Distinct spatiotemporal patterns for disease duration and stage in Parkinson’s disease

Purpose

To assess correlations between the degree of dopaminergic depletion measured using single-photon emission computed tomography (SPECT) and different clinical parameters of disease progression in Parkinson’s disease (PD).

Methods

This retrospective study included 970 consecutive patients undergoing ¹²³I-ioflupane SPECT scans in our institution between 2003 and 2013, from which we selected a study population of 411 patients according to their clinical diagnosis: 301 patients with PD (69.4?±?11.0 years, of age, 163 men) and 110 patients with nondegenerative conditions included as controls (72.7?±?8.0 years of age, 55 men). Comprehensive and operator-independent data analysis included spatial normalization into standard space, estimation of the mean uptake values in the striatum (caudate nucleus + putamen) and voxel-wise correlation between SPECT signal intensity and disease stage as well as disease duration in order to investigate the spatiotemporal pattern of the dopaminergic nigrostriatal degeneration. To compensate for potential interactions between disease stage and disease duration, one parameter was used as nonexplanatory coregressor for the other.

Results

Increasing disease stage was associated with an exponential decrease in ¹²³I-ioflupane uptake (R ² ?=?0.1501) particularly in the head of the ipsilateral caudate nucleus (p?<?0.0001), whereas increasing disease duration was associated with a linear decrease in ¹²³I-ioflupane uptake (p?<?0.0001; R ² ?=?0.1532) particularly in the contralateral anterior putamen (p?<?0.0001).

Conclusion

We observed two distinct spatiotemporal patterns of posterior to anterior dopaminergic depletion associated with disease stage and disease duration in patients with PD. The developed operator-independent reference database of 411 ¹²³I-ioflupane SPECT scans can be used for clinical and research applications.

     

Anoperineal disease in Hidradenitis Suppurativa : MR imaging distinction from perianal Crohn’s disease

Objective

To determine whether Hidradenitis suppurativa (HS)-related anoperineal disease can be distinguished from Crohn’s disease (CD) using MRI.

Methods

Pelvic MRI of 23 HS and 46 CD patients with anoperineal disease between 2007 and 2014 were independently analysed by two radiologists. For diagnosis of HS, sensitivity, specificity and positive likelihood ratios (LRs) of inflammatory features and of their anatomical distribution were calculated and compared to those of CD.

Results

In HS, fistulae were less present (P?=?.033) and less frequently involved the sphincters (P?=?.001) than in CD. Granulomas were more frequent (P?=?.0005). For anterior/inguinal and posterior localizations, sensitivity, specificity and LR for diagnosis of HS were 70% (49.1, 84.4), 87% (74.3, 93.9), 5.3 (2.41, 11.79) and 57% (36.8, 74.4), 93% (82.5, 97.8), 8.67 (2.74, 27.41), respectively. Combination of signs including posterior involvement, absence of rectal wall thickening and bilaterality of features yielded specificity of 100% (95% CI: 92.3–100) for HS.

Conclusion

Although MRI presentations of anoperineal disease may overlap between CD and HS, specific diagnosis of HS is possible with a combination of three features: absence of features’ predominance in perianal area, absence of rectal wall thickening and bilaterality of features.

Key Points

? Spectrum of MRI features of HS-related anoperineal disease is wide. ? Specific diagnosis of HS-related anoperineal disease is possible using MR imaging. ? A combination of three MR signs allows distinction between HS and CD.

     

Myonecrosis of Behcet’s disease

Behcet’s disease is an inflammatory disease of unknown cause characterized by intermittent episodes of acute inflammation manifested by oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. We report a rare case of myonecrosis associated with Behcet’s disease. Myonecrosis of Behcet’s disease can mimic soft tissue abscess and therefore awareness of this entity in the appropriate clinical setting is important for initiation of appropriate and timely treatment.     

Heart disease vis-à-vis trauma

Virtually all forensic experts deal not only with criminal, suspicious, accidental and suicidal deaths, but are also confronted with a wide range of deaths where a significant natural element is revealed at autopsy. The assaulted victim that dies suddenly or unexpectedly from a stroke during or immediately succeeding the receipt of some non-fatal injuries, or otherwise suffers a clinically unexplained death, can pose far greater difficulties over causation than a gun-shot or a stabbing. This paper presents an analysis of the problem and an approach for determining the cause of death in cases of concurrent trauma with heart disease, and in cases with a substantial natural element of disease but exclusion of trauma. Relevant cases with history, autopsy findings, histopathological findings and toxicological findings are presented in order to illustrate the issue from a practical angle.     

Atypical vertebral Paget’s disease

A 40-year-old Mauritanian man consulted for back pain. A computed tomography of the spine showed patchy sclerosis of the fifth and seventh thoracic vertebral bodies with normal neural arch of T5 and sclerosis and hypertrophy of the neural arch of T7, as well as diffuse sclerosis of the T11 vertebral body with a normal neural arch. At MRI, low signal-intensity on T1-weighted images and high signal-intensity on T2-weighted images involved the whole T5 and T7 vertebrae and the vertebral body of T11. Working diagnoses included metastatic disease and lymphoma, and a biopsy of T7 and then T11 was carried out. Both showed pathological findings very suggestive of Paget’s disease. Since CT is usually the more specific radiological examination in vertebral Paget’s disease, we thought it could be useful to report this atypical CT presentation (patchy sclerosis of the vertebral body without diffuse bone texture changes and isolated involvement of the vertebral body) of vertebral Paget’s disease.     

Neuro-Behçet disease mimicking brain tumor

Neuro-Behcet disease is one of the clinical forms of Behcet disease. We report a case of neuro-Behcet disease mimicked a brain tumor. This case was initially considered as a brain tumor from mass lesion with edema at left basal ganglia on radiologic images. The lesion, however, was not neoplasia by pathologic diagnosis. By using steroid therapy, the size had been markedly shrunken on the follow-up images. The clinical symptoms were also improved.     

Kimura’s disease of the elbows

Kimuras disease rarely involves a location outside the head and neck. We report a case of Kimuras disease in a young Asian man involving both elbows. Magnetic resonance imaging showed a soft-tissue mass of irregularly infiltrative strands in the subcutaneous fat accompanied with medial epitrochlear lymphadenopathy. The MRI appearance is described and the entity is briefly reviewed.     

Giant-cell-rich pseudotumour in Paget’s disease

Paget’s disease (PD) of the bone is a disorder of bone remodelling that may be polyostotic or monostotic. Although development of a sarcoma in PD is well-recognised, it is less well recognised that pseudosarcomas in bone and soft tissue can also arise in this condition. In this report we document the case of a large giant-cell-rich pseudotumour that developed in the tibia and overlying soft tissues in a case of polyostotic PD. Bone and soft tissues were highly vascular and contained abundant haemorrhage with focal areas of new bone formation and a diffuse infiltrate of osteoclastic giant cells. The lesion has not recurred or produced metastases 3 years after removal. Clinicians should be aware that a benign giant-cell-rich pseudotumour can develop in PD and that it needs to be distinguished from other giant-cell-rich tumours.     

Can patients with McArdle's disease run?

Patients with McArdle's disease commonly adopt a sedentary lifestyle. This sedentary behaviour, however, usually worsens the limited exercise capacity of these patients. Although eccentric muscle work can be associated with rhabdomyolysis, supervised eccentric training with gradually increasing loads has important advantages compared with conventional concentric work, particularly for patients with a poor cardiorespiratory system. We report the beneficial effects (particularly, increased VO(2peak) (from 14.6 to 30.8 ml/kg/min) and increased gross muscle efficiency (from 13.8% to 17.2%)) induced by a supervised aerobic training programme of 7 months duration including 3-4 running sessions (< or =60 min/session) per week in a 38-year-old patient. These preliminary data suggest the potential therapeutic value of this type of exercise in these patients.     

Behçet's disease involving the breast

Beh?et's disease is a vasculitis of unknown origin that was traditionally defined by oral and genital ulcers and uveitis. We describe a case of a patient with a diagnosis of Beh?et's syndrome who presented a palpable lesion in the right breast with inflammatory signs. X-ray findings posed a differential diagnosis between tumoral and inflammatory pathology. The pathological findings confirmed a small-vessel vasculitis. We found two reports of breast involvement by this disease in the literature. Our patient was studied by mammogram and sonogram which together with clinical history are important to prevent delay in diagnosis and unnecessary therapeutic procedures.     

Ultrasonographic evaluation of peyronie’s disease

High-resolution ultrasonography was performed on 58 patients with Peyronie’s disease and on 6 healthy men; in 3 patients the examination was repeated during medical treatment for a follow-up period of 4 months. Peyronie’s plaques always corresponded to abnormal echographic findings. In 54/58 cases (93%) they appeared as hyperechoic lesions. In 4 cases (7%) the plaques were hypoechoic and corresponded to a localized widening of the pericavernous tissues: this condition was observed more frequently in the earliest stages of the disease. There was good agreement between the palpable size of the plaques and their size measured by ultrasonography, with some degree of clinical overestimation of the smallest lesions. Calcified mature plaques corresponded to dense hyperechoic lesions with acoustical shadowing. High-resolution ultrasonography is proposed as a first-line diagnostic approach and as a reliable follow-up examination for Peyronie’s disease. The possibility of differentiating calcified plaques from the more recent ones is helpful to allow a correct choice of medical or surgical treatment.     

The spine in Paget’s disease

Paget’s disease (PD) is a chronic metabolically active bone disease, characterized by a disturbance in bone modelling and remodelling due to an increase in osteoblastic and osteoclastic activity. The vertebra is the second most commonly affected site. This article reviews the various spinal pathomechanisms and osseous dynamics involved in producing the varied imaging appearances and their clinical relevance. Advanced imaging of osseous, articular and bone marrow manifestations of PD in all the vertebral components are presented. Pagetic changes often result in clinical symptoms including back pain, spinal stenosis and neural dysfunction. Various pathological complications due to PD involvement result in these clinical symptoms. Recognition of the imaging manifestations of spinal PD and the potential complications that cause the clinical symptoms enables accurate assessment of patients prior to appropriate management.     

Comparison of sonographically measured bowel wall vascularity, histology, and disease activity in Crohn’s disease

The purposes of this study was to provide a retrospective comparison of semiquantitatively measured bowel wall vascularity by power Doppler sonography, endoscopic-histopathological biopsy findings, and disease activity in patients with confirmed Crohn’s disease. Thirty-two out of 1,332 patients with histologically confirmed Crohn’s disease (18 female, 14 male; mean age 38.8 years) met the inclusion criteria: ileocolonoscopy with biopsy and power Doppler sonographic determination of bowel wall vascularity with assessment of disease activity within a period of 5 days. Sonographic determination of bowel wall vascularity was based on a semiquantitative score. Endoscopic bowel wall biopsy specimens were assessed using a self-developed inflammation score and the disease activity was calculated using Crohn’s disease activity index (CDAI). A significant association (p < 0.05) was shown for results of histology and bowel wall vascularity in the terminal ileum (κ = 0.66; sensitivity 95%; specificity 69%). There was no observed association between CDAI and histology, although there was an association between CDAI and bowel wall vascularity (sensitivity 82%). Increased bowel wall vascularity in the terminal ileum measured by power Doppler ultrasound reflects inflammatory activity in histologically examined bowel wall. Power Doppler ultrasound may be able to monitor activity changes of the bowel wall determined by pharmaceutical treatment. B. H. Drews and T. F. E. Barth contributed equally.     

Peripheral sympathetic dysfunction in patients with Parkinson’s disease without autonomic failure is heart selective and disease specific

The study was undertaken to investigate by means of iodine-123-labelled metaiodobenzylguanidine (MIBG) scintigraphy the peripheral sympathetic function in patients with Parkinson’s disease (PD) without autonomic failure and in patients with related neurodegenerative diseases with parkinsonism. Seventy patients (33 men and 37 women, mean age 63±9.7 years) with parkinsonism and ten control subjects underwent MIBG scintigraphy. Of these 70 patients, 41 were diagnosed as having idiopathic PD, 9 multiple system atrophy (MSA), 6 progressive supranuclear palsy (PSP) and 2 corticobasal degeneration (CBD); the remaining 12 were diagnosed as having neurodegenerative disease with parkinsonism (P-nism) that did not meet the diagnostic criteria of any specific disease. Cardiac planar and tomographic imaging studies and subsequent whole-body imaging were performed 20 min and 3 h after the injection of 111 MBq MIBG. The early MIBG heart to mediastinum (H/M) ratio in PD (1.61±0.29) was significantly lower than that in the control group (2.24±0.14, P<0.01), P-nism (2.15±0.31, P<0.01), MSA (2.08±0.31, P<0.05) and PSP (2.30±0.24, P<0.01). The delayed H/M ratio in PD (1.47±0.34) was also significantly lower than that in the control group (2.37±0.14, P<0.01), P-nism (2.13±0.38, P<0.01), PSP (2.36±0.36, P<0.01) and MSA (2.17±0.36, P<0.01). In patients with PD, early and delayed H/M ratios were significantly decreased in disease stages I, II and III (established using the Hoehn and Yahr criteria) as compared with control subjects, and there were no significant differences among the stages. Only PD showed a significantly higher washout rate (WR) than that in the control subjects (27%±8.0% vs 11%±4.2%, P<0.01). Early and delayed uptake ratios of the lung, parotid gland, thyroid gland, liver and femoral muscles in each of the patient groups were not significantly different from those in control subjects. Only the early and delayed uptake ratios of the lower leg muscles in MSA were significantly lower than those in the control group (P<0.05). In conclusion: In patients with PD without autonomic failure, only cardiac MIBG uptake was severely reduced in the earliest phase of the disease (stage I). Parkinsonian syndromes other than PD did not demonstrate significant reduction in MIBG uptake in any organs except for the lower legs in MSA. In patients with PD without autonomic failure, reduction in MIBG uptake occurs selectively in the heart; this is considered to be a specific finding for PD and useful for the differential diagnosis of the parkinsonian syndromes. Received 13 September and in revised form 29 December 1999