1/128. basal ganglia germinoma with progressive cerebral hemiatrophy.The authors describe a 7-year-old Chinese-American female with a germinoma of the basal ganglia who presented with progressive hemiparesis and cerebral hemiatrophy. The additional finding of markedly elevated antiphospholipid antibodies suggests the possibility of an autoimmune pathogenesis for the progressive cerebral atrophy, as well as the later development of cognitive decline, tics, and obsessive-compulsive behaviors.- - - - - - - - - - ranking = 1keywords = cerebral (Clic here for more details about this article) |
2/128. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis.Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination.- - - - - - - - - - ranking = 0.16666666666667keywords = cerebral (Clic here for more details about this article) |
3/128. Parasagittal cerebral injury: magnetic resonance findings.Parasagittal cerebral injury is a specific pathologic lesion that can be found in full-term newborn babies suffering from hypoxic-ischemic encephalopathy. It is defined by the presence of cortical and subcortical white-matter necrosis involving the parasagittal and superomedial areas of the cerebral convexities. We report on two patients who showed parasagittal cerebral injury on magnetic resonance imaging. In both cases antecedents of hypoxic-ischemic encephalopathy were noted. In one of the patients basal ganglia involvement was also detected by cranial magnetic resonance imaging. In the follow-up studies the presence of upper-limb pyramidal signs and dyspraxia were two of the more pronounced symptoms. We will discuss the usefulness of neuroimaging, especially magnetic resonance imaging with coronal views, in the diagnosis of the parasagittal cerebral injury.- - - - - - - - - - ranking = 1.3333333333333keywords = cerebral (Clic here for more details about this article) |
4/128. CT and MRI in severe hypophosphataemia with central nervous system involvement.We report a 38-year-old woman with extreme hypophosphataemia in whom CT and MRI disclosed bilateral lesions within the basal ganglia, thalamus and occipital lobes. After adequate substitution of phosphate the lesions grossly resolved and the patient recovered. This case is the first to demonstrate that profound changes of serum phosphate may be associated with reversible brain lesions.- - - - - - - - - - ranking = 0.14215905222065keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/128. 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.- - - - - - - - - - ranking = 0.16666666666667keywords = cerebral (Clic here for more details about this article) |
6/128. Unusual signs for dural arteriovenous fistulas with diffuse basal ganglia and cerebral calcification.We present a case of multiple dural arteriovenous fistulas (AVFs) in a 60-year-old man with the chief complaint of worsening headache, altered mental status and progressively unsteady gait over the course of one year. Computerized tomography revealed diffuse, symmetric calcification in the bilateral basal ganglia and bilateral periventricular and subcortical white matter. magnetic resonance imaging revealed multiple, enhanced, punctate and linear vessels. These images were due to reflux into the parenchymal veins in the dural AVF of the superior sagittal sinus within the basal ganglia and deep white matter of both cerebral hemispheres. cerebral angiography disclosed multiple dural AVFs. The exact mechanism of basal ganglia and subcortical calcification is proposed to be an arterial steal phenomenon or persistent venous congestion, with calcification occurring in a chronic hypoperfused state or with dystrophic changes in the walls of congested veins.- - - - - - - - - - ranking = 0.83333333333333keywords = cerebral (Clic here for more details about this article) |
7/128. Rapid growth in a cavernoma.The availability of magnetic resonance imaging has greatly increased the detection of cavernous malformations of the central nervous system in both symptomatic and asymptomatic patients. These lesions may be responsible for previously unexplained neurological events or may even have been incorrectly diagnosed. We describe a patient presenting with focal neurological signs in whom an initial diagnosis of malignant glioma had been made. Following excision, the pathological diagnosis was cavernoma. This vascular lesion has continued to show rapid growth and aggressive behaviour despite multiple surgical resections. The indications for operative and non-operative intervention will be discussed.- - - - - - - - - - ranking = 0.035539763055162keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/128. meige syndrome secondary to basal ganglia injury: a potential cause of acute respiratory distress.BACKGROUND: meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on meige syndrome and the relationship between dystonias and respiratory compromise is presented. methods: A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms. RESULTS: Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated. CONCLUSION: This case highlights two interesting aspects of Meige's syndrome: (1) Focal bilateral basal ganglia lesions appear to be responsible for this patient's movement disorder which is consistent with relative overactivity of the direct pathway from striatum to globus pallidus internal and substantia nigra pars reticularis; (2) Respiratory involvement in a primarily craniofacial dystonia to the point of acute airway compromise.- - - - - - - - - - ranking = 0.16666666666667keywords = cerebral (Clic here for more details about this article) |
9/128. Vascular Parkinsonism: a case report and review of the literature.Vascular Parkinsonism (VP) is characterised by sudden onset and rapid progression of clinical symptoms, absent or poor response to dopamine substitution therapy, and postural instability with shuffling gait and absence of tremor, making it a clinically distinct entity from idiopathic Parkinson's disease (IPD). Furthermore, it displays certain typical findings in neurological investigations. We report on a patient presenting features of VP associated with an intracerebral lesion not ascribed to VP to date, namely an isolated ischaemic focal lesion located in the left cerebral peduncle between the substantia nigra and nucleus ruber as evidenced by magnetic resonance imaging (MRI). The pathophysiological organic correlate for contralateral extrapyramidal symptoms in this patient may be an interruption of nigro-thalamic projection, interrupting the final subcortical station in the cortic-striato-pallido-nigro-thalamico-cortical loop central to the pathophysiology of parkinsonian syndromes. Non-response t o levodopa therapy could be a consequence of disruption of the cortico-basal ganglia-cortical loop on account of ischaemic destruction of subcortico-cortical axons, the underlying pathology, therefore, not being the result of a loss of nigral dopaminergic neurons or striatal dopamine deficiency pathogonomonic of IPD. To our knowledge, this is the first case of clinically manifest VP to be described with a single lesion in the contralateral cerebral peduncle between the substantia nigra and nucleus ruber, and suggests alternative intracerebral patterns for the distribution of disease-causing lesions in VP, and possibly new pathophysiological explanations for the nature of this disease.- - - - - - - - - - ranking = 0.66666666666667keywords = cerebral (Clic here for more details about this article) |
10/128. Acute necrotizing encephalopathy presenting as a basal ganglia syndrome.Acute necrotizing encephalopathy is a relatively new disease. The characteristic clinical findings are of febrile illness followed by rapid deterioration in mental status and seizures. The hallmark of the disease is multifocal bilateral symmetric lesions affecting the thalamus, hypothalamus, brainstem tegmentum, cerebral white matter, and cerebellum. The etiology is unknown, but immune-mediated mechanism was suggested. We present a 12-year-old previously healthy girl who developed increased sleepiness progressing to stupor and coma. magnetic resonance imaging (MRI) of the brain showed the characteristic findings previously described in acute necrotizing encephalopathy. Her mental status improved dramatically with steroid treatment, and the MRI findings resolved completely within 6 months. Following the acute illness, she developed a complex neuropsychiatric disorder consistent with basal ganglia syndrome.- - - - - - - - - - ranking = 0.16666666666667keywords = cerebral (Clic here for more details about this article) |
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