Damir Petravić

Recurrent optic neuromyelitis with endocrinopathies: a new syndrome or just a coincidence?

The spectrum of optic neuromyelitis (ONM) ranges from monophasic or recurrent idiopathic forms of the disease, to ONM associated with autoimmune disorders. A distinct form of the disease, called recurrent ONM with endocrinopathies, characterized by spinal cord involvement (cavitations with syringomyeloid sensory disturbance), rapid evolution to blindness and paraplegia, characteristic cerebrospinal fluid (CSF) findings, and association with hypothalamus - pituitary dysfunction, has recently been described. The first case of ONM with endocrinopathies in a female Caucasian from Europe is presented, supporting the existence of this syndrome as a separate entity.

Stroke – an adverse reaction to sildenafil

Although cardiovascular side effects of sildenafil are recognized, little is known about stroke risk in patients who take this drug. We report a patient with multiple strokes in the posterior cerebral circulation in close temporal association with sildenafil use. This case report further emphasize that stroke is, although rare, real side effect of sildenafil. Potential users should be warned before taking it, especially if they have other stroke risk factors, as recommended by the manufacturer.

CD95/Fas expression on peripheral blood T lymphocytes in patients with multiple sclerosis: effect of high-dose methylprednisolone therapy

Recent data indicate that the apoptotic process, mediated by the CD95/Fas cell surface receptor, is impaired in activated lymphocytes of patients with relapsing-remitting multiple sclerosis. Using flow cytometric-immunophenotyping, we analyzed the expression of CD95/Fas on peripheral blood CD4+ and CD8+ T lymphocytes (PBL) in 10 MS patients in relapse, and the effect of pulse corticosteroid therapy on the apoptosis of autoreactive lymphocytes. The proportions of CD8+ and CD8+CD95+ T lymphocytes were significantly higher in MS patients in relapse before than after pulse corticosteroid therapy. Conversely, the proportions of CD4+ and CD4+CD95+ T cells were significantly lower before than after therapy, but not significantly different from healthy persons. The different expression of CD95/Fas on peripheral blood CD8+ T lymphocytes in relapsing RRMS and in healthy controls suggests a possible involvement of apoptosis in the pathogenesis of MS. Our results also show that pulse corticosteroid therapy influences the CD95/Fas expression on CD8+ and CD4+ T lymphocytes in patients with RRMS.

Horner syndrome due to jugular vein thrombosis (Lemierre syndrome)

A 60-year-old patient was referred to our Department for evaluation of anisocoria. His neurological examination revealed Horner syndrome on the right side. The rest of the examination was normal. Six months before, he had acute tonsillitis: throat cultures revealed a polymicrobial flora consisting of both aerobic and anaerobic bacteria, which was treated with 1 600 000 international units of bipenicillin intramuscularly for 10 days. On admission, he had a normal complete blood count, C-reactive protein and chest X-ray. Throat and blood cultures were normal. Brain multi-sliced …

Level of sFas/APO 1 in serum and cerebrospinal fluid in multiple sclerosis

The aim of the study was to measure sFas/APO 1 serum and cerebrospinal fluid (CSF) levels in patients with relapsing-remitting multiple sclerosis (MS) during relapses, as an index of inhibition of apoptosis of activated lymphocytes in eight patients with clinically definite multiple sclerosis, and 12 healthy controls. The level of serum and CSF sFas/APO 1 was determined by commercially available enzyme-linked immunosorbent assay (ELISA) kits. No significant differences were detected in the sFas/APO 1 serum level between patients and controls, but the levels in CSF was lower in the former. Our results suggest the possibility of Fas mediated apoptosis as a contributing factor in the pathogenesis of multiple sclerosis.

Subacute cerebellar degeneration associated with calcitonin producing tumor.

To the best of our knowledge, we report on the first case of subacute cerebellar degeneration caused by calcitonin producing lung tumor. Subacute cerebellar degeneration is one of the most common paraneoplastic neurologic syndromes. When Greenlee and Brashear [1] discovered the onconeuronal antibody specifically staining the cytoplasm of Purkinje cells and deep cerebellar neurons in patients with subacute cerebellar degeneration and gynecologic tumors in 1983, it has become clear that immune system plays a crucial role in this disease.

Skew Deviation: Case Report and Review of the Literature.

ABSTRACT Objective: To present a patient with a sudden onset ocular tilt reaction (OTR) and review recent knowledge and evolving insights of the underlying pathophysiological mechanisms of skew deviation and OTR. Methods: A middle-aged hypertensive man who had previously suffered stroke with good recovery presented with sudden-onset double vision, slurred speech, ataxia, and a head tilt. Romberg test was positive. The patient denied having disturbances of visual acuity, eye pain, or recent trauma. The right eyeball was pushed upward. The patient complained of double vision in any gaze direction. Movements of the extraocular muscles (EOMs) in the horizontal plane were normal, whereas vertical version and convergence were not possible. We administered a Hess-Lancaster test, cover test, fundoscopic examination, Parks-Bielschowsky three-step test, upright-supine test, brain magnetic resonance imaging (MRI), transcranial doppler (TCD) ultrasonography, electrocardiogram (ECG), Holter monitor (24 h), and echocardiography. Results: The Hess-Lancaster test showed superior rectus muscle and inferior obliquus muscle palsy to the left and rectus inferior muscle and superior obliquus muscle palsy to the right. The right eyeball fell behind when looking downward and the left eyeball when looking upward. Cover alternating test was positive from vertical, R/L. Examination of the ocular fundus showed incyclotorsion of elevated right eye and excyclotorsion of depressed eye. The Parks-Bielschowsky three-step test was negative. A brain MRI with gadolinium revealed a small zone of diffusion restriction in the medial portion of the right cerebral peduncle and right thalamus. There was a gradual improvement in the patient’s neurological status following treatment. Conclusion: Skew deviation, a not uncommon clinical condition, should be promptly recognized when binocular vertical diplopia cannot be interpreted by trochlearis and oculomotor nerve lesion, myasthenia gravis, or orbital pathology. Maddox rod, cover test, Parks-Bielschowsky three-step, and other tests should help to establish the diagnosis. The prognosis depends on etiology, but it is commonly favorable; the majority of patients recover spontaneously after less than a year. More invasive management options should be discussed thereafter.

Reversible Posterior Leukoencephalopathy Syndrome

Reversible posterior leukoencephalopathy syndrome was first described by Hinchey et al. in 1996, as a syndrome clinically associated with headache, altered consciousness, seizures and visual symptoms. On brain magnetic resonance imaging (MRI), T2-weighted and diffusion-weighted images, there is hyperintense region in the parietal-occipital lobe involving lesions of the white matter, basal ganglia, brain stem, cerebellum, and gray matter. This syndrome frequently occurs in patients with hypertensive encephalopathy. The normal response of the cerebral arterioles to acute rising blood pressure is sympathetic nerve-mediated vascular constriction to prevent increasing blood flow (autoregulation). But in the case of reversible posterior leukoencephalopathy syndrome, the response does not work well when there is excess high pressure or recent onset of a modest increase in blood pressure, and excess dilatation of the arterioles following disruption of cerebral small vessel endothelial cells (i.e., the blood-brain barrier) can occur, resulting in vasogenic brain edema. Therefore, disruption of cerebral vascular endothelial cells plays a critical role in the pathogenesis of reversible posterior leukoencephalopathy syndrome. Initially, this syndrome was believed to be secondary to arterial hypertension, renal disease, or immunosuppressive therapy. However, it has recently been identified in a wide variety of conditions, including eclampsia, hemolytic-uremic syndrome, connective tissue diseases, malignancies, etc. Authors describe a case of a 72-year-old woman with a history of arterial hypertension, who suddenly developed headache, confusion, left homonymous hemianopsia, and left hemiparesis, associated with high blood pressure. Brain MRI revealed extensive white matter lesion in the right parietal and occipital lobe, splenium corpus callosum and left occipital lobe, suggestive of expansive process. However, after extensive diagnostic work-up, diagnosis of reversible posterior leukoencephalopathy was established, connected with arterial hypertension. Following antihypertensive and antiedematous therapy her condition improved. At the moment patient is without neurological symptoms, control brain MRI revealed resolution of the white matter lesion. Early recognizing of this syndrome is of great importance, because prompt medicamentous therapy and distinction from brain tumour can postpone invasive diagnostic procedures (stereotactic brain biopsy).