Aortitis requiring aortic repair associated with glaucoma, thyroiditis, glaucoma, and neuropathy: case report.

Aortitis may be due to infectious and non-infectious causes. We observed aortitis, associated with glaucoma, thyroiditis, pericarditis, pleural effusion and neuropathy in a 63-years old woman. Despite antibiotic therapy, inflammatory signs persisted and resolved only after initiation of glucocorticoid therapy. Increasing aortic ectasia necessitated resection of the ascending aorta and implantation of a Vascutek 30 mm prosthesis. Histologically a granulomatous aortitis was diagnosed. Since all other possible causes were excluded, an immunological mechanism of the aortitis is suspected and possible triggering factors are discussed.

Pneumosinus dilatans and hypercalcification of the falx and ligamentum petroclinoideum in myotonic dystrophy 1.

OBJECTIVES: Pneumosinus dilatans (PSD) is a rare condition characterized by abnormally enlarged aerated sinuses, but normal thickness and preserved integrity of the sinus walls. PSD occurs most commonly in young men. Its etiology remains elusive but has been associated with various conditions, such as hydrocephalus, trauma, Dyke-Davidoff syndrome, meningocele, arachnoidal cyst, meningioma, neurofibromatosis 2, and neurolemmoma. PSD has not been reported in myotonic dystrophy type 1 (MD1). CASE REPORT: We present a 43-year-old man with MD1 because of a CTG-expansion of 1333 repeats in the DMPK gene. A computed tomography scan of the cerebrum, carried out for assessment of central nervous system involvement in MD1, surprisingly demonstrated PSD. Because PSD was asymptomatic no surgical treatment was recommended to the patient. Whether PSD was a manifestation of MD1 or a mere coincidence remains elusive. CONCLUSION: This case shows that PSD may occur together with MD1. Whether this association is causal or coincidental remains to be elucidated. It also remains speculative whether PSD contributes to the facial dysmorphism frequently present in MD1 patients. If PSD becomes symptomatic surgical intervention should be considered.

Cerebellar nocardiosis and myopathy from long-term corticosteroids for idiopathic thrombocytopenia.

Infection of the central nervous system with Nocardia sp. usually manifests as supratentorial abscesses. Supratentorial and cerebellar abscesses from infection with Nocardia sp. following immunosuppression with long-term corticosteroids for idiopathic thrombocytopenia (ITP) have not been reported. An 83 years-old, human immunodeficiency virus (HIV)-negative, polymorbid male with ITP for which he required corticosteroids since age 53 years developed tiredness, dyspnoea, hemoptysis, abdominal pain, and progressive gait disturbance. Imaging studies of the lung revealed an enhancing tumour in the right upper lobe with central and peripheral necrosis, multiple irregularly contoured hyperdensities over both lungs, and right-sided pleural effusions. Sputum culture grew Nocardia sp. Neurological diagnostic work-up revealed dysarthria, dysphagia, ptosis, hypoacusis, tremor, dysdiadochokinesia, proximal weakness of the lower limbs, diffuse wasting, and stocking-type sensory disturbances. The neurological deficits were attributed to an abscess in the upper cerebellar vermis, myopathy from corticosteroids, and polyneuropathy. Meropenem for 37 days and trimethoprime-sulfamethoxazole for 3 months resulted in a reduction of the pulmonary, but not the cerebral lesions. Therefore, sultamicillin was begun, but without success. Long-term therapy with corticosteroids for ITP may induce not only steroid myopathy but also immune-incompetence with the development of pulmonary and cerebral nocardiosis. Cerebral nocardiosis may not sufficiently respond to long-term antibiotic therapy why switching to alternative antibiotics or surgery may be necessary.

Risk of stroke and thrombus formation from delay incontinence of a PLAATO-device in Friedreich ataxia.

We present the case of a 76-year-old female with suspected Friedreich ataxia, in whom leakage and thrombosis of a percutaneously implanted left atrial appendage occluder (PLAATO) was observed 2 years after implantation. Because of late developing leakages and thrombi, regular transesophageal echocardiographic examinations should be carried out in patients with occluded left atrial appendages. In view of these complications and the potential important hemodynamic role of the left atrial appendage, the benefit of its occlusion is questionable, and the indications of PLAATO should be strongly reconsidered.

Cerebrovascular events in left ventricular hypertrabeculation/noncompaction with and without myopathy.

UNLABELLED: Little is known about frequency, morphology, and causes of stroke in patients with left-ventricular hypertrabeculation/noncompaction (LVHT). Aim of this study was to find out how many LVHT-patients suffer from stroke, which abnormalities are found on cerebral imaging, and if stroke LVHT-patients differ from non-stroke LVHT-patients with regard to cardiac and neuromuscular abnormalities. METHOD AND MATERIALS: Records of all included patients were reviewed for stroke diagnosed upon history, clinical examination, or cerebral CT/MRI (CCT, cMRI). RESULTS: Among 104 LVHT-patients included, stroke was diagnosed in 16 (15%). Of these two had a transitory ischemic attack and one a prolonged ischemic deficit. Among 36 patients undergoing a CCT (n=32), cMRI (n=9), or both (n=5) territorial stroke was found in 7, lacunar stroke in 2, and intracerebral bleeding in 1. Other imaging findings were atrophy (n=7), microangiopathy (n=4), leucencephalopathy (n=3), basal-ganglia-calcification (n=2), or non-specific hypodensities (n=2). Imaging studies without vascular lesions were found in 2 patients. At least one classical risk factor for stroke was found in 15 patients. Stroke-patients did not differ from non-stroke-patients regarding cardiac or neuromuscular findings. CONCLUSIONS: If systematically looked for, stroke is a frequent feature of LVHT. A causal relation between LVHT and stroke, however, is rather the exception than the rule.

Importance of T2*-weighted gradient-echo MRI for diagnosis of cortical vein thrombosis.

We examined six patients with isolated venous thrombosis (n = 2), or venous thrombosis combined with sinus thrombosis (n = 4) (CVT). The clinical symptoms were non-specific (acute cephalea, paresis, epileptic seizure, progressive speech disorder). All examinations were performed on a 1.5 T system (Magnetom Symphony, Siemens, Erlangen, Germany), maximum gradient field strength 30 mT/m, minimal gradient rise time 450 micros, according to the following protocol: Transverse T2-weighted turbo spin-echo (TSE), fluid attenuated inversion recovery (FLAIR), T1-weighted spin-echo (SE), before and after administration of contrast medium, T2*-weighted conventional gradient-echo (GRE), T2*-weighted spin-echo echo planar imaging (SE EPI), both without and with diffusion weighting as well as two-dimensional (2D) venous time-of-flight (TOF) MRA. The venous thromboses were best detectable in the T2*-weighted conventional GRE sequence in all patients. In two patients, the CVT was discernible only in this sequence. The sinus thrombosis was well discernible only in the T2*-weighted GRE sequence in only one case; in the remaining cases it was detectable only with difficulty. For these cases, other sequences such as SE, diffusion-weighted, or 2D-TOF-MRA sequence were superior. The T2*-weighted conventional GRE sequence was superior to the T2*-weighted SE EPI sequence in all patients. To sum up, it can be concluded, that T2*-weighted conventional GRE sequences are possibly the best method of detection of acute cortical vein thromboses. Therefore, it seems to be of benefit to integrate a T2*-weighted conventional GRE sequence into the MR-protocol for the diagnosis of isolated cortical vein thrombosis.