BILATERAL CAROTID BODY PARAGANGLIOMAS - LITERATURE REVIEW AND COMMENTS IN A PATIENT WITH NO SIGNS OF MEN SYNDROME.

Paragangliomas are rare neuroendocrine slow-growing tumors, often asymptomatic, that originate from embryonic neural crest cell. In the head and neck area, the most common location is the carotid body, followed, with decreasing frequency, in jugular, tympanic and vagal sites. Bilateral carotid body tumors are extremely rare. Aim: To present the most important features of carotid body paragangliomas, illustrating the clinical characteristics, associated with a thorough analysis of the diagnostic imaging elements, but also the current therapeutic strategies, with respective anatomical, surgical considerations and potential complications that can occur. Surgical resection is the main line of treatment. The complex anatomy of the cervical region and the close relationships of carotid body paragangliomas with carotid vessels and cranial nerves, as well as its intense vascularization makes the surgical intervention a real challenge even for an experienced surgeon. Discussion: Starts from a bilateral carotid paraganglioma in a 35-year-old male, with painless lateral neck swelling, accidentally discovered by his barber about two years ago. Diagnosis was suspected on the basis of history, clinical and radiological findings. "Wait and scan" strategy plus endocrinologic assessment for MEN syndromes were considered the optimal therapeutic approach in this case.

Functional and structural balances of homologous sensorimotor regions in multiple sclerosis fatigue.

Fatigue in multiple sclerosis (MS) is a highly disabling symptom. Among the central mechanisms behind it, an involvement of sensorimotor networks is clearly evident from structural and functional studies. We aimed at assessing whether functional/structural balances of homologous sensorimotor regions-known to be crucial for sensorimotor networks effectiveness-decrease with MS fatigue increase. Functional connectivity measures at rest and during a simple motor task (weak handgrip of either the right or left hand) were derived from primary sensorimotor areas electroencephalographic recordings in 27 mildly disabled MS patients. Structural MRI-derived inter-hemispheric asymmetries included the cortical thickness of Rolandic regions and the volume of thalami. Fatigue symptoms increased together with the functional inter-hemispheric imbalance of sensorimotor homologous areas activities at rest and during movement, in absence of any appreciable parenchymal asymmetries. This finding supports the development of compensative interventions that may revert these neuronal activity imbalances to relieve fatigue in MS.

Inter-hemispheric functional connectivity changes with corpus callosum morphology in multiple sclerosis.

Multiple sclerosis (MS) affects myelin sheaths within the central nervous system, concurring to cause brain atrophy and neurodegeneration as well as gradual functional disconnections. To explore early signs of altered connectivity in MS from a structural and functional perspective, the morphology of corpus callosum (CC) was correlated with a dynamic inter-hemispheric connectivity index. Twenty mildly disabled patients affected by a relapsing-remitting (RR) form of MS (EDSS⩽3.5) and 15 healthy subjects underwent structural MRI to measure CC thickness over 100 sections and electroencephalography to assess a spectral coherence index between primary regions devoted to hand control, at rest and during an isometric handgrip. In patients, an overall CC atrophy was associated with increased lesion load. A less efficacious inter-hemispheric coherence (IHCoh) during movement was associated with CC atrophy in sections interconnecting homologous primary motor areas (anterior mid-body). In healthy controls, less efficacious IHCoh at rest was associated with a thinner CC splenium. Our data suggest that in mildly disabled RR-MS patients a covert impairment may be detected in the correlation between the structural (CC thickness) and functional (IHCoh) measures of homologous networks, whereas these two counterparts do not yet differ individually from controls.

Cortico-muscular coherence as an index of fatigue in multiple sclerosis.

BACKGROUND: Highly common in multiple sclerosis (MS), fatigue severely impacts patients' daily lives. Previous findings of altered connectivity patterns led to the hypothesis that the distortion of functional connections within the brain-muscle circuit plays a crucial pathogenic role. OBJECTIVE: The objective of this paper is to identify markers sensitive to fatigue in multiple sclerosis. METHODS: Structural (magnetic resonance imaging with assessment of thalamic volume and cortical thickness of the primary sensorimotor areas) and functional (cortico-muscular coherence (CMC) from simultaneous electroencephalo- and surface electromyographic recordings during a weak handgrip task) measures were used on 20 mildly disabled MS patients (relapsing-remitting course, Expanded Disability Status Scale score ≤ 2) who were recruited in two fatigue-dependent groups according to the Modified Fatigue Index Scale (MFIS) score. RESULTS: The two groups were similar in terms of demographic, clinical and imaging features, as well as task execution accuracy and weariness. In the absence of any fatigue-dependent brain and muscular oscillatory activity alterations, CMC worked at higher frequencies as fatigue increased, explaining 67% of MFIS variance (p=.002). CONCLUSION: Brain-muscle functional connectivity emerged as a sensitive marker of phenomena related to the origin of MS fatigue, impacting central-peripheral communication well before the appearance of any impairment in the communicating nodes.

Excess of nonceruloplasmin serum copper in AD correlates with MMSE, CSF [beta]-amyloid, and h-tau.

OBJECTIVE: To assess whether serum copper in Alzheimer disease (AD) correlates with cognitive scores, beta-amyloid, and other CSF markers of neurodegeneration. METHODS: The authors studied copper, ceruloplasmin, total peroxide, and antioxidants levels (TRAP) in serum; beta-amyloid in plasma; and copper, beta-amyloid, h-tau, and P-tau in the CSF of 28 patients with AD and 25 healthy controls, in relation to clinical status. RESULTS: Serum copper (p < 0.0001), peroxides (p = 0.002), a copper fraction unexplained by ceruloplasmin (p < 0.0001), and CSF h-tau (p = 0.001) were increased in AD, whereas serum TRAP (p = 0.03) and CSF beta-amyloid were decreased (p < 0.0001). Plasma beta-amyloid increased with age in healthy controls (r = 0.6; p = 0.05). CSF markers of AD correlated with serum copper variables. CSF copper was partially dependent on the serum copper fraction unexplained by ceruloplasmin (t = 2.2, p = 0.04). CSF beta-amyloid seemed to be related to serum copper (r = -0.46; p = 0.002). Mini-Mental Status Examination scores correlated positively with beta-amyloid (r = 0.46, p = 0.002) and inversely with copper unexplained by ceruloplasmin (r = -0.45, p = 0.003). CONCLUSIONS: The authors' results confirm the existence of changes in copper component distribution, particularly the copper fraction unexplained by ceruloplasmin and support the hypothesis of a beta-amyloid and copper connection in Alzheimer disease.

Excess of serum copper not related to ceruloplasmin in Alzheimer disease.

OBJECTIVE: To assess the role of serum copper in relation to ceruloplasmin and other peripheral markers of inflammation in Alzheimer disease (AD). METHODS: The authors studied serum levels of copper, ceruloplasmin, and transferrin, as well as total peroxides, antioxidants, and other peripheral markers of inflammation in 47 patients with AD, 24 patients with vascular dementia (VaD), and 44 healthy controls. Biochemical variables were related to the patients' and controls' clinical status. RESULTS: The authors found that copper (p < 0.001), peroxides (p = 0.026), and ceruloplasmin (p = 0.052) were increased and TRAP was decreased (p = 0.006) in patients with AD, while no other markers of inflammation were altered. The calculation of the ratio between copper and ceruloplasmin suggested the presence in the serum of AD patients, but not of VaD or normal controls, of a large pool of non-ceruloplasmin-bound copper. CONCLUSIONS: Changes in the distribution of the serum copper components, consisting of an increase of a copper fraction not explained by ceruloplasmin, seem to be characteristic of Alzheimer disease and may be implicated in the pathogenesis of the disease.

Postpartum cerebral ischaemia after accidental dural puncture and epidural blood patch.

Puerperal women are reported to have a rate of cerebral infarction 13 times greater than non-pregnant females. We report a case of cerebral ischaemia in a 30-yr-old healthy parturient after epidural analgesia for labour, complicated by dural puncture treated with two epidural blood patches. Investigations showed the development of cerebral ischaemia on postpartum day 14. A transcranial Doppler ultrasonography showed vasospasm of the left middle cerebral artery still present at 3-month follow-up. At 1-yr follow-up, the patient had homonymous hemianopsia. We discuss the possible causative mechanism of the cerebral ischaemia in relation to the dural puncture and epidural blood patch.

Elevation of serum copper levels in Alzheimer's disease.

OBJECTIVE: To determine whether serum trace metals and oxidative species are related to abnormal cognition in AD. METHODS: The authors studied serum peroxides, copper, iron, transferrin, and antioxidant capacity in 79 patients with AD (mean age 74.3 years; 25 men, 54 women) and in 76 cognitively normal individuals (mean age 70.1 years; 33 men, 43 women). The relation of these oxidative and trace metals to APOE epsilon4 allele frequency, neuropsychological performance, and cerebrovascular or atrophic burden, as estimated by brain MRI and ultrasonography of cerebral vessels, was evaluated. RESULTS: Copper level was higher (p < 0.001) in subjects with AD than control subjects (specificity = 95%, sensitivity = 60%) with a cutoff serum level of 16 micro mol/L (1.02 mg/L). An increase of 1 micro mol/L in serum copper accounted for 80% of the risk of having AD and correlated with poor neuropsychological performance and medial temporal lobe atrophy (p < 0.03). Antioxidant capacity decreased and correlated with medial temporal lobe atrophy (p < 0.009) and with APOE epsilon4 allele (p = 0.004). CONCLUSIONS: Copper may play a role in neurodegenerative processes in AD, and serum copper measurement may prove to be a peripheral diagnostic marker for AD.

Interhemispheric differences of sensory hand areas after monohemispheric stroke: MEG/MRI integrative study.

Seventeen clinically stabilized monohemispheric stroke patients were studied in order to investigate the chronic topographical modifications induced on primary sensory cortical hand areas by a monohemispheric stroke within the middle cerebral artery territory. Magnetoencephalographic (MEG) localization of the cortical areas activated following electrical separate stimulation of the median nerve, thumb, and little fingers was integrated with magnetic resonance imaging. Spatial localization of Equivalent Current Dipoles (ECDs) of the short-latency cortical responses generated in primary sensory cortices, "hand area" (distance between 1st and 5th digits ECDs), interhemispheric differences of such parameters, as well as of somatosensory-evoked fields waveshapes were investigated and compared with a control population. Lesions involving the cortico-subcortical areas receiving sensory input from the hand induced excessive asymmetry of MEG spatial parameters and response morphology between the unaffected (UH) and the affected hemisphere (AH). "Hand area" was significantly larger on AH in 20% of cases after a subcortical, and in 13% after a cortical, lesion. Responses from AH were excessively delayed in 20% ECDs. Interhemispheric ECDs strength differences were larger than normal in 25% of cases after both types of lesions; the strength in the AH being enlarged after all cortical, and only 24% of subcortical strokes. In a significant percentage of monohemispheric strokes, excessive interhemispheric differences were found between AH and UH, suggesting that brain areas outside the normal boundaries and usually not reached by a dense sensory input from the opposite hand and fingers may act as somatosensory "hand" centers. Correlation analysis between clinical outcome and cortical reorganization in the AH suggests that this mechanism is linked with hand sensorimotor recovery.

Patterns of cognitive impairment in secondary progressive stable phase of multiple sclerosis: correlations with MRI findings.

Cognitive impairment is commonly described in multiple sclerosis (MS), but conflicting results have been reported about its pattern by previous studies focused on heterogeneous patient groups. The aim of this study was to investigate the cognitive skills of a homogeneous group of secondary progressive MS patients, and to examine the relationship of this impairment to MRI parameters. Forty-four MS patients underwent a series of neuropsychological tests devised to explore the main cognitive domains, and T1- and T2-weighted brain MRI. Results showed the presence of deficits of attention, memory, planning abilities, problem-solving and conceptual reasoning (frontal functions) in a subgroup of MS patients. Correlations between the performance in some 'frontal' tests and the extent of frontal lobe MRI lesional area were present, but rather unspecific, the same performance being also correlated with the nonfrontal lesional area. These findings suggest that in MS, overall macroscopic and microscopic brain damage is more important than the corresponding focal brain disease, even in determining deficits of selective cognitive domains.

On the reorganization of sensory hand areas after mono-hemispheric lesion: a functional (MEG)/anatomical (MRI) integrative study.

The topography of primary sensory cortical hand area following a monohemispheric lesion (sudden = stroke; progressive = neoplasm) was investigated in relationship with clinical recovery of sensorimotor deficits. Twenty seven patients with monohemispheric lesions were studied in a clinically stabilized condition. Functional informations from magnetoencephalography (MEG) were integrated with anatomical data from magnetic resonance imaging (MRI). MEG localizations of the neurons firing at early latencies in primary sensory cortex after separate stimulation of median nerve, thumb and little fingers of each hand were carried out. Characteristics of cerebral equivalent current dipoles (ECDs) activated by each contralateral stimulation, the 'hand extension' (i.e., the distance in millimetres between ECDs of first and fifth digits), as well as interhemispheric differences of the tested parameters were investigated. Finally, ECDs' locations were integrated with MRI. Lesions involving cortical (C) or subcortical (s.c.) areas receiving sensory input from the hand were often combined to increase interhemispheric asymmetry of the tested parameters (22% for C and 49% for s.c. lesions). This might be due to an activation of neuronal districts which in the affected hemisphere (AH) differ from those normally activated in the unaffected hemisphere (UH) and in the control population. Moreover, the 'hand extension' was enlarged on the AH--more frequently after a SC lesion--mainly due to a medial shift of the little finger ECD, combined to a tendency of both finger ECDs to shift frontally. After a C lesion, responses from the AH were often stronger than normal. Spatial reorganizations were also seen in the UH (7% of C and 14% of SC lesions). 'Hand extension' in the UH was selectively enlarged for the P30m only when combined with a similar enlargement in the AH. Significant interhemispheric asymmetries due to neuronal reorganization in the AH were associated with worse clinical outcomes compared to patients without asymmetries.

Anatomy of the lumbar epidural region using magnetic resonance imaging: a study of dimensions and a comparison of two postures.

Many techniques used to investigate the epidural region may alter the anatomy. Magnetic resonance imaging (MRI) has been introduced as a non-invasive diagnostic technique. The aim of this study was to investigate the anatomy of the lumbar epidural region using MRI, studying the morphology, the dimensions and the modification that may result from a change of position. Ten young, healthy female volunteers were studied to obtain results relevant to obstetric anesthesia. The following measurements were made: anterior epidural space (AES); posterior epidural space (PES); ligamenta flava; distance between the PES and the skin (S-ES); and interspace distance (ISD). All these distances were measured with the subject supine and the lumbar spine either in a neutral or a non-lordotic (flexed) position. The S-ES ranged from 2.7 to 8.1 cm. This distance was greater in the lower than in the upper lumbar segments. No differences were observed in AES, PES and ligamenta flava between the neutral and flexed positions. Flexion of the spine did not affect the S-ES but increased the ISD (P < 0.05). The S-ES at any lumbar segment or interspace and the thickness of ligamenta flava at the L2/3 interspace correlated significantly with body weight (respectively, P < 0.01 and P < 0.02). In conclusion, MRI may clearly reveal the anatomy of the epidural region. The circumferentially and metamerically segmented compartments of the epidural space were clearly noted and measured. Ligamenta flava seem to be thinner in younger than in older subjects. This may partly explain a reduced loss of resistance sometimes perceived in obstetric patients.

[Biomechanical changes in and lesions of the lumbosacral spine in horse riding. Magnetic resonance assessment]. / Modificazioni biomeccaniche e alterazioni traumatiche del rachide lombo-sacrale negli sport equestri. Valutazione con Risonanza Magnetica.

Spinal injuries account for 5-15% of sport-related traumas. Equestrian sports are considered, together with rugby, motor sport and diving, the riskiest sport for severe spinal injuries. We investigated the biomechanical changes and repeated microtraumas in equitation. We examined with MR the lumbar spines of 12 professional horsewomen and horsemen, 18-51 years old (mean: 33.4 years), belonging to F.I.S.E. (the Italian Federation for Equestrian Sports) and classified as Olympic riders, Senior and Young European riders. An 0.3-T resistive and an 0.2-T permanent magnets with dedicated surface coils were used. We also examined a control group of non-professional healthy volunteers homogeneous by age. MR findings were classified as follows: 1) changes in normal bending and angles of the lumbar spine; 2) injuries and changes in lumbar disks; 3) changes in spinal ligaments; 4) vertebral body injuries. MR was very sensitive in the assessment of all lumbosacral components in all the athletes; besides yielding useful findings to integrate with clinical results, MR also has a predictive value relative to both the continuation of sports activity and the possible damage at the end of it.

[Gadolinium-DTPA dimeglumine administered orally for the study of the abdomen with magnetic resonance. Clinical evaluation and tolerance]. / Gadolinio-DTPA dimeglumina per uso orale nello studio dell'addome con risonanza magnetica. Valutazione clinica e della tollerabilità.

The tolerance to and the clinical value of a new positive oral contrast agent, Gd-DTPA-Dimeglumine, were evaluated. Ten healthy volunteers and 30 patients with abdominal and pelvic tumors were studied. Fourteen patients underwent MR scans only after cm administration, while 16 patients were examined both before and after. All images were obtained by means of a 1.5-T MR unit using SE and GE pulse sequences on the axial and coronal planes. Before the examination, a 1-mmol Gd-DTPA-Dimeglumine solution with mannitol and sodium citrate diluted in 1L of water was administered to all patients in 100-150-ml fractions. The product was well tolerated and its taste was good; adverse reactions were uncommon and mild. The solution was stable during gastrointestinal tract transit. Bowel signal was hyperintense on both GE and SE pulse sequences, which clearly demonstrated the bowel lumen distinguishing it from the surrounding fatty tissue and depicting even the smallest vascular structures. Moreover, from a clinical point of view, the hepatic, pancreatic and colic borders were better demonstrated in both normal and pathologic conditions.