11/95. Crossed cerebellar diaschisis secondary to refractory frontal seizures in childhood.We report a girl with refractory partial seizures since 7 years of age, secondary to right frontal cortical dysplasia, who developed MRI and SPECT abnormalities in the contralateral hemicerebellar cortex. These became more marked, leading to left hemicerebellar atrophy. Crossed cerebellar diaschisis has been described mostly in hemispheric stroke and supratentorial tumours, but less often in epilepsy. It is usually a transient phenomenon. This report shows that crossed cerebellar diaschisis can develop within two years of seizure onset and evolve over time.- - - - - - - - - - ranking = 1keywords = atrophy (Clic here for more details about this article) |
12/95. Secondary cervical dystonia associated with structural lesions of the central nervous system.We tested the hypothesis that structural lesions of the central nervous system (CNS) associated with cervical dystonia more commonly involve the cerebellum and its primary afferent pathways than basal ganglia structures. Cervical dystonia is the most common focal dystonia, the majority of cases are idiopathic, and only a small percentage of patients have a family history of dystonia or other movement disorders. Pathophysiological mechanisms operative in solely or predominantly appendicular dystonias such as writer's cramp and Oppenheim's dystonia, respectively, may not be directly applicable to axial dystonias. The localization of structural lesions of the CNS associated with secondary cervical dystonia may provide some insight into the neural structures potentially involved in primary cervical dystonia. The National Library of medicine Gateway (from 1960) and a clinical database maintained by the senior author (from 1999) were searched for cases of secondary cervical dystonia associated with structural lesions of the CNS. Search terms included one or more of the following: dystonia, torticollis, cervical, secondary, and symptomatic. Lesion localization and type, patient age, patient gender, head position, occurrence of sensory tricks, and associated neurological findings were tabulated for each case. Structural lesions associated with cervical dystonia were most commonly localized to the brainstem and cerebellum. The remaining cases were equally divided between the cervical spinal cord and basal ganglia. Although inconsistent, head rotation tended to be contralateral to lesion localization. Additional neurological abnormalities were present in the majority of patients with secondary cervical dystonia. The relative paucity of basal ganglia pathology and concentration of lesions in the brainstem, cerebellum, and cervical spinal cord in patients with secondary cervical dystonia suggests that dysfunction of cerebellar afferent pathways may be important to the pathophysiology of primary cervical dystonia.- - - - - - - - - - ranking = 0.013543623513122keywords = idiopathic (Clic here for more details about this article) |
13/95. Idiopathic scoliosis as a presenting sign of familial neurologic abnormalities.STUDY DESIGN: Familial cases of "idiopathic" scoliosis associated with neurologic abnormalities are reported with a review of the literature. OBJECTIVE: To investigate the prevalence of neurologic abnormalities such as syringomyelia, Chiari 1 malformation, and tonsillar ectopia in patients with genetically determined "idiopathic" scoliosis. SUMMARY OF BACKGROUND DATA: Idiopathic scoliosis is widely considered to be a genetic disorder of unknown etiology. magnetic resonance imaging (MRI) studies have shown that several cases of "idiopathic" scoliosis show neurologic abnormalities including syringomyelia and Chiari 1 malformation. Recently, several familial cases of either syringomyelia or Chiari malformation were reported, and it is suspected that genetic factors may influence the development of the craniovertebral malformation. It was hypothesized that some cases of "idiopathic" scoliosis include a craniovertebral malformation that is genetically determined. methods: This study, using clinical examinations and MRI, investigated 71 patients with scoliosis and a family history of "idiopathic" scoliosis in third-degree relatives for the presence of neurologic abnormalities. If neurologic abnormalities were confirmed with MRI, the relatives affected with scoliosis were also examined. RESULTS: Nine (13%) patients showed neurologic abnormalities on MRI. magnetic resonance imaging showed syringomyelia with Chiari 1 malformation in four patients, Chiari 1 malformation in three patients, and tonsillar ectopia in two patients. Among the relatives of these patients, 4 of 15 individuals affected with scoliosis also showed neurologic abnormalities on MRI. CONCLUSIONS: It is suggested that familial neurologic abnormalities may have a wide range of expression, and that some patients with "idiopathic" scoliosis present with genetically determined craniovertebral malformations such as syringomyelia, Chiari 1 malformation, and tonsillar ectopia.- - - - - - - - - - ranking = 0.081261741078731keywords = idiopathic (Clic here for more details about this article) |
14/95. Stasis of 111In-DTPA in the posterior fossa in patients with cerebellar degeneration.Prolonged stasis of 111In-DTPA was noted in the poterior fossa in two patients during cisternography. Both patients had clinical signs of cerebellar dysfunction and pneumoencephalographic evidence of marked cerebellar degeneration. Cisternography may be a useful adjunct in evaluation patients with suspected cerebellar atrophy.- - - - - - - - - - ranking = 1keywords = atrophy (Clic here for more details about this article) |
15/95. Madras motor neuron disease variant, clinical features of seven patients.Madras motor neuron disease (MMND) has the characteristic features of onset in the young, atrophy and weakness of the limbs, multiple cranial nerve palsies particularly the seventh, ninth to twelfth and sensorineural hearing loss with unique geographic distribution to southern part of india. During a period of 28 years (1974-2001), 7 (13%) among 54 patients of MMND seen at a tertiary referral center at Bangalore, india, had the additional features of optic atrophy in all and cerebellar involvement in three of them. There were three males and four females, the mean age at onset was 11.7 years, with a mean duration of illness of 6.4 years. All except one patient were ambulant and independent in the activities of daily living. family history of MMND was present in more than a quarter (28.6%) of patients. Compared to MMND, these patients had onset of illness at a younger age and family history was more frequently observed, however, these differences were not statistically significant. Bulbar palsy was an invariable feature, being present in all patients compared to 38.3% of MMND and the difference was statistically significant (p=0.003). This clinical profile may be considered to be a variant of Madras motor neuron disease (MMNDV).- - - - - - - - - - ranking = 2keywords = atrophy (Clic here for more details about this article) |
16/95. Cerebellitis in an adult with abnormal magnetic resonance imaging findings prior to the onset of ataxia.BACKGROUND: brain magnetic resonance imaging (MRI) findings during acute cerebellar ataxia in cases of postinfectious cerebellitis are frequently normal. This has resulted in the use of other imaging modalities, such as single-photon emission computed tomography, to aid diagnosis. OBJECTIVE: To illustrate the chronologic occurrence of cerebellar ataxia, abnormal findings on MRI, and cerebral spinal fluid pleocytosis in an adult case of postinfectious cerebellitis. methods: Case report. RESULTS: A patient with a 6-week history of occipital headaches and only mild tandem gait difficulty had abnormal MRI findings that were consistent with cerebellar inflammation. As cerebellar ataxia progressed in parallel with cerebral spinal fluid pleocytosis, MRI findings indicative of cerebellar inflammation resolved, while single-photon emission computed tomography showed cerebellar hyperperfusion. Recovery of neurologic function was accompanied by clearing of the pleocytosis and residual MRI-detected cerebellar atrophy. CONCLUSION: This case demonstrates that transient abnormalities can be detected by MRI before clinical manifestations of cerebellitis appear, while hyperperfusion detected by single-photon emission computed tomography is prolonged.- - - - - - - - - - ranking = 1keywords = atrophy (Clic here for more details about this article) |
17/95. Bathing headache: a variant of idiopathic thunderclap headache.Bathing headache is rarely described in literature. We report four middle-aged Taiwanese women who developed severe throbbing headache with maximum intensity of onset during bathing. Diffuse cerebral vasospasm was demonstrated in one of them. All their headaches resolved spontaneously (n = 1) or after nimodipine treatment (n = 3). Except for one patient with vasospasm in whom reversible posterior leukoencephalopathy and an asymptomatic cerebellar infarction developed, the others recovered without any complications. The clinical profile of bathing headache points to idiopathic thunderclap headache. It may not be as benign as previously reported. nimodipine might be effective in treatment of this special headache syndrome.- - - - - - - - - - ranking = 0.067718117565609keywords = idiopathic (Clic here for more details about this article) |
18/95. Transient cerebellar mutism in the course of acute cerebellitis.Transient mutism after posterior fossa surgery in children or associated with cerebellar hemorrhage or trauma is a recognized phenomenon. However, its association with parainflammatory cerebellitis has been rarely described. We report on a previously healthy 3-year-old child with severe cerebellitis after acute gastroenteritis of unidentified cause. Severe ataxia and transient mutism were the prevailing clinical features. magnetic resonance imaging revealed swelling of the cerebellum with protruding cerebellar tonsils at the level of the occipital foramen. Recovery from the acute illness was slow and incomplete. Residual cerebellar dysfunction manifested with dysphonic and dysarthric speech, as well as motor coordination problems and was associated with atrophy of the vermis and cerebellar hemispheres in follow-up studies.- - - - - - - - - - ranking = 1keywords = atrophy (Clic here for more details about this article) |
19/95. MRI findings in acute cerebellitis.Acute cerebellitis is an inflammatory process involving the cerebellum. We report the clinical, CT and MRI features of four cases and a review of the literature. Bilateral diffuse hemispheric abnormalities represent the most common imaging presentations. Our observations demonstrate the various imaging appearances of acute cerebellitis. Simultaneous involvement of both hemispheres and the vermis has not been reported previously. The development of cerebellar atrophy following an initial normal MR imaging examination is also a new finding. In atypical clinical presentation, MR imaging can lead to the diagnosis. MR imaging findings have, however, no prognostic value.- - - - - - - - - - ranking = 1keywords = atrophy (Clic here for more details about this article) |
20/95. Infectious acute hemicerebellitis.We report the case of a 5-year-old girl with initial symptoms of encephalitis who presented 24 hours later with hemiataxia, unilateral dysmetria, and hemiparesis. brain magnetic resonance image (MRI) revealed a high T2-weighted signal in the ipsilateral hemicerebellar cortex. Forty-five days later, a second MRI disclosed signs of hemiatrophy and cortical gliosis. The clinical outcome was favorable, with only a slight lack of motor coordination in the involved hand remaining. Three other cases of hemicerebellitis have been reported in the literature, two of them presenting with hemicerebellar symptoms and one mimicking a tumor. Follow-up imaging studies some months later have shown hemiatrophy of the cerebellar cortex, except in one case with a normal control MRI. The pathophysiology of this unilateral involvement is difficult to explain. We underline the need to consider this rare entity in asymmetric cerebellar clinical presentations and to perform MRI rather than computed tomography to reach the correct diagnosis.- - - - - - - - - - ranking = 2keywords = atrophy (Clic here for more details about this article) |
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