1/10. Cerebellar hypoplasia-endosteal sclerosis: a long term follow-up.Cerebellar hypoplasia with endosteal sclerosis is an infrequent entity that has been described in only four cases. Major clinical symptoms are cerebellar hypoplasia causing ataxia, hypotonia, mild to moderate developmental delay, microcephaly, growth retardation, endosteal sclerosis, tooth eruption disturbances, and hip dislocations. We report on a girl with this entity, whom we followed for 11 years. The endosteal sclerosis remained stationary over time, as were the clinical neurological symptoms, but neuroadiological symptoms were slowly progressive. We provide a short review of this probably autosomal recessively inherited disorder. (c) 2005 Wiley-Liss, Inc.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/10. Gluten sensitivity in Japanese patients with adult-onset cerebellar ataxia.OBJECTIVE: Gluten sensitivity is associated with multiple neurological abnormalities including gluten ataxia, motor neuron disease-like neuropathy, small fiber type neuropathy, cognitive impairment, and even parkinsonism. We investigated whether or not gluten sensitivity is involved in Japanese patients with idiopathic cerebellar ataxia with extracerebellar presentation. patients OR MATERIALS: Fourteen patients with idiopathic cerebellar ataxia with extracerebellar presentation (autonomic instability, parkinsonism, or pyramidal dysfunction in varying combinations) were screened for anti-gliadin antibodies (AGA) to analyze for the presence or absence of gluten sensitivity. patients with typical MR findings of multiple system atrophy of the cerebellar type were excluded. As disease controls without cerebellar ataxia, 9 patients with Parkinson's disease and 18 patients with amyotrophic lateral sclerosis were screened for AGA. Forty-seven normal controls were also screened for AGA. RESULTS: We found a high prevalence of AGA in 5 (36%) of 14 cerebellar ataxia patients, but in only 1 (4%) of 27 disease controls without cerebellar ataxia (odds ratio, 14.4; 95% CI, 1.41147; p<0.05) and in only 1 (2%) of 47 normal controls (odds ratio, 25.6; 95% CI, 2.66246; p<0.001). Among the cerebellar ataxia patients, atypical features such as sensorimotor neuropathy and/or mild cognitive impairment were more prevalent in the AGA-positive group (60%) than in the AGA-negative group (0%). In one of the ataxic patients with AGA, a gluten-free diet had positive effects on neurological symptoms and nutritional status. CONCLUSION: Gluten sensitivity is involved in at least some of the unexplained neurological symptoms of Japanese patients with adult-onset, sporadic cerebellar ataxia.- - - - - - - - - - ranking = 0.14285714285714keywords = sclerosis (Clic here for more details about this article) |
3/10. The clinical, pathological and genetic aspects of sporadic late onset cerebellar ataxia: observations on a series of ten patients.Ten patients with sporadic late onset cerebellar ataxia (LOCA) are described. The mean age of onset was 50.4 /- 7.13 years. The important clinical features were gait ataxia, poor coordination of hands, intention tremors, exaggerated deep tendon reflexes, extrapyramidal symptoms and extensor plantar responses. Computerised tomography (CT) scanning in one patient showed a low density mid-line lesion, suggesting early cerebellar atrophy. Histopathological examination in one patient, clinically diagnosed as multiple sclerosis, revealed complete loss of purkinje cells from the cerebellar folia with gliosis in the molecular layer and loss of small granular neurones. A marked loss of the neurones from the olivary nuclei with astrocytic proliferation was also seen. The disorder is probably genetically determined although a single Mendelian inheritance is unlikely in the absence of recurrence in the first degree relatives. recurrence risks for gentic counselling are suggested.- - - - - - - - - - ranking = 1.3176066450739keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
4/10. Neuro-ophthalmologic findings in vestibulocerebellar ataxia.A young adult who presents with periodic vertigo, diplopia, and tinnitus, later followed by progressive ataxia, may not have multiple sclerosis as might initially be suspected, but rather may represent a type of familial spinocerebellar degeneration previously described by Farmer and Mustian as "vestibulocerebellar ataxia." We recently encountered a patient who presented with these symptoms, and who was found to have downbeat nystagmus, ocular dysmetria, skew deviation, optokinetic dissociation, and a vertical gain bias on horizontal eye movements. Although family involvement was specifically denied by the patient, seven members of her family representing four generations were examined, and found to have similar findings. The importance of a careful neuro-ophthalmologic examination in each available family member of any patient presenting with this constellation of symptoms is emphasized. A discussion of the spinocerebellar degenerations, differential diagnosis, and literature review are included.- - - - - - - - - - ranking = 1.3176066450739keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
5/10. Enzymatic analysis of individual posterior root ganglion cells in olivopontocerebellar atrophy, amyotrophic lateral sclerosis and Duchenne muscular dystrophy.Four enzyme activities related to glucose metabolism, i.e. those of glucose-6-phosphate dehydrogenase (G6PDH; EC 1.1.1.49), lactic dehydrogenase (LDH; EC 1.1.1.27), pyruvate dehydrogenase complex (PDC) and citrate synthase (CS; EC 4.1.3.7) were estimated in posterior root ganglion cells (PRGCs) of the spinal cord in patients suffering from olivopontocerebellar atrophy (OPCA), amyotrophic lateral sclerosis (ALS), and Duchenne muscular dystrophy (DMD) by means of the NAD, nadp and CoA cycling methods. In ALS and DMD, the enzyme activities examined were within normal ranges. In OPCA, PDC activity was significantly reduced and LDH activity tended to be lower than that in controls.- - - - - - - - - - ranking = 0.71428571428571keywords = sclerosis (Clic here for more details about this article) |
6/10. Acute cerebellar ataxia of childhood and optic neuritis with retrochiasmal involvement.A case of a 4-year-old white girl affected with unilateral optic neuritis and acute cerebellar ataxia is presented. Visual evoked potentials (VEP) demonstrated prolonged latency of the right optic nerve as well as the left retrochiasmal pathways. This child had complete resolution of the cerebellar ataxia and the optic neuritis with return to normal of her VEP. This case is an example of a disease characterized by a single episode of multifocal involvement of the CNS differing from diffuse encephalomyelitis and multiple sclerosis.- - - - - - - - - - ranking = 1.3176066450739keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
7/10. magnetic resonance imaging in a multiple sclerosis patient with progressive ataxia.A 23-year-old female developed progressive cerebellar ataxia as the only presentation of multiple sclerosis for 6 years. Abnormal evoked potentials, an increased oligoclonal band of IgG-K in the cerebrospinal fluid and disseminated white matter lesions in magnetic resonance images (MRI) were compatible with a diagnosis of clinically definite multiple sclerosis. After cyclosporine treatment, the ataxia became stable without further deterioration. A follow-up MRI six months later did not reveal any active lesions. The importance of MRI in the confirmation of the clinical diagnosis and the evaluation of therapeutic effects in patients with atypical multiple sclerosis is emphasized.- - - - - - - - - - ranking = 9.2232465155176keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
8/10. An immunologic abnormality common to Bickerstaff's brain stem encephalitis and Fisher's syndrome.The nosological position of Bickerstaff's brain stem encephalitis (BBE) has yet to be established, and its etiology is not clear. Because anti-GQ1b antibody frequently occurs in patients with Fisher's syndrome (FS) and there are clinical similarities between FS and BBE, we investigated anti-ganglioside antibodies in sera from 3 BBE patients who had transient long tract signs in addition to acute ophthalmoplegia and cerebellar-like ataxia in order to clarify the etiology and nosological position of BBE. High IgG anti-GQ1b antibody titers were present in all 3 sera samples but decreased with the clinical course of the illness. In contrast, no anti-GQ1b antibody was found in sera from patients with other neurologic diseases which were able to produce transient brain stem disturbance: multiple sclerosis, neuro-Behcet's disease, brain stem infarction, herpes simplex virus encephalitis, and Wernicke's encephalopathy. The finding that BBE and FS shared common autoantibody suggests that autoimmune mechanism common to FS is likely in BBE, and that both conditions represent a distinct disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia.- - - - - - - - - - ranking = 1.3176066450739keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
9/10. Effect of weak electromagnetic fields on body image perception in patients with multiple sclerosis.cerebellar ataxia is one of the most disabling symptoms of multiple sclerosis (MS) and also one of the least responsive to pharmacotherapy. However, cerebellar symptoms often improve dramatically in MS patients by brief, extracerebral applications of picotesla flux electromagnetic fields (EMFs). This report concerns two MS patients with chronic disabling ataxia who experienced rapid improvement in gait and balance after receiving a series of treatments with EMFs. To assess whether improvement in cerebellar gait is accompanied by changes in body image perception, a parietal lobe function, both patients were administered the Human Figure Drawing Test before and after a series of brief treatments with EMFs. Prior to application of EMFs these patients' free drawings of a person showed a figure with a wide-based stance characteristic of cerebellar ataxia. After receiving a series of EMFs treatments both patients demonstrated a change in body image perception with the drawings of the human figure showing a normal stance. These findings demonstrate that in MS improvement in cerebellar symptoms by pulsed applications of picotesla EMFs is associated with changes in the body image.- - - - - - - - - - ranking = 6.5880332253697keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
10/10. Hereditary adult-onset Alexander's disease with palatal myoclonus, spastic paraparesis, and cerebellar ataxia.We describe a progressive neurologic disorder in three sisters characterized clinically by palatal myoclonus, spastic weakness, hyperreflexia, mild cerebellar dysfunction, and ocular motor abnormalities. Postmortem examination of one patient demonstrated widespread Rosenthal fiber deposition associated with demyelination. The father previously was reported to have similar pathologic findings and carried a clinical diagnosis of multiple sclerosis. These clinical and pathologic findings describe a rare familial leukodystrophy that corresponds most closely to cases reported as adult Alexander's disease. Although similar pathologically to the well-characterized infantile variant of Alexander's disease, it is not known whether this adult variant represents the same disease process.- - - - - - - - - - ranking = 1.3176066450739keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
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