Cases reported "Basal Ganglia Diseases"

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11/426. Acute onset of chorea and dystonia following a febrile illness in a 1-year-old boy.

    A 12-month-old boy with acute onset hemichorea and dystonia following a gastroenteritis has abnormal signal intensities of his basal ganglia on brain magnetic resonance imaging (MRI). A rigorous laboratory investigation is successful in diagnosing his rare condition. A discussion of the differential of abnormal basal ganglia on MRI is presented to help illustrate this case.
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ranking = 1
keywords = tic
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12/426. hemolytic-uremic syndrome with involvement of basal ganglia and cerebellum.

    hemolytic-uremic syndrome is a microangiopathy often associated with neurologic symptoms. Several patients with persistent lesions in cerebrum and basal ganglia have been reported. We present two children with bilateral basal ganglia and additional unilateral cerebellar lesions in magnetic resonance imaging. These resolved completely in one child. In the other child there were still residuals after 11 weeks. The neurologic symptoms of both improved after several therapeutic plasma exchanges and disappeared after months.
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ranking = 7
keywords = tic
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13/426. Deterioration of parkinsonian symptoms following treatment of dopaminergic hallucinosis with olanzapine.

    The atypical antipsychotic drug olanzapine has been proposed for treatment of dopaminergic psychosis in Parkinson's disease (PD). We report on a 68-year-old patient who developed a severe akinetic-rigid extrapyramidal syndrome, accompanied by additional paranoid symptoms, following olanzapine treatment of optic hallucinosis in PD. Olanzapine may also induce clinically relevant extrapyramidal side effects in PD patients.
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ranking = 3
keywords = tic
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14/426. Progeroid syndrome with facial teleangiectatic erythema, posterior subcapsular cataracts, calcification of basal ganglia and atrium septum defect type 2.

    In this report we present the long-term follow-up findings in a young female born to consanguineous parents with the unique association of (1) a progeroid syndrome, (2) facial dysmorphism with relative microcephaly, triangular face, retrognathism and skin erythema, (3) bilateral posterior cataracts, (4) basal ganglia calcifications and (5) atrium septum defect type 2. intelligence is borderline. Clinical evolution after normal puberty was positive with regression of the facial erythematous changes. Over the years differential diagnosis included progeria, hypohidrotic ectodermal dysplasia, Rothmund-Thompson syndrome, cockayne syndrome, bloom syndrome, but the clinical spectrum of abnormalities and the evolution with age were not compatible with one of these diagnoses. Parental consanguinity is in favour of autosomal recessive inheritance.
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ranking = 5
keywords = tic
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15/426. Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature.

    OBJECTIVE: According to the existing viewpoint, Corticobasal degeneration (CBD) is thought of as a predominantly extrapyramidal motor disorder that is distinct and unrelated to frontotemporal dementia (FTD), the most common form of non-Alzheimer dementias. A lack of understanding of the aetiopathogenesis, and poor correlation between the pathology and the clinical syndromes, has resulted in a disparity in the classification of cases of non-Alzheimer dementias. This report intends to highlight the overlap between FTD and CBD in the light of the evolution of these terms, and to discuss the implications of these findings on the nosology of CBD and the classification of non-Alzheimer dementias. methods AND RESULTS: Two cases who presented with cognitive dysfunction, which, on comprehensive neuropsychological testing warranted an antemortem diagnosis of FTD are reported. A detailed necropsy study of their brains, however, favoured a pathological diagnosis of CBD. The literature on the overlap between CBD and FTD is also reviewed. CONCLUSIONS: Firstly, evidence is emerging to suggest that the clear distinction drawn between FTD and CBD by the existing viewpoint, needs revision. Secondly, until such time that a comprehensive classification of non-Alzheimer dementias is evolved, it may be better to distinguish between the clinical and pathological levels of description and to classify cases, in vivo, on the basis of the clinical phenotype.
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ranking = 5.0102465307477
keywords = tic, motor
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16/426. Rhythmic cortical and muscle discharges induced by fatigue in corticobasal degeneration.

    We describe a patient presenting clinical features of corticobasal degeneration (CBD), including reflex myoclonus in the left upper limb. This patient complained of a marked worsening of involuntary movements in the left upper limb after exercise. We analysed the electrophysiological characteristics of myoclonus in the basal state and after a fatiguing exercise in the left upper limb. In the basal condition, single trials recording EEG showed a cortical complex occurring 20 ms after stimulation of the left median nerve. Surface EMG recordings of the left first dorsal interosseous (FDI) revealed an isolated biphasic C1 response 49 ms after stimulation. After exercise, single trials recording EEG following shocks to the left median nerve showed rhythmic complexes with a duration of approximately 80 ms. EEG complexes were made of a series of 3 bursts, with intervals between bursts tending to cluster at approximately 22 ms. These rhythmic complexes were associated with repetitive activity in the left FDI. We conclude that rhythmic cortical and muscle discharges can be induced by fatigue in CBD.
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ranking = 12
keywords = tic
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17/426. Congenital encephalomyopathy with epilepsy, chorioretinitis, basal ganglia involvement, and muscle minicores.

    A woman had severe psychomotor retardation, epilepsy, rigidity, and chorioretinitis. magnetic resonance imaging showed cerebellar and cerebral atrophy and hypointensities in T2-weighted images of the thalami and basal ganglia. Muscle biopsy documented size variations in rounded muscle fibers, fibrosis, and minicores on electron microscopy. Merosin staining was normal. These hitherto unreported features do not permit classification of our patient within the current types of encephalomyopathy and congenital muscular dystrophies.
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ranking = 1.0102465307477
keywords = tic, motor
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18/426. Acute encephalopathy with bilateral striatal necrosis: favourable response to corticosteroid therapy.

    A case of acute encephalopathy with selective bilateral symmetrical striatal lesions is reported. The patient was a previously healthy 4-year-old boy who became obtunded after a febrile illness and fell into a state of delirium with severe pain in the feet. He showed abnormal postures: hyperextension of the neck and upper limbs and extreme flexion of both lower limbs, and abnormal involuntary movements of the limbs: tremor, athetotic movement and right hemiballismus. Analysis of serum antibody titres suggested recent primary infection of herpes simplex type 1 (HSV-1). Cranial T2-weighted magnetic resonance imaging (MRI) demonstrated areas of high-signal intensity involving the whole basal ganglia bilaterally. He showed rapid clinical improvement after the initiation of corticosteroid therapy; complete clinical recovery was noted 3 months after the onset. Serial MRI studies demonstrated a rapid reduction of the lesions, resulting in only slight T2-hyperintense areas in both caudate nuclei. The pathogenesis of the disorder remains unknown, though an autoimmune mechanism has been speculated. The clinical and laboratory findings in this case suggested a possible role of HSV-1 in the pathomechanism of the disorder and a beneficial effect of early corticosteroid therapy.
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ranking = 8
keywords = tic
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19/426. Aromatic L-amino acid decarboxylase deficiency: an extrapyramidal movement disorder with oculogyric crises.

    Aromatic L-amino acid decarboxylase (AADC) deficiency results in an impaired synthesis of catecholamines and serotonin, and has been reported only in two middle eastern families. We report on a European family with an affected child. The child showed the characteristic clinical picture of an extrapyramidal movement disorder, oculogyric crises and vegetative symptoms seen in the three patients described previously. Treatment with a combination of the AADC cofactor pyridoxine, the monoamine oxidase B inhibitor selegiline and bromocriptine was started during the fifth year of life and showed only a moderate clinical improvement in contrast to patients who have been treated since the first year of life.
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ranking = 6
keywords = tic
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20/426. Intraoperative direct subcortical stimulation for identification of the internal capsule, combined with an image-guided stereotactic system during surgery for basal ganglia lesions.

    BACKGROUND: The two main problems of surgery for basal ganglia lesions are: first, the difficulty of accurately localizing the lesion in this deep location; and second, the proximity to the internal capsule, with the risk of permanent postoperative sequelae. The author describes the use of intraoperative direct electrical subcortical stimulation in the identification and preservation of the internal capsule, combined with an image-guided stereotactic system for the selection of the best surgical approach in a case of deep cavernoma. CASE DESCRIPTION: A 33-year-old man was admitted to our institution with a history of three episodes of transitory left hemiparesia in the last 12 years. Neurological examination revealed a mild left weakness. magnetic resonance imaging (MRI) showed typical features of a right posterior capsular-lentiform cavernoma. To prevent another hemorrhagic event, surgery was performed via a right transdistal sylvian approach, using a computer-assisted stereotactic method that allowed us to reach the lesion directly and direct stimulations to detect the subcortical pyramidal pathways. The patient had a transitory worsening with complete recovery in 10 days. Control MRI showed total resection. CONCLUSION: As described at the cortical level, the intraoperative direct subcortical stimulations seem also to represent an easy, safe, accurate, and reliable method of real-time functional identification of the internal capsule during surgery for basal ganglia lesions. The combination with an image-guided stereotactic system to accurately localize the lesion minimizes the risk of postoperative sequelae, and seems to warrant an increase of the surgical indications in this location.
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ranking = 16
keywords = tic
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