Relapsing demyelinating disease after chicken pox in a child.
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Chickenpox↗
Biomedical subjects
C Colosimo
Publications and source records attributed to C Colosimo.
Phase II trial of temozolomide in children with recurrent high-grade glioma.
PURPOSE: The objective of the study was to evaluate the efficacy and toxicity of Temozolomide (TMZ) administered for 5 consecutive days in three daily dosing in children with recurrent or refractory high-grade glioma. PATIENTS AND METHODS: Twenty-four patients with a median age of 10.5 years were enrolled onto this open-label, multicenter, phase II study. The patients were previously treated with surgical resection (17 of 24), radiotherapy (19 of 24) and chemotherapy (18 of 24). Therapy was administered orally three times a day for 5 consecutive days at the dose of 200 mg/m(2)/dx5 for chemotherapy naive patients. In patients heavily pretreated with chemotherapy the starting dose was of 150 mg/m(2)/dx5. RESULTS: A total of 95 cycles were administered. The median progression free-survival (PFS) was 3 months for the entire group while disease stabilization was obtained in 7 patients (29.1%), all with supratentorial tumors. No CR or PR was observed. TMZ treatment showed a limited toxicity. Thrombocytopenia was the most common hematological adverse effect. Our data suggest a marginal activity of TMZ in children with recurrent high-grade glioma.
Adolescent↗
Clinical value of botulinum toxin in neurological indications.
Botulinum toxin type-A (BoNT-A) prevents the release of acetylcholine at cholinergic junctions, thereby causing temporary muscle weakness lasting 3-4 months. It is now widely used to treat a broad range of clinical disorders characterized by muscle hyperactivity. BoNT-A has proved effective in the management of several neurological conditions and, in particular, in the management of movement disorders (e.g. blepharospasm, cervical dystonia, laryngeal dystonia, limb dystonia, hemifacial spasm, focal tics, tremor and other hyperkinetic disorders). As a treatment of spasticity, BoNT-A can improve mobility and dexterity as well as preventing the development of distressing and costly secondary complications. In cerebral palsy, BoNT-A is of value, being able to delay or even avoid surgery until motion patterns have become established.
Adult↗
TIA associated with over-the-counter cold preparation.
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Cerebral Arteries↗
Transient MRI abnormalities in a case of occipital lobe epilepsy with favorable outcome.
We describe the case of a 13-year-old boy, in good health, with transient occipital MRI abnormalities just after one generalized seizure and the appearance of macropsia. The EEG showed a 3-5 Hz sub-continuous left occipital activity, with sporadic sharp waves. Macropsia disappeared in 2 years. This case may suggest that the presence of transient MRI abnormalities does not exclude a favorable prognosis.
Adolescent↗
The European Multiple System Atrophy-Study Group (EMSA-SG).
Introduction. The European Multiple System Atrophy-Study Group (EMSA-SG) is an academic network comprising 23 centers across Europe and Israel that has constituted itself already in January 1999. This international forum of established experts under the guidance of the University Hospital of Innsbruck as coordinating center is supported by the 5th framework program of the European Union since March 2001 (QLK6-CT-2000-00661). Objectives. Primary goals of the network include (1) a central Registry for European multiple system atrophy (MSA) patients, (2) a decentralized DNA Bank, (3) the development and validation of the novel Unified MSA Rating Scale (UMSARS), (4) the conduction of a Natural History Study (NHS), and (5) the planning or implementation of interventional therapeutic trials. Methods. The EMSA-SG Registry is a computerized data bank localized at the coordinating centre in Innsbruck collecting diagnostic and therapeutic data of MSA patients. Blood samples of patients and controls are recruited into the DNA Bank. The UMSARS is a novel specific rating instrument that has been developed and validated by the EMSA-SG. The NHS comprises assessments of basic anthropometric data as well as a range of scales including the UMSARS, Unified Parkinson's Disease Rating Scale (UPDRS), measures of global disability, Red Flag list, MMSE (Mini Mental State Examination), quality of live measures, i.e. EuroQoL 5D (EQ-5D) and Medical Outcome Study Short Form (SF-36) as well as the Beck Depression Inventory (BDI). In a subgroup of patients dysautonomic features are recorded in detail using the Queen Square Cardiovascular Autonomic Function Test Battery, the Composite Autonomic Symptom Scale (COMPASS) and measurements of residual urinary volume. Most of these measures are repeated at 6-monthly follow up visits for a total study period of 24 months. Surrogate markers of the disease progression are identified by the EMSA-SG using magnetic resonance and diffusion weighted imaging (MRI and DWI, respectively). Results. 412 patients have been recruited into the Registry so far. Probable MSA-P was the most common diagnosis (49% of cases). 507 patients donated DNA for research. 131 patients have been recruited into the NHS. There was a rapid deterioration of the motor disorder (in particular akinesia) by 26.1% of the UMSARS II, and - to a lesser degree - of activities of daily living by 16.8% of the UMSARS I in relation to the respective baseline scores. Motor progression was associated with low motor or global disability as well as low akinesia or cerebellar subscores at baseline. Mental function did not deteriorate during this short follow up period. Conclusion. For the first time, prospective data concerning disease progression are available. Such data about the natural history and prognosis of MSA as well as surrogate markers of disease process allow planning and implementation of multi-centre phase II/III neuroprotective intervention trials within the next years more effectively. Indeed, a trial on growth hormone in MSA has just been completed, and another on minocycline will be completed by the end of this year.
Animals↗
Functional MRI study of diencephalic amnesia in Wernicke-Korsakoff syndrome.
Anterograde amnesia in Wernicke-Korsakoff syndrome is associated with diencephalic lesions, mainly in the anterior thalamic nuclei. Whether diencephalic and temporal lobe amnesias are distinct entities is still not clear. We investigated episodic memory for faces using functional MRI (fMRI) in eight controls and in a 34-year-old man with Wernicke-Korsakoff syndrome and diencephalic lesions but without medial temporal lobe (MTL) involvement at MRI. fMRI was performed with a 1.5 tesla unit. Three dual-choice tasks were employed: (i) face encoding (18 faces were randomly presented three times and subjects were asked to memorize the faces); (ii) face perception (subjects indicated which of two faces matched a third face); and (iii) face recognition (subjects indicated which of two faces belonged to the group they had been asked to memorize during encoding). All activation was greater in the right hemisphere. In controls both the encoding and recognition tasks activated two hippocampal regions (anterior and posterior). The anterior hippocampal region was more activated during recognition. Activation in the prefrontal cortex was greater during recognition. In the subject with Wernicke-Korsakoff syndrome, fMRI did not show hippocampal activation during either encoding or recognition. During recognition, although behavioural data showed defective retrieval, the prefrontal regions were activated as in controls, except for the ventrolateral prefrontal cortex. fMRI activation of the visual cortices and the behavioural score on the perception task indicated that the subject with Wernicke-Korsakoff syndrome perceived the faces, paid attention to the task and demonstrated accurate judgement. In the subject with Wernicke-Korsakoff syndrome, although the anatomical damage does not involve the MTL, the hippocampal memory encoding has been lost, possibly as a consequence of the hippocampal-anterior thalamic axis involvement. Anterograde amnesia could therefore be the expression of damage to an extended hippocampal system, and the distinction between temporal lobe and diencephalic amnesia has limited value. In the subject with Wernicke-Korsakoff syndrome, the preserved dorsolateral prefrontal cortex activation during incorrect recognition suggests that this region is more involved in either the orientation or attention at retrieval than in retrieval. The lack of activation of the prefrontal ventrolateral cortex confirms the role of this area in episodic memory formation.
Adult↗
Management of multiple system atrophy: state of the art.
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease of undetermined aetiology presenting with parkinsonian, autonomic, cerebellar, and pyramidal signs. Despite the lack of any effective therapy to reverse MSA, some of the symptoms may be improved with adequate symptomatic therapies. Medical treatment is largely aimed at mitigating the parkinsonian and autonomic features. The therapeutic results of levodopa therapy in cases of MSA are difficult to interpret because of their variability. Nevertheless, the simple statement that patients with MSA do not respond to levodopa is false. Clinical and pathologically proven series document levodopa efficacy in about 40-60% of patients with MSA and predominant parkinsonian features. Other antiparkinsonian compounds (dopamine agonists, amantadine) may also be employed, but they are not more effective than levodopa. Orthostatic hypotension (OH) can be suspected from the patient s history and subsequently documented in the clinic by measuring lying and standing blood pressure. The diagnosis ideally should be confirmed with additional laboratory tests to determine the cause and evaluate the functional deficit, so as to aid treatment. A number of pharmacological agents with different mechanisms of action have been used in MSA to reduce OH when this is symptomatic. OH can also be alleviated by avoiding aggravating factors, such as the effects of food, micturition, exposure to a warm environment, and physiological diurnal changes, and by using other non-pharmacological strategies. The treatment of the very common genitourinary symptoms (incontinence, retention, impotence) should also be considered in order to improve the quality of life of these patients.
Animals↗
Diffusion tensor imaging in primary cervical dystonia.
BACKGROUND: It is the traditional view that primary dystonia arises from abnormal basal ganglia function but causes no apparent morphological changes. OBJECTIVE: To determine whether cervical dystonia leads to ultrastructural changes in the brain, using diffusion tensor imaging to compare brain structure in 15 patients with cervical dystonia with 10 healthy controls. DESIGN: Fractional anisotropy (FA) and mean diffusivity (MD) were obtained in 17 brain regions of interest. RESULTS: Patients had higher FA values than controls in both putamina and lower FA values in the genu and in the body of the corpus callosum. Patients also had lower MD values in the left pallidum, the left putamen, and both caudati. CONCLUSIONS: In patients with cervical dystonia, diffusion tensor imaging shows ultrastructural changes in specific brain areas, including the basal ganglia.
Anisotropy↗
Multidisciplinary approach for diagnosing syncope: a retrospective study on 521 outpatients.
OBJECTIVES: To describe causes of syncope in outpatients in whom structural heart disease was ruled out as a cause, and to analyse the role of a multidisciplinary approach in a syncope unit for the diagnosis of patients with syncope of unknown origin. METHODS: Cardiovascular autonomic nervous system (ANS) function was evaluated extensively in 521 outpatients by careful history, physical examination including orthostatic blood pressure measurement and standard ECG, and tilt testing. RESULTS: Causes of syncope remained unknown in 29.2% of cases. ANS dysfunction was found in 58.6% of those presenting with either neurally mediated syncope (53.6%) or chronic autonomic failure (5%); 3.8% of the patients suffered from syncope of cardiogenic origin (2.5%) or non-neurogenic hypotension (1.3%), and 8.4% had loss of consciousness of non-syncopal origin. Loss of consciousness was confirmed as being related to seizures in under 30% of patients initially diagnosed as having epilepsy. CONCLUSIONS: Neurally mediated syncope represents the commonest type of syncope. ANS evaluation including tilt testing should be considered as preliminary screening in patients with syncope in the absence of definite heart abnormalities. Neurologists should consider syncope from ANS failure as a comorbid factor in patients with seizures where the clinical characteristics are not straightforward.
Adult↗
A comparison of Gd-BOPTA and Gd-DOTA for contrast-enhanced MRI of intracranial tumours.
A two-centre intra-individual crossover study was performed in 23 patients with suspected high-grade glioma or metastases to assess and compare the safety and enhancement characteristics of two different MRI contrast media (gadobenate dimeglumine, Gd-BOPTA and gadoterate meglumine, Gd-DOTA) at equivalent doses of 0.1 mmol/kg body weight. T1-weighted spin-echo (SE) and T2-weighted fast SE images were obtained before and T1-weighted images 0, 2, 4, 6, 8 and 15 min after injection. T1-weighted images with magnetisation transfer contrast were acquired 12 min after injection. Qualitative assessment by blinded, off-site readers (reader 1:19 patients; reader 2:21) and on-site investigators (23) revealed significant (P< or =0.005) overall preference for Gd-BOPTA over Gd-DOTA for contrast enhancement (Gd-BOPTA preferred in 18, 15 and 18 cases; Gd-DOTA in 0, 1 and 1 and no preference in 1, 5 and 4; off-site readers 1 and 2, and on-site investigators, respectively). A similar significant preference for Gd-BOPTA was expressed by off-site readers and on-site investigators for lesion-to-brain contrast, lesion delineation, internal lesion structure, and overall image preference. Quantitative assessment by off-site readers revealed significantly (p<0.05) greater lesion enhancement with Gd-BOPTA than with Gd-DOTA at all times from 2 min after injection.
Aged↗
The role of brain infarcts and hippocampal atrophy in subcortical ischaemic vascular dementia.
We investigated if, in patients with vascular lesions, the variable that best discriminated demented from non-demented patients was the severity of the vascular pathology or the degree of hippocampal atrophy. A total of 39 patients multiple subcortical infarcts, who could be considered as possible vascular dementia with small vessel pathology, with underwent a neuropsychological study and brain magnetic resonance imaging (MRI) DSM IV criteria supported by neuropsychological data were used to distinguish demented from non-demented patients. The MRI study took into account the degree of hippocampal atrophy (hippocampal height and interuncal distance) and the severity of vascular pathology (number of brain infarcts). The distribution of lesions and a factor analysis showed that hippocampal atrophy is a better predictor of dementia than the number of brain infarcts. Multiple subcortical infarcts alone are probably not able to cause clinical dementia but the presence of vascular lesions increases the expression of concomitant Alzheimer's disease.
Aged↗
Direct recording of the output of the motor cortex produced by transcranial magnetic stimulation in a patient with cerebral cortex atrophy.
OBJECTIVE: To examine the descending motor activity evoked by transcranial magnetic stimulation (TMS) in a chronic alcoholic patient with a slight atrophy of the peri-central cortex and compare with that observed in neurologically intact subjects. METHODS: EMGs from the first dorsal interosseous (FDI) muscle, and descending activity from an electrode implanted in the high cervical epidural space for relief of pain were recorded after TMS of the hand area of motor cortex. A figure-of-8 coil was used to induce either a posterior-anterior (PA) or a latero-medial (LM) flow across the central sulcus. RESULTS: In intact subjects, LM stimulation evoked the earliest volley, which we termed a D wave. This was followed by later, presumed I waves at intervals of about 1.5 ms. At a stimulus intensity of 120% resting threshold (RMT), up to 5 I waves were seen. PA stimulation rarely evoked D waves at intensities up to 120% RMT, but 3 or 4 I waves were visible in all subjects. The patient had an increased resting threshold, and the descending volleys were dominated by a D wave. I waves were unclear, with two possible small peaks at 5.5 and 7.2 ms. CONCLUSIONS: The lack of I waves in the patient was probably due to an impairment of interneuronal circuitry in the context of the brain damage related to chronic alcohol abuse, and is consistent with a trans-synaptic origin of the I waves in humans. The intact D wave is consistent with the assumption that the D wave represents direct stimulation of the axons of intact corticospinal neurones in the subcortical white matter. The patient's increased RMT reflects the fact that usually multiple descending volleys are needed to discharge resting spinal motoneurones.
Alcoholism↗
Is hypertension associated with hemifacial spasm?
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Case-Control Studies↗
Desmoplastic infantile ganglioglioma.
BACKGROUND: Desmoplastic infantile gangliogliomas are rare intracranial tumors that mostly occur in the first 2 years of life. They are exclusively supratentorial, generally have a voluminous size, and are partially cystic. Histologically they are characterized by a divergent astrocytic and ganglionic differentiation and a prominent desmoplastic stroma; more primitive cells may be observed, which present a higher number of mitoses and these areas can mimic the features of malignant astrocytomas. Surgery is the treatment of choice. Data available from the literature suggest that no complimentary treatment is needed in cases of complete tumor resection. Chemotherapy is an option in infants with infiltration of eloquent CNS structures and progressive disease after surgery. DIGs have generally a good prognosis: recurrence-free intervals of up to 14 years have been reported and spontaneous disappearance of tumor residuals has also been described. CASE REPORTS ND DISCUSSION:We report two cases of DIGs. The first child underwent a staged partial removal of a huge right fronto-temporo-parietal tumor when she was 2 months old. At that time histological diagnosis was anaplastic astrocytoma and on these grounds she underwent six chemotherapy cycles, with a partial reduction of the tumor residual. When she was 16 months old a new operation and complete removal of the tumor residual was performed; histological diagnosis was DIG. A review of the initial histological samples confirmed this diagnosis. Twenty-two months after surgery no tumor recurrence has been documented. This case is an example of a difficult differential diagnosis, which can lead to incorrect management choices. The second patient was operated on when he was 9 months old for a mostly cystic right temporo-parieto-occipital DIG. At surgery a deep nodule, strictly adherent to the Galen and internal cerebral veins, was not removed. MRI control 9 months after surgery showed the disappearance of the tumor residual. Eleven years after surgery no tumor recurrence has been documented. The history of this patient confirms that tumor residuals do not need complimentary treatment; indeed they do not usually grow and, as in our patient, they can spontaneously disappear.
Astrocytes↗
Infantile myofibromatosis of the central nervous system.
BACKGROUND: Infantile myofibromatosis is the most common fibrous disorder of infancy and childhood. It may occur in two distinct forms: multicentric and solitary. In both cases involvement of the central nervous system (CNS) is unusual: brain myofibromas are usually intracranial in proximity of the dura mater, with infiltration of the calvarial bones and secondary brain compression. Spine myofibromas are exceptional and most of the cases reported in the literature represent secondary locations of visceral lesions. The natural history of myofibromas of the CNS in infantile myofibromatosis is characterized by a period of rapid growth, subsequent stabilization, and spontaneous regression in many cases. On these grounds surgical treatment is reserved for lesions that compress the brain or spinal cord and show an obvious progression. Small lesions or tumor residuals in asymptomatic children without visceral involvement should be closely observed as first choice. CASE REPORT: We report the case of a 10-month-old child with a solitary intramedullary myofibroma, extended from C7 to the conus. The patient underwent partial surgical removal of this lesion. Control MRI has shown the spontaneous slow reduction of the tumor residual and a progressive improvement of preoperative neurological conditions has been observed. To our knowledge this is the first case in which a solitary myofibroma was localized within the spinal cord.
Adolescent↗
Multiphasic demyelinating disease involving central and peripheral nervous system in a child.
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Central Nervous System Diseases↗
Apraxia of eyelid opening in a case of atypical corticobasal degeneration.
Apraxia of eyelid opening (AEO) occurs in several clinical conditions, even in the absence of any other neurological sign; nonetheless, in most of the cases AEO has been reported in association with basal ganglia diseases, such as corticobasal degeneration (CBD). We describe a patient with a clinical diagnosis of frontotemporal dementia who, later, developed parkinsonian signs and AEO. We suggest that the finding of AEO in patients with a frontotemporal syndrome could be a helpful expedient for the early diagnosis of atypical clinical findings of CBD, characterised by behavioural and cognitive aspects at first.
Basal Ganglia↗