated cases of syringomyelia with low-situated cerebellar tonsils who visited our outpatient clinic. MR images both before and after improvement were available for two of these patients; CT-myelography before improvement and MR images after improvement were used for ...

Cerebellar agenesis is a rarely observed malformation that is frequently associated with other defects. We describe a neonate with an isolated cerebellar agenesis. In addition to the absence of recognizable cerebellar tissue, cranial magnetic resonance imaging demonstrated a hypoplastic base of the pons and absence of the normal outline of ...

MRI in a 17-year-old boy with known congenital agammaglobulinaemia (CA) demonstrated signs of chronic leptomeningeal inflammation with thickened, enhancing meninges. Furthermore, high signal was found symmetrically on T2-weighted images in the frontal and parietal white matter. The patient presented with severe general brain dysfunction and recent cerebellar ataxia. Extensive investigation ...

A 52-year-old man presented with sudden onset of severe headache, fever, mental confusion and mild signs of left hemisphere dysfunction. Computed tomographic and magnetic resonance imaging findings suggested a preoperative diagnosis of subependymoma of the left lateral ventricle, which was subsequently confirmed by surgery. The neoplasm could be totally removed ...

PURPOSE: To determine the frequency and patterns of periictal cerebellar hyperperfusion, whether it is associated with increased cerebellar atrophy, and whether cerebellar hyperperfusion and cerebellar atrophy are associated with predisposing clinical factors or with the outcome of epilepsy surgery. METHODS: Periictal and interictal SPECT scans and volumetric brain magnetic resonance ...

A 45-year-old woman developed a rare case of palatal myoclonus with no other neurological signs after undergoing extirpation of a small cerebellar low-grade astrocytoma that was located in the right dentate nucleus. The palatal myoclonus has persisted for 4 years after the operation. Magnetic resonance T2-weighted imaging revealed a high-intensity ...

MR imaging findings are described in four patients with cerebellar cortical dysplasia. Typically, cerebellar disorganized folia were seen as an irregular bumpy gray-white matter interface. In addition, cystlike cortical abnormalities were observed in two patients and associated supratentorial developmental abnormalities were seen in three patients. To our knowledge, cerebellar cortical ...

We report on seven Japanese families with spinocerebellar ataxia type 6 (SCA6) carrying small CAG repeat expansions in the calcium channel alpha1A subunit gene. The number of the expanded CAG repeat, ranged from 22 to 25, showed no intergenerational instability and had a significant inverse correlation with the age of ...

We describe a 25 year old woman diagnosed with MELAS during an acute stroke-like episode. Global aphasia, migraine-like headaches and hemi-anopsia were her main clinical features. MR imaging revealed extensive cortical and subcortical left hemispheric signal abnormalities. [Tc-99m]ECD SPECT scanning revealed crossed cerebrocerebellar diaschisis. Aphasia in the absence of gross ...

OBJECTIVE: This study examined the collective sensitivities of decruitment, hyperactivity, and rebound caloric nystagmus (RCN) for lesions of the brain stem/cerebellum against the current gold standard of imaging, the contrast enhanced magnetic resonance imaging (MRI). Study Design: This is a retrospective study of patients who underwent a vestibular evaluation and ...

We describe two infants in whom rhombencephalosynapsis was diagnosed with MR imaging in vivo. In contrast to Dandy-Walker malformation, the vermian maldevelopment in this anomaly is characterized by an absence of the anterior vermis and a deficiency of the posterior vermis. The cerebellar hemispheres are fused. In an attempt to ...

Cerebellitis, also known as acute cerebellar ataxia, is an inflammatory syndrome of cerebellar dysfunction that may reflect an infectious, post-infectious, or post-vaccination disorder. We present serial magnetic resonance imaging (MRI) findings in a partially reversible, idiopathic cerebellitis. Bilateral cerebellar parenchymal abnormalities were noted, including hyperintensities on T2-weighted images and cerebellar ...

A 14-year-old boy presented acutely with occipital headache, nausea and vomiting. MRI showed obstructive hydrocephalus and marked bilateral cerebellar swelling with increased signal on T2-weighted imaging. Following treatment with oral corticosteroids, the clinical and radiological signs resolved. The clinical course and radiological appearances were consistent with cerebellitis associated with a ...

We describe MR findings in patients with Minamata disease who have been followed for a long time. All patients examined were affected after daily eating of a large quantity of methylmercury-contaminated seafood, from 1955 to 1958, and showed typical neurological findings. On MR images, the visual cortex, the cerebellar vermis ...

Cerebellar hypoplasia is a prominent feature of fetal brain pathology. The lesion is rarely isolated or asymmetric. Various aetiological factors have been proposed. The frequency of cerebellar hypoplasia as a congenital defect in humans is unknown. A single case is described of unilateral cerebellar hypoplasia with intact vermis that was ...

PURPOSE: Our purpose was to determine the neuroradiologic findings of Marinesco-Sjögren syndrome on plain skull radiographs, CT, and MR images. METHODS: Eight patients with proved Marinesco-Sjögren syndrome (age range, 4 to 56 years) had a total of nine CT scans, seven MR imaging studies, and two plain radiographic examinations of ...

Linkage and DNA analysis, magnetic resonance (MR) imaging, and single-voxel proton MR spectroscopy were obtained in 10 members of an Italian kindred with spinocerebellar ataxia type 1 (SCA1). The size of the basis pontis, cerebellar hemispheres, middle cerebellar peduncles, and medulla oblongata were decreased in 4 members carrying the SCA1 ...

A patient is presented who had bilateral abductor vocal fold paralysis pathologically proven to be due to multiple system atrophy (MSA) in the absence of other neurological features. MSA is a degenerative neurological condition that includes olivopontocerebellar atrophy, Shy-Drager syndrome and striatonigral degeneration. The usual predominant features of MSA are ...

Micturitional disturbance is rarely mentioned in human herpetic brainstem encephalitis although the pontine tegmentum, called the pontine micturition centre, seems to regulate the lower urinary tract in experimental animals. The case of a 45 year old man, who developed subacute coma and hiccup-like dysrhythmic breathing, and needed assisted ventilation is ...

We report a 63 year-old woman who developed central pontine and extrapontine myelinolysis after rapid correction of hyponatremia. Lesions on brain MRI showed hypermetabolism on 18FDG-PET scan in the early stage of the disease and became hypometabolic on the follow-up scan. We suggest that active microglia and astrocytes are the ...

A 30-year-old woman hit her head during an automobile accident and was admitted to the hospital. One week later magnetic resonance imaging (MRI) showed a right frontal/parietal lesion. Among the behavioral sequelae were mild ataxia with trunkal instability and dysfluent speech accompanied by prominent shaking of the right leg, face ...

This is a report of clinical, CT and MRI findings in a patient with toxic spongiform leucoencephalopathy after heroin ingestion. The disease is observed in drug addicts who inhale pre-heated heroin. The clinical onset, which usually occurs some days or even longer after the last heroin consumption, is characterized by ...

Bilateral anterior inferior cerebellar artery (AICA) territory infarcts are rare. Their occurrence usually signifies severe intracranial vertebrobasilar disease. Unlike head computed tomography, magnetic resonance (MR) imaging reveals these infarcts clearly and MR angiography allows the intracranial vasculature to be defined noninvasively. We now report a patient with bilateral AICA territory ...

Cerebellar involvement in tuberous sclerosis is apparently less than cerebral involvement. In this article, we report CT and MR imaging findings in four tuberous sclerosis patients with apparent cerebellar changes. In one patient with extensive folial calcifications, hypoplasia of the superior vermis, and agenesis of the inferior vermis were associated ...

We report two cases of glossopharyngeal neuralgia associated with a vascular loop of the postero-inferior cerebellar artery, diagnosed by magnetic resonance imaging. Reviewing the literature, we found this to be the first report of a magnetic resonance-validated vascular abnormality related to such a condition. One patient was cured by surgical ...

A 33-year-old woman presented with a 3-year history of progressive numbness in the hand, cerebellar ataxia, limb weakness, nystagmus, and dysarthria. T2-weighted MRI revealed abnormal foci of increased signal intensity mimicking demyelinating plaques in the periventricular white matter, and brain 18FDG-PET scan showed increased uptake in the pons. Biopsy from ...