Two cases of orbital myositis with 67Ga accumulation in the orbits]

We reported two patients of orbital myositis with abnormal accumulation of 6 Ga in the bilateral orbits. The first patient, a 34-year-old man, developed bilateral lid swelling and external ophthalmoplegia. T1-weighted MRI of the bilateral orbits showed enlargement of the bilateral extraocular muscles. 67Ga scintigraphy also showed abnormal accumulation in the orbits. The second patient, a 64-year-old woman, developed diplopia caused by restricted abduction of the bilateral eyes. 67Ga scintigraphy showed abnormal accumulation in the bilateral orbits, in spite of no finding in the MRI of the extraocular muscles. Both cases were treated with 1 g of methylprednisolone per day for 3 days and subsequently 1 mg/kg per day of oral prednisolone, which resulted in improvement of opthalmoplegia and disappearance of the abnormal accumulation of 67Ga in the orbits. There is no report about orbital myositis with abnormal accumulation of 67Ga in the orbit. Our cases suggest that 67Ga shows the activity of orbital myositis and can be useful for diagnosis, especially in case of no abnormality in MRI study.     

Camptocormia in Parkinson's disease mimicked by focal myositis of the paraspinal muscles.

We report on a 63-year-old man with idiopathic Parkinson's disease who developed kyphosis and a severe forward flexion of the thoracolumbar spine. A typical feature was an increase during walking or standing and it completely disappeared in the supine position, mimicking the clinical phenomenon of camptocormia (bent spine). In addition to the abnormal posture, a weakness of the erector spinal muscles, local pain, reddening, and elevated temperature of the paraspinal muscles were evident. Creatine kinase was initially elevated, electromyography showed spontaneous activity and a myopathic pattern. Magnetic resonance imaging and bioptic examinations revealed a focal myositis of the paraspinal muscles. This case indicates that camptocormia can be mimicked by focal myositis of paraspinal muscles and must be included in the differential diagnosis, especially when additional symptoms as inflammatory signs or weakness are present.     

Specific matrix metalloproteinase expression in focal myositis: an immunopathological study

OBJECTIVES: The aim of our study was to investigate immunoexpression of matrix metalloproteinases MMP2, MMP7 and MMP9 in muscle specimens from patients with focal myositis (FM) vs polymyositis (PM) and dermatomyositis. MATERIALS AND METHODS: We studied muscle biopsy samples from seven patients affected by FM; samples from five patients each with PM and dermatomyositis were studied as disease controls. RESULTS: MMP2 immunoreactivity was present in PM and dermatomyositis, MMP7 only in PM, and MMP9 in PM, dermatomyositis and FM. CONCLUSIONS: Our results confirm that increased MMP9 immunoreactivity in muscle fibres is a common feature of all inflammatory myopathies and suggest that MMP2 and MMP7 cannot be implicated in the inflammatory events of FM.     

Hepatitis C virus infection and myositis: a virus localization study

We report a case of myositis associated with chronic hepatitis C virus infection. Muscle biopsy and immunohistochemistry showed perifascicular atrophy, few necrotic and regenerating fibres, scarce perivascular infiltrates, deposits of immunoglobulin G, C3, fibrinogen and MAC in muscle vessel walls, and non-uniform expression of major histocompatibility complex-I antigens among muscle fibres. Hepatitis C virus NS3 antigen and hepatitis C virus RNA were detected in infiltrating cells but not within muscle fibres or endothelial cells. Our findings suggest that humoral-mediated immune mechanisms, not directly related to hepatitis C virus infection of muscle structures, may sustain the local inflammatory reaction in this patient.     

Nuclear localization of valosin-containing protein in normal muscle and muscle affected by inclusion-body myositis

Inclusion-body myopathy with Paget's disease and frontotemporal dementia (IBMPFD) is a disease of muscle, bone, and brain that results from mutations in the gene encoding valosin-containing protein (VCP). The mechanism of disease resulting from VCP mutations is unknown. Previous studies of VCP localization in normal human muscle samples have found a capillary and perinuclear distribution, but not a nuclear localization. Here we demonstrate that VCP is present in both myonuclei and endothelial cell nuclei in normal human muscle tissue. The immunodetection of VCP varies with acetone or paraformaldehyde fixation. Within the nucleus, VCP associates with the nucleolar protein fibrillarin and Werner syndrome protein (Wrnp) in normal and IBMPFD muscle. In patients with inclusion-body myositis (IBM), normal nuclear localization is present and some rimmed vacuoles are lined with VCP. These findings suggest that impairment in the nuclear function of VCP might contribute to the muscle pathology occurring in IBMPFD.     

Dropped-head syndrome due to steroid responsive focal myositis: a case report and review of the literature

Isolated severe weakness of the paraspinal musculature, either at the cervical level leading to a "dropped head syndrome" or at the thoracic level leading to a "bent-spine syndrome", is a rare disorder. Etiologically it may be present in a variety of neurological diseases including Parkinson's disease, multiple system atrophy, neuromuscular or motor neuron diseases, as well as non-inflammatory, inflammatory, dystrophic or metabolic myopathies. We present a previously healthy 74-year-old man with a 2-month history of progressive difficulty in lifting his chin off his chest. Magnetic resonance imaging and skeletal muscle biopsy revealed an isolated myositis of the neck extensor and trapezius muscles, which responded well to steroid therapy. This case and other rare reports obtained from a systematic review of the literature indicate that in a subgroup of patients "dropped head syndrome" or "bent spine syndrome" is caused by a myositis, which emphasizes the necessity to obtain a MRI examination, as well as a muscle biopsy to diagnose this potentially treatable disease.     

A case of isolated focal myositis successfully treated by arterial injection of prednisolone--its findings by CT scan and MRI

A 25-year-old woman with a 2 years history of painful enlargement of the left calf visited our clinic because of ache in the left calf which became severe enough to interfere her walk. She had no history of trauma to the calf, and family history was negative for muscle disease. Physical examination showed an enlarged left calf muscle with pain which was evoked by stretching of the Achilles tendon. There was no muscle weakness or neurological abnormality. Laboratory tests were all within normal limits, including erythrocyte sedimentation rate and serum enzymes such as creatine kinase and aldolase. The CT scan revealed an isolated round-shaped low density area in the left calf muscle, which showed up as a markedly high signal intensity on T2 weighted images of MRI. The lesion was confirmed to be the lateral head of the gastrocnemius muscle. The specimen obtained from the affected muscle demonstrated degenerative and inflammatory changes together with excess fibrosis. There was no sarcoid lesion or malignancy. From the above results, the present case was thought to have isolated focal myositis. Prednisolone 80 mg (40 mg, twice, at 4-day intervals) was given to the left femoral artery without any trouble. Thereafter the patient became free from pain and difficulty in walking, and the lesion demonstrated by CT scan disappeared in 15 days. However, the high signal intensity on T2 weighted images faintly remained until the 54th day after the treatment. In summary, MRI is more sensitive than CT scan for detection of isolated focal myositis, which might be successfully treated by arterial injection of prednisolone within a short period than any other conventional methods.     

Clinical, pathological and magnetic resonance imaging features of focal myositis: report of three cases

Focal myositis is a rare idiopathic form of inflammatory myositis involving a single muscle. The presentation is often as a soft tissue pseudotumour. Magnetic resonance imaging (MRI) is a useful noninvasive imaging modality to demonstrate focal nature of the lesion. We describe three patients presenting as pseudotumour of the lower leg. MRI showed hyperintense signals in the involved muscle.     

Detection and localization of focal cortical dysplasia by voxel-based 3-D MRI analysis

PURPOSE: Focal cortical dysplasia (FCD) is a frequent cause of partial epilepsy. Its diagnosis by visual evaluation of magnetic resonance images (MRIs) remains difficult. The purpose of this study was to apply a novel automated and observer-independent voxel-based technique for the analysis of 3-dimensional (3-D) MRI to detect and localize FCD. METHODS: The technique was based on algorithms of the SPM99 software and included the spatial normalization of 3-D MRI data sets to a common stereotaxic space and the segmentation of cortical grey matter. The resulting data sets represented grey-matter density maps where each voxel encoded the grey-matter concentration at the corresponding position in the original MRI. A normal database was set up by calculating and averaging the grey-matter density maps of 30 healthy volunteers. The MRI data sets of seven epilepsy patients with FCD were evaluated retrospectively for dysplastic lesions by voxelwise subtraction of the mean grey-matter density map of the normal database and searching automatically for local and global maxima in the resulting data set. RESULTS: In all patients, the results of voxel-based 3-D MRI analysis corresponded both to the location of the dysplastic lesions in conventional MRI and to seizure semiology and EEG findings. In one case, surgery was performed, and the diagnosis FCD was supported by histology. CONCLUSIONS: The technique of voxel-based 3-D MRI analysis and comparison with a normal database seems to provide a valuable additional screening tool for the detection of FCD.     

Orbital myositis induced by alendronate: A case report

Background and purpose

Bisphosphonates are widely used, notably for osteoporosis treatment. Their common side effects are well known. However, they can trigger less common effects such as orbital inflammation. Here, the case is reported of an orbital myositis triggered by alendronate.

Methods

This is a case report at an academic medical center. An orbital magnetic resonance imaging scan, a thoraco-abdominal computed tomography scan and blood sample analyses were performed.

Results

A 66-year-old woman treated by alendronate for her osteoporosis was investigated. She developed an orbital myositis after the first intake. Neurological examination revealed a painful diplopia with decreased downward and adduction movements of the right eye and edema of the upper eyelid. Orbital magnetic resonance imaging showed an orbital myositis of the right eye. No other cause of orbital myositis was found than the alendronate intake. After alendronate arrest and a short course of prednisone, the symptoms resolved.

Conclusion

This case highlights that alendronate can cause an orbital myositis whose early diagnosis is of major importance because it is a treatable side effect.     

Cortical activation pattern during saccadic eye movements in humans: localization by focal cerebral blood flow increases.

Regional cerebral blood flow (rCBF) was measured in human subjects during saccadic eye movements by a 254-channel dynamic gamma camera. Focal rCBF increases were repeatedly observed in an area within the middle precentral and premotor regions which corresponds to the frontal eye field in humans. Our findings suggest that this region is localized between the "face" and "hand" areas in the precentral gyrus and extends anteriorly out of the primary motor strip into the adjacent premotor zone. In addition, saccades were associated with focal blood flow increases in regions corresponding to the frontal supplementary motor area and to the posterior temporooccipital visual association cortex. Similar changes in patterns of rCBF occurred during contralateral and ipsilateral horizontal saccades and also during vertical saccades, and did not differ between the right and left hemispheres. Focal rCBF increases were observed in the frontal eye field during several additional test procedures including ocular fixation and visual perception of a nonmoving target, auditory stimulation with closed eyes, and reading. It is presumed that these focal flow increases reflect increased localized neuronal activity and metabolic rate and therefore permit visualization of the cortical activation pattern associated with saccadic eye movements in humans.     

Pre-treatment of adrenomedullin suppresses cerebral edema caused by transient focal cerebral ischemia in rats detected by magnetic resonance imaging

Recent studies suggest the protective effects of adrenomedullin (AM) on ischemic brain damage. The present study was aimed at investigating the effects of AM and its receptor antagonist, AM_22-52, on ischemia-induced cerebral edema and brain swelling in rats using magnetic resonance imaging. Rats were subjected to 60 min of middle cerebral artery occlusion (MCAO) followed by reperfusion. Intravenous injection of AM (1.0 μg/kg), AM_22-52 (1.0 μg/kg), or saline was made before MCAO. Effects of AM injection just after reperfusion were also investigated. One day after ischemia, increases in T₂-weighted signals in the brain were clearly observed. Total edema volume, as well as brain swelling, was greatly and significantly reduced by pre-treatment of AM (reduced by 53%). Extent of brain swelling was significantly correlated with the volume of cerebral edema. The protective effect of AM against edema was more clearly observed in the cerebral cortex (reduced by 63%) than the striatum (reduced by 31%). Increased T₂ relaxation time in the cortex was recovered partially by pre-treatment of AM. Post-treatment of AM had no effects. Pre-treatment of AM_22-52 tended to exacerbate the edema. In another line of experiment, cocktail administration of AM with melatonin, a pineal product having neuroprotective potential as a free radical scavenger, failed to enhance the protective effects of AM alone. The present study clearly suggests the prophylactic effects of AM against cerebral edema, especially the cortical edema, in a rat stroke model.     

S-1 radiculopathy as a possible predisposing factor in focal myositis with unilateral hypertrophy of the calf muscles.

Associated with chronic S-1 radiculopathy, a 44-year-old man developed unilateral hypertrophy of the calf muscles. Electromyography revealed neurogenic alterations in the corresponding limb compatible with S-1 radiculopathy. In addition, MR-tomographic and bioptic findings were consistent with a focal inflammatory myopathy of the enlarged right gastrocnemius muscle. Predisposing factors for the localisation of a focal myositis are unknown. This case report highlights the diagnostic difficulties in distinguishing focal myositis and denervation hypertrophy following S-1 radiculopathy or secondary inflammation related to denervation. We consider the possibility that in our case the inflammatory process might have been triggered by electromyographically proven chronic denervation related to radiculopathy.     

Clinical evidence for the utility of movement compensation algorithm in magnetoencephalography: Successful localization during focal seizure

A movement compensation (MC) algorithm may help to evaluate seizure focus in magnetoencephalography despite patient movement. We report a boy whose ictal MEG focus was localized to the same sublobar region before and after head turning when MC was applied, but which was erroneously localized to a different area without MC. This study provides the first clinical evidence for utility of MC in magnetoencephalography for localizing focal seizures.     

Inclusion body myositis: the mumps virus hypothesis

The resemblance of the filamentous inclusions in inclusion body myositis (IBM) to mumps virus nucleoproteins and the report of immunoreactivity of the inclusions for mumps virus antigens have implicated the mumps virus in the etiology of IBM. We tested the mumps virus hypothesis by in-situ hybridization with a cDNA probe specific for the mumps virus nucleocapsid gene, and immunocytochemically with antibodies against "soluble" and "viral" mumps antigens. The tests were performed on muscle specimens (IBM, 20; acid maltase deficiency, 4; chloroquine myopathy, 2; nonweak control subjects, 5) and mumps virus-infected and uninfected HEp-2 cells. The in-situ hybridization study showed a strong specific signal in the infected HEp-2 cells but no specific signal in IBM, other myopathies, or nonweak control subjects. The immunocytochemical study showed specific binding of the antimumps antibodies to the infected HEp-2 cells but demonstrated only nonspecific binding of these antibodies around rimmed vacuoles in IBM, acid maltase deficiency, and chloroquine myopathy. These studies cast doubt on the mumps hypothesis of IBM.