[Ocular manifestations of Churg-Strauss syndrome: review article and case report]. / Augenmanifestationen bei Churg-Strauss-Syndrom: Übersichtarbeit und Fallvorstellung.

This paper summarizes the possible ocular manifestations of Churg-Straus syndrome (CCS) from the literature and presents an unusual case report from routine clinical practice with conjunctival granuloma, orbital pseudotumor and choroidal folds. The CSS is an ANCA-associated granulomatous vasculitis which can be manifested in various organs and represents a life-threatening situation for the patient. Ocular manifestations are rare and can spread to all segments of the eye and orbit. The most frequent forms of ocular involvement described in the literature are retinal occlusion and orbital pseudotumor with various degrees of expression.

[Botulinum toxin A after microvascular ALT flap in a patient with (corrected) squamous cell carcinoma of the tongue]. / Botulinumtoxin A gegen Hypersalivation. Einsatz bei Wundheilung nach Resektion eines Zungenkarzinoms.

We report the case of a 23-year-old man presenting with a cT4 cN1 M0squamous cell carcinoma of the right tongue. After tumor resection and covering of the defect with a microvascular anterolateral thigh (ALT) flap, the patient showed distinct drooling without any substantial regression after anticholinergic therapy. For this reason 75 units of Botox® were injected into the submandibular and parotid glands. After the intraglandular injections, good reduction of saliva secretion was achieved. The treatment improved flap healing, aspiration and patient satisfaction. Injection of botulinum toxin A into the salivary glands is a sufficient therapy for postoperative hypersalivation.

A comparison of angiographic CT and multisection CT in lumbar myelographic imaging.

BACKGROUND AND PURPOSE: The purpose of this work was to provide an intraindividual comparison of angiographic CT (ACT) and multisection CT (MSCT) in lumbar myelographic imaging and to evaluate possible benefits of ACT, which is a further development of rotational angiography providing image data of high spatial and CT-like contrast resolution. MATERIALS AND METHODS: In 26 patients with degenerative lumbar spine disease a lumbar ACT was performed in combination with conventional lumbar myelography and followed by postmyelographic MSCT. Conventional lumbar myelography and lumbar ACT were performed with a flat panel detector-equipped angiographic device. Postmyelographic MSCT was performed with a 16-section CT scanner. Three experienced neuroradiologists rated anonymized sets of multiplanar reformatted CT and ACT images regarding diagnostic and technical parameters. The ratings were repeated after 2 months. Weighted kappa statistics were calculated to describe the levels of intraobserver and interobserver agreement. RESULTS: The analysis shows that MSCT achieves higher ratings than ACT in all of the parameters asked. An adequate diagnostic quality was only assigned to 80% of the ACT acquisitions compared with 97% of the MSCT acquisitions. All of the mean kappa values were above 0.60, demonstrating a substantial intraobserver and interobserver agreement for MSCT, as well as for ACT. CONCLUSION: Using ACT, radiographic myelography and myelographic CT can be performed at the same imaging system. However, the results of our study show that the current myelographic ACT image quality fails to apply diagnostic standards. We, therefore, cannot recommend ACT as a general alternative to postmyelographic MSCT.

[Case-report. Malignant nerve sheath tumor in a cow with symptoms of suspected BSE]. / Fallbericht: Maligner Nervenscheidentumor bei einer Kuh mit BSE-verdächtiger Symptomatik.

We report here on a 3 1/2-year-old mother cow with a malignant perineural tumour near the pontine angle of the cerebellum, but which first drew attention because of clinical signs of BSE. Neurological symptoms that manifested during the course of the disease included disturbances in behaviour, movement and aesthesia, as described by BRAUN et al. (2001) in cases of BSE. Inconsistent with a diagnosis of BSE were focal neurological disturbances (head held aslant to the right, tendency to fall to the right, right-sided facial weakness, left-sided nystagmus and ventral strabismus). Following euthanasia, magnetic resonance imaging (MRI) revealed a tumour in the cerebellopontine angle. Histological findings describe a malignant peripheral nerve tumour of the vagal nerve with rhabdoid differentiation (a so-called Triton tumour) with an intracranial and an extracranial part.

[Polyneuropathy as a sole syndrome in malignant thymoma]. / Polyneuropathie als einziges paraneoplastisches Syndrom bei malignem Thymom.

Up to 40% of patients with malignant thymoma suffer from paraneoplastic symptoms (90% myasthenia, 10% other symptoms). A 55-year-old patient developed ascending symmetrical sensorimotor tetraparesis. A malignant thymoma without metastases was diagnosed 6 months later. Despite thymectomy followed by radiation and high-dose corticosteroid therapy, the polyneuropathy progressed. Six months after onset, the patient was bound to a wheelchair. Immunosuppressive therapy with cyclophosphamide was initiated, leading to marked remission. After ten cycles, the patient was able to walk independently with walking aids. After the sixth and tenth cycle, respectively, attempts to discontinue immunosuppression led to relapse. In several diagnostic workups, however, there was no tumour relapse. After 13 cycles, cyclophosphamide was replaced by immunoglobulins (0.4 g/kg per day i.v. for 5 days/month) due to progressive renal failure. The patient died just before the second course of this treatment. In conclusion, in the differential diagnosis of rapidly progressive polyneuropathy, a malignant thymoma should be considered, even in the absence of myasthenia. Immunosuppression with cyclophosphamide resulted in amelioration of symptoms in this patient.

Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report.

A 10-year-old girl presented with a cerebral malignant ectomesenchymoma (MEM), a very unusual tumour with undifferentiated mesenchymal as well as ectodermal elements. Somatostatin receptor scintigraphy (SRS) was performed during the diagnostic workup. The recurrent residual tumour mass was exactly visualized with SRS, and was negative after successful treatment of the patient. The potential application of SRS in initial staging, follow-up and therapy planning in MEM is discussed. This is the first application of SRS in MEM.

Diagnosis of a multifocal B cell lymphoma with preceding demyelinating central nervous system lesions by single voxel proton MR spectroscopy.

Single voxel proton magnetic resonance spectroscopy (MRS) provides a rapid non-invasive fingerprint of tissue chemistry. A case history is presented in which a B cell lymphoma with antecedent demyelinating lesions of the CNS was suspected by MRS and confirmed by neuropathological examination and immunoglobulin gene rearrangement.

[MRI findings in Wernicke encephalopathy]. / MRT-Befunde bei der Wernicke-Enzephalopathie.

Wernicke's encephalopathy (WE) is a consequence of vitamin B1 (thiamine) deficiency and in the majority of cases due to alcoholism. We report here the case of a 26-year-old male alcoholic who had stayed helplessly at home for 4 days until hospital admission. Clinical diagnosis was difficult due to major disturbance of consciousness. MRI showed an increase in signal intensity (T2-, FLAIR-weighted) around the third ventricle, the quadrigeminal bodies, the fornices, the mamillary bodies, the floor of the fourth ventricle and around the aqueduct. These findings were indicative of WE although of unusual extent. In this case MRI correlated well with clinical symptomatology. Therapy with thiamine was started immediately and symptoms as well as MRI findings resolved partially. The presented case illustrates the diagnostic usefulness of MRI in WE especially if the patient is of reduced consciousness and clinical investigation is limited.

Progression of arteriovenous malformation in moyamoya syndrome.

We report a case of moyamoya disease (MMD) associated with arteriovenous malformation (AVM). The 30-year-old female patient presented with left-hemispheric transient ischaemic attacks (TIAs) involving dysphasia and right-sided hemiparesis. CT-scan and lumbar puncture showed no evidence of intracranial haemorrhage. Cerebral angiography revealed typical moyamoya vessels and occlusion of multiple cerebral arteries with consecutive collateral blood supply. Moreover, a left-parietal AVM with a diameter of approximately 2 cm was detected. An extra-intracranial arterial bypass (EIAB) connecting the left superficial temporal artery (STA) with a cortical branch of the left middle cerebral artery (MCA) was performed (STA-MCA anastomosis) and yielded subsequent resolution of the neurological deficit. Nine months post-operatively neurological deficits similar to those of the initial presentation recurred. Repeated angiography suggested comparatively increased AVM blood flow, and successful extirpation of the AVM gradually re-established almost full functional ability. However, deterioration of the neurological condition developed again. We herewith present the first European case of moyamoya disease associated with arteriovenous malformation and report the clinical course under an alternative neurosurgical treatment consisting of STA-MCA anastomosis and delayed extirpation of the AVM.