Computerized tomography and magnetic resonance muscle imaging in Miyoshi's myopathy

We describe clinical, pathological, and muscle imaging findings in a patient with an early adult-onset progressive muscular weakness in association with atrophy beginning in the legs and involving both gastrocnemi in particular. Muscle biopsy findings showed a severe dystrophic process with no vacuoles, consistent with Miyoshi's myopathy. Computerized tomography and magnetic resonance imaging scans were used to provide an ongoing permanent record of the various stages of the disease. © 1996 John Wiley & Sons, Inc.     

Diffusion-weighted magnetic resonance imaging is useful for assessing inflammatory myopathies

Introduction: Short tau inversion recovery (STIR) sequences in whole-body MRI are usually used for detecting muscle edema (ME) in inflammatory myopathies. We evaluated b-value 800 diffusion-weighted imaging (b800 DWI).Methods: Two radiologists independently and a consensus reader retrospectively reexamined 60 patients with inflammatory myopathies and 15 controls. For each participant, 78 muscles were analyzed with 3 sets of imaging acquisitions: T1-weighted (T1) turbo spin echo and STIR; T1 and DWI; and T1, STIR and DWI. Mean edema per patient was compared between sequences. Agreement was evaluated. Results: Diffusion-weighted imaging detected more ME compared with STIR (P < 0.001). Agreement between readers was better with both sequences (k = 0.94) than with b800 DWI (k = 0.89) or STIR (k = 0.84) alone. Discussion: Diffusion-weighted imaging is a valuable add-on for the study of inflammatory myopathies. Muscle Nerve 59:555–555, 2019     

Magnetic resonance imaging and computed tomography in Alzheimer's disease

Structural neuroimaging with CT and MRI plays an important role in the routine clinical investigation of dementia patients. One major purpouse of the technique is to exclude secondary, treatable dementia disorders. In this respect these techniques are of utmost importance. Another major purpose of these techniques is to differentiate AD from healthy controls and other dementias.
Although some research groups have reported a high sensitivity and specificity for diagnosing AD by measuring the volumes (with MRI) of structures in the temporal lobes, there are no unequivocal "radiological" markers (such as the size of the lateral ventricles or sulci) that can differentiate AD from controls or vascular dementia. There have been a large number of CT and MRI studies showing that the volumes of the temporal lobes or part of the temporal lobes, such as the hippocampal formation are reduced in AD, as compared to healthy subjects.
The role of white matter changes (WMH, leukoaraiosis) in the development of AD or as a diagnostic marker for AD is unclear. The degree of WMH has been shown to be strongly age dependent and there are contradictory data concerning its relation to cognitive decline. There is stronger agreement concerning the relationship between LA assessed with CT and cognitive functions than there is between WMH on MRI and cognitive functions.
Correlative studies with MRI, CT and SPECT or PET are few. Usually the sensitivity and specificity of diagnosing AD can be increased by combining two or more modalities. One other goal with this approach is to use one structural modality, such as MRI or CT as a template for functional images in order to obtain a higher accuracy in measurement of blood perfusion or glucose metabolism.     

Muscle magnetic resonance imaging shows distinct diagnostic patterns in Welander and tibial muscular dystrophy

OBJECTIVES: This is a report on a retrospective muscle magnetic resonance imaging (MRI) study on 11 patients affected by Welander distal myopathy (WDM) and 22 patients with tibial muscular dystrophy (TMD) carried out in order to define the pattern and characteristics of muscle involvement. RESULTS: WDM patients showed involvement of gastrocnemius, soleus, tibial anterior (TA) and extensor digitorum longus (EDL), as well as hamstrings and hip adductor muscles. TMD patients showed involvement of the TA and EDL muscles, and in some patients also hamstring and posterior compartment muscles of the legs. Some patients showed asymmetry of muscle involvement. CONCLUSION: We conclude that muscle MRI examination proved to be very useful in the determination of the exact pattern of muscle involvement in WDM and TMD. Clinical testing using the Medical Research Council scale is not sensitive enough to establish the pattern of muscle involvement in focal muscle diseases.     

Anterior interosseous nerve syndrome following peripheral catheterization: magnetic resonance imaging and electromyography correlation

Anterior interosseous nerve syndrome (AINS) has not been widely recognized as a possible complication following peripheral catheterization. Herein we present a retrospective review of patients with AINS over the last 5 years. Six cases were identified, 4 associated with catheterization. AINS may be a rare complication of catheterization. Magnetic resonance imaging (MRI) may serve as an adjunct diagnostic modality to conventional electromyography (EMG) and nerve conduction studies, especially in patients who are intolerant of pain.     

Needle electromyography and histopathologic correlation in myopathies

Introduction: The molecular mechanism of immune-mediated necrotizing myopathy (IMNM) remains unknown. Autophagy impairment, described in autoimmune diseases, is a key process in myofiber protein degradation flux and muscle integrity and has not been studied in IMNM. Methods: Muscle biopsies from patients with IMNM (n = 40), dermatomyositis (DM; 24), polymyositis (PM; 8), polymyositis with mitochondrial pathology (4), sporadic inclusion body myositis (8), and controls (6) were compared by immunohistochemistry. Results: The proportions of myofibers containing autophagy markers LC3b and p62 were higher in IMNM than in DM or PM and correlated with creatine kinase levels. In IMNM, compartmentalized LC3b puncta were located in regenerating and degenerating myofibers surrounded by major histocompatibility complex type II⁺ inflammatory cells. Several IMNM myofibers accumulated ubiquitin and misfolded protein. Discussion: The detection of LC3b⁺ or p62⁺ myofibers could be used in differentiating IMNM from PM. The identification of autophagy-modifying molecules potentially could improve patients’ outcomes. Muscle Nerve, 2019     

Magnetic resonance imaging of muscle injury and atrophy in glycolytic myopathies

Exertional muscle pain, contractures, recurrent rhabdomyolysis, and pigmenturia are common in certain muscle glycolytic disorders. However, the frequency, distribution, and long-term significance of these findings are poorly understood. First we performed magnetic resonance imaging (MRI) of the extremities as a screening test for the detection of muscle abnormalities incurred in activities of daily living in four patients with myophosphorylase deficiency (MPD) and three with muscle phosphofructokinase deficiency (PFKD). MRI findings of abnormal muscles detected upon screening were next compared with changes observed in a prospective study of muscle contractures involving the forearms of four of the patients (two MPD, two PFKD). Screening revealed abnormalities of proximal thigh muscles in three of seven patients, in two of whom (one MPD, one PFKD) a recent history of exertional myalgia coincided with increases in T1 and T2 estimates of isolated thigh muscles. In the third patient (PFKD), focal atrophy of the adductor magnus was present bilaterally. In prospective studies, focal areas of prolonged T1 and T2 appeared in the flexor digitorum superificalis in all four cases and in the flexor digitorum profundus in two cases. Serial imaging suggested that the onset of MRI abnormalities begins within 24 hours of contracture and persists for at least several days and possibly for much longer, with complete recovery apparently the rule. These cases suggests a high prevalence of focal muscle abnormalities in patients with glycolytic myopathies and show the potential of MRI to detect them.     

Magnetic resonance imaging in clinical psychiatry

Although psychiatric researchers have been quick to adopt magnetic resonance imaging (MRI) of the brain in their investigations, its clinical application has been slow to develop and most psychiatrists remain unaware of its potential advantages and disadvantages compared with CT scanning. In this article the procedures are compared and the potential advantages of MRI highlighted with the help of neuropsychiatric case histories. Clinical situations are then discussed in which a psychiatrist should consider ordering a MRI scan subsequent to or instead of a CT scan.     

Muscle CT, biopsy and EMG in diagnosis of neuromuscular diseases

The diagnostic value of EMG and muscle biopsy has been compared with muscle CT in 53 patients with neuromuscular diseases. CT concordance with clinical diagnosis was found in 62% of myopathies and was highest in Duchenne PMD and scapulo-peroneal myopathy and very low in metabolic and inflammatory myopathies. In neurogenic diseases muscle CT agreed with clinical diagnosis in 63% of patients: the highest concordance was found in acquired polyneuropathies.

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Sommario Il valore diagnostico dell' EMG e della biopsia muscolare viene confrontato con quello della TC muscolare in 53 pazienti affetti da malattie neuromuscolari. La TC muscolare concorda con la diagnosi clinica nel 62% delle miopatie, con un massimo nelle distrofie di Duchenne e nella miopatia scapolo-peroneale, mentre è di scarsa utilità nelle miopatie metaboliche ed infiammatorie. Nelle malattie neurogene la TC muscolare è in concordanza con la diagnosi clinica nel 63% dei casi, con un massimo nelle neuropatie acquisite.

     

Computer tomography and magnetic resonance imaging in cvanide poisoning

Summary A 29-year-old chemistry student took 50 ml of a 1% potassium cyanide solution (500 mg) in an attempted suicide. He became comatose, mydriatic, and was admitted to hospital in an apneic state. He woke up after 7 h and developed Parkinsonism in the following weeks. This regressed slowly during the 2 months after the poisoning apart from dysarthria, bradykinesia of the upper limbs, and very brisk monosynaptic reflexes. At 3 weeks after the intoxication, computerized tomography was largely normal, and there was CSF-dense hypodensity in both putamina after 5 months. Sharply delimited signal elevation in T2 corresponding to the two putamina was detected by magnetic resonance imaging 8 weeks and 5 months after ingestion of the poison.     

Thigh magnetic resonance imaging for the evaluation of disease activity in patients with idiopathic inflammatory myopathies followed in a single center

Introduction: In patients with idiopathic inflammatory myopathies (IIM), magnetic resonance imaging (MRI) has been proposed as a useful tool for diagnosis and follow‐up. It may identify muscle inflammation (edema) and fatty infiltration for evaluation of disease activity and damage. Little information is available on the role of MRI in assessment of large cohorts of adult patients with IIM. Methods: Fifty‐one patients underwent MRI of the thigh muscles, laboratory tests, and clinical evaluation, including Physician Global Assessment (PGA) of myositis activity and the Manual Muscle Test 8 (MMT8). Results: Muscle edema correlated significantly with creatine kinase values (P = 0.017) and PGA (P < 0.001). A significant correlation between edema and MMT8 values (P = 0.025) was observed when patients with muscle fatty infiltration were excluded. With respect to clinical diagnosis, the sensitivity of MRI was 92.3%, and specificity was 83.3%. Conclusions: MRI appears to provide additional information that complements clinical and biochemical examinations. Muscle Nerve 54 : 666–672, 2016     

The sensitivity of 18-fluorodopa positron emission tomography and magnetic resonance imaging in Parkinson''s disease

Parkinson's disease (PD) as the most important movement disorder is characterized by a progressive loss of nigral dopamine neurons and a subsequent degeneration within several other transmitter systems. Functional brain imaging with positron emission tomography (PET) and the radiotracer 18-fluorodopa (FDOPA) is capable to quantify the deficiency of dopamine synthesis and storage within pre-synaptic striatal nerve terminals. Therefore, FDOPA-PET allows the diagnosis of PD in early disease stages and the differentiation of clinically unclear cases from other movement disorders, e.g. essential tremor. Additionally, FDOPA-PET imaging permits the follow-up of disease progression, the assessment of medical and surgical PD therapy strategies with possible neuroprotective properties and the detection of pre-clinical disease in subjects at risk for the disorder. The classical domain of morphological magnetic resonance imaging (MRI) is the differentiation of symptomatic Parkinsonism from PD. However, recent advances in MRI data acquisition and analysis techniques demonstrated MRI to be also a valuable tool for detection of nigral pathology in PD and for differentiation of neurodegenerative disorders with atypical Parkinsonism.     

A case of psychotic disorder associated with a right temporal lesion: a special reference to magnetic resonance imaging and single photon emission computed tomography findings

A case of psychotic disorder with a right temporal lesion was reported. The patient, a 19 year old male, who underwent a brain surgery to remove the trigeminal Schwannoma, occupying from the right cerebellopontine angle to the right middle cranial fossa. One year postoperatively, he presented with a psychotic disorder, including auditory hallucinations, delusions of persecution and reference, thought hearing, thought insertion and passive experiences. T1-weighted images on magnetic resonance imaging (MRI) demonstrated a low intensity signal area in the right temporal cortex and white matter. T2-weighted images demonstrated a high intensity signal within the same region. Single photon emission computed tomograghy (SPECT) demonstrated a severe low perfusion corresponding to the region in which the MRI demonstrated the abnormalities. The clinical and neuroimaging studies of this case suggest that psychotic disorder may occur in association with a right temporal lesion and MRI and SPECT are useful to evaluate an organic basis for the psychotic disorder.     

Stereotactic co-registration of magnetic resonance imaging and histopathology in post-mortem multiple sclerosis brain

A number of groups have examined the pathological substrate of signal changes on magnetic resonance imaging (MRI) in post-mortem (PM) brain of patients with multiple sclerosis (MS). Such studies will benefit from using a standardized method to reliably co-register regions of interest on MRI and tissue specimens. We investigated the usefulness of a stereotactic navigation system for this purpose. We also addressed the sensitivity of different standard MRI sequences with regard to lesion conspicuity in PM MS brain. Post-mortem brains of eight patients with MS were studied. Formalin-fixed coronal slices were placed in the head frame of a stereotactic system. Proton density-, T2-weighted and fast fluid-attenuated inversion recovery (FLAIR) scans were obtained and visually matched with scans that had been previously obtained on the same, but fresh, specimens. Guided by the stereotactic target points, the dissection of the fixed specimens was performed. After processing the blocks for embedding in paraffin, sections were stained with haematoxylin-eosin and Luxol fast blue. T2-weighted MRI of fixed brain revealed 24 areas suspected to be MS lesions, all of which were confirmed histologically. Three of these lesions were not visible on macroscopic inspection. There were 14 additional hyperintensities on T2-weighted or FLAIR MRI of the fresh specimens, five of which did not correlate to MS lesions histologically. Stereotactic navigation is a useful approach to co-register MRI and histopathology in PM brain of MS patients and may improve the precision of MRI-guided sampling of tissue specimens. Standard T2-weighted MRI appeared to be the single most useful approach for lesion detection in fresh and fixed specimens.     

磁共振扩散张量成像技术在外伤性视神经病中应用研究

目的 探讨磁共振扩散张量成像技术在评价外伤性视神经病中的作用. 方法选择6例单侧外伤性视神经病患者,采用磁共振扩散张蛩成像技术检测双侧视神经.应用DTV2和Volume One 1.44软件测量视神经的部分各向异性值、平均表观扩散系数,并进行损伤眼和正常眼的自身对比;应用神经白质纤维柬追踪技术显示视神经的空间完整性和连续情况.结果 所有患者眶内段视神经显示良好,2例患者双侧管内段显示欠佳.6例患者损伤眼眶内段视神经部分各向异性平均值较正常眼明显下降,分别为0.244±0.067和0.452±0.053,差异有统计学意义(P=0.000);平均表观扩散系数平均值较正常眼明显上升,分别为(1.417±0.121)×10<'-3>mm<'2>/s和(1.087±0.118)×10<'-3>mm<'2>/s,差异有统计学意义(P=0.003).4例患者可以获得用于评估双侧视神经空间连接情况的扩散张量纤维束成像图,损伤眼和正常眼之间存在明显差异.结论磁共振扩散张量成像技术能够为评估外伤性视神经病的视神经纤维病理情况提供有价值的信息.     

Cranial magnetic resonance imaging in genetically proven myotonic dystrophy type 1 and 2

Abstract. Cranial magnetic resonance imaging (MRI) in 19 German patients with genetically proven myotonic dystrophy Type 1 (DM1, n = 10) or Type 2 (DM2, n = 9) showed pathological findings consisting of white matter lesions (WML) and/or brain atrophy in 9/10 DM1 and 8/9 DM2 patients. Anterior temporal WML (ATWML) were exclusively seen in DM1 patients. Our findings indicate a high frequency of central nervous system (CNS) involvement in both disorders. However, temporopolar pathology, previously associated with intellectual dysfunction, seems to be restricted to DM1.