Dimitrios Karacostas

Adaptation of Addenbrooke’s Cognitive Examination-Revised for the Greek population

Background/Purpose:  Addenbrooke’s Cognitive Examination‐Revised [ 1 ] is a highly sensitive and specific bedside test for the diagnosis of dementia. The aim of this study was to validate a Greek version of this simple, bedside instrument and to investigate its psychometric properties.

Meningioma growth and interferon beta-1b treated multiple sclerosis: coincidence or relationship?

Although the coincidence of multiple sclerosis (MS) and central nervous system (CNS) tumors has been reported in over 30 cases in English literature, meningioma growth was associated with interferon-beta (INF-b) treated MS only in two of them. We report the case of a 19-year-old woman with clinically possible, laboratory supported MS, and a concomitant right intraventricular tumor with magnetic resonance imaging (MRI) characteristics consistent with meningioma (similar signal with grey matter on T1 and T2-weighted images and homogenous, intense enhancement). Two years after initiation of INF-b treatment, follow-up brain MRI revealed enlargement of the intraventricular mass and relative increase in the number of white matter lesions without significant clinical deterioration. She underwent almost total resection of the mass and histology confirmed the diagnosis of papillary meningioma. Based on the immunohistochemistry results, we speculate that INF-b resulted in meningioma growth by enhancing platelet derived growth factor (PDGF) receptors or/and down-regulating transforming growth factor receptors on the tumor itself.

Validation of the Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS) in Greek population with multiple sclerosis

BACKGROUND Cognitive impairment is experienced by about 50% of patients with Multiple Sclerosis (MS) worldwide and affects their employment, disease management and quality of life in general. The Brief International Cognitive assessment for MS (BICAMS) is a brief, practical and potentially universal battery for cognitive impairment in MS patients. It consists of three tests: the Symbol Digit Modalities Test (SDMT), the California Verbal Learning Test-2 (CVLT-2) and the Brief Visuospatial Memory Test-Revised (BVMT-R). OBJECTIVE The objective of this study was to validate the BICAMS in Greek MS patients and controls. METHODS Forty four MS patients and seventy nine healthy control (HC) participants were recruited and tested. They were group matched for age, education, gender and also premorbid cognitive reserve. All of them completed the three tests of the BICAMS battery. Instead of CVLT-2, the Greek validated form (Greek Verbal Learning Test, GVLT), was used. In addition, cognitive reserve was assessed using the Cognitive Reserve Index questionnaire (CRIq) standardized for the Greek population. RESULTS Significant difference was found in the performance of the two groups in all tests (p<0.0001, p<0.02, p<0.009 for SDMT, GVLT and BVMT-R respectively). Test-retest reliability was good for all the tests. Based on the criterion of 1 or more tests below the 5th percentile of healthy controls performance, 47% of patients were found impaired. CONCLUSIONS The study provides validation of BICAMS in Greek population and therefore facilitates the use of this battery in clinical practice and in future studies of MS patients in Greece.

Parry-Romberg Syndrome Associated with Localized Scleroderma

Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea).

Isolated distal hand weakness as the only presenting symptom of myasthenia gravis

Weakness of distal extremity muscles is unusual in myasthenia gravis (MG) and in the limited references, isolated hand weakness has been the presenting symptom in only one case. A 30-year-old woman developed asymmetric distal hand weakness, particularly involving finger extensors and abductors, which remained the only presenting symptom for 1 year, before diplopia was added. The clinical profile and laboratory work-up excluded other possible causes of pure hand weakness and confirmed the diagnosis of MG. To our knowledge, our patient represents the second case in the literature, pointing out that isolated distal hand weakness could remain the only presenting symptom of MG for a long time.

Behavioral disturbances differentiate frontotemporal lobar degeneration subtypes and Alzheimer's disease: evidence from the Frontal Behavioral Inventory

Behavioral assessment is useful for the diagnosis of frontotemporal lobar degeneration (FTLD). We explored the ability of the Frontal Behavioral Inventory (FBI) to discriminate between patients with distinct subtypes of FTLD and patients with Alzheimers disease (AD), as well as the influence of demographic variables on FBI scores.

Predictable ventricular shift after focal cerebral ischaemia in rats: practical considerations for intraventricular therapeutic interventions

Intracerebroventricular (ICV) route of administration is a useful experimental method to study the effects of chemicals or cellular grafts in the ventricular compartment of the brain after focal ischaemia. However, the induced oedema may cause structural dislocating phenomena and render a stereotaxic ICV invasion difficult and practically unavailable especially during the acute post-ischaemia phase. The aim of this study was to measure these structural ventricular dislocations and set new stereotaxic coordinates for successful and cost-effective ICV invasion 6–18 h after focal cerebral ischaemia. Wistar rats were subjected to 2 h middle cerebral artery occlussion (t-MCAO), were neurologically evaluated (modified Neurological Stroke Scale [mNSS], modified Bedersons Scale [mBS] and grid-walking test [GWT]) and brain slides were studied at 6 and 18 h post-occlusion for infarction volume, hemispheric oedema, middle line dislocation and stereotaxia of the lateral ventricles. Our data indicated that stereotaxic coordinates of the lateral ventricles in the infarcted and contralateral hemispheres significantly (P < 0.05) changed at both time-points and were linearly correlated with the mNSS, mBS and some GWT scores (P < 0.001). This correlation allowed for the calculation of simple (linear) mathematical equations (stereotaxic coordinate = b0 + b1*mNSS, where ‘b0’ and ‘b1’ are fixed number and factor, respectively, calculated by regression analysis) that determined individually new coordinates for each animal. Verification experiments revealed that the new coordinates render ICV invasion feasible in up to 80% of infarcted rats (number needed to treat 1.65), compared with only 19.4% using the classical coordinates for normal rats. Therefore, we propose a new, time- and cost-effective methodology for practically feasible ICV invasion in rats 6–18 h after t-MCAO.

Isolated myopathy as the initial manifestation of primary systemic amyloidosis.

Sirs: Primary systemic amyloidosis (AL) is an uncommon disorder in which immunoglobulin light chains are deposited in the tissues as amyloid, resulting in organ dysfunction [1]. Common presentations are nephrotic syndrome, cardiomyopathy, peripheral neuropathy or hepatomegaly [1, 4]. In contrast, amyloid myopathy (AM) is an unusual manifestation of AL [1, 9] and muscle weakness as the initial symptom of the disease is rare [1, 7, 11, 12] A 65-year old man, with no medical or family history, presented with 1-year muscle weakness, fatigue and 4.5kg weight loss. The weakness progressed so that he was unable to walk without support or lift his arms above his head. General physical examination was unremarkable. According to the Medical Research Council (MRC) scale, he had grade 3 in the neck flexors, extensors and the proximal upper extremities muscles bilaterally. Grade 4 was noted in the distal hand muscle groups. In the legs, the hip extensors, flexors and abductors were grade 2, the knee extensors and flexors grade 3 and ankle dorsiflexors and evertors were 4. There were no fasciculations, muscle atrophy, pseudohypertrophy, nodular lesions or macroglossia. All cranial nerves and the rest of the neurological examination were normal. An extensive hematological, biochemical and immunological investigation, including serum protein electrophoresis and immunoelectrophoresis, was either normal or negative. Erythrocyte sedimentation rate ranged from 59 to 66 mm/h. Echocardiography revealed concentric myocardial thickening, with an interventricular septal thickness of 1.6 cm (normal 0.6–1.1 cm), diastolic dysfunction, but normal valves, systolic ejection fraction and no “scintillating” appearance. Cervical spine MRI was normal. Neurophysiology revealed normal sensory and motor conduction velocities, amplitudes and distal latencies, with no significant decrement after stimulation at 3Hz. Needle EMG demonstrated positive waves and fibrillation potentials in all muscles tested. Early motor unit potentials (MUPs) were of short duration, low amplitude and polyphasic in all muscles examined. Maximal effort showed the same pattern of MUPs described above, except for the biceps and the first dorsal interosseous, in which short, high amplitude, polyphasic potentials were recorded. Recruitment was full and early in all muscles tested. A left deltoid muscle biopsy with frozen sections processed for hematoxylin-eosin demonstrated several angular atrophic and degenerated or necrotic muscle fibers, with no inflammatory cellular infiltration (Fig. 1). Congo red staining revealed amyloid deposits infiltrating the thickened walls of small intramuscular vessels (Fig. 2). No amyloid was observed surrounding or infiltrating muscle fibers. Immunoperoxidase showed lambda light chains in the affected vessel walls. His weakness deteriorated and 8 months later, he was confined to a wheelchair and had multiple nodular subcutaneous lesions. Biopsy from the skin and small intestine revealed diffuse amyloid deposition surrounding blood vessels in Congo red stained sections. Immunohistology for amyloid A and prealbumin was negative in both, establishing AL. During the next 4 months, colchicine treatment was ineffective and the patient died at home from cardiac arrest, at the age of 66 years. Lubarsch [5] first reported amyloid involvement of muscle with vascular and interstitial deposits in skeletal muscle and the LETTER TO THE EDITORS

Internal Carotid Artery Floating Thrombus in Relapsing Polychondritis

Relapsing polychondritis is a rare autoimmune disease characterized by inflammation of cartilaginous tissues. It may be associated with systemic and cerebral vasculitis and exceptionally with ischemic stroke. Brain infarction associated with internal carotid artery thrombus, in a setting of relapsing polychondritis, has never been reported. We present a 52‐year‐old man without any known risk factors for stroke, treated with prednisone and azathioprine for relapsing polychondritis, who presented a minor left hemisphere stroke. Ultrasound of the neck vessels revealed an isoechogenic thrombus in the left internal carotid artery superimposed on a smooth moderately stenosing isoechogenic atheroma of the carotid bulb. The patient was treated with high‐dose tinzaparin and was followed with serial ultrasound. After 16 days, the thrombus demonstrated a hypoechogenic core surrounded by a hyperechogenic rim and the following day it resolved completely. Thrombus formation on a small unruptured plaque may reflect involvement by relapsing polychondritis of the intimal proteoglycans that hold a role in the development of atheromatosis.

Coexistence of Multiple Sclerosis and Ankylosing Spondylitis: Report of Two Cases

Background: Multiple sclerosis (MS) only rarely coexists with ankylosing spondylitis (AS). The optimal management of these patients represents a major challenge. Methods: In the present study, we report 2 cases of AS with definite MS comorbidity. One of the AS-MS cases had received anti-TNFα treatment, which was discontinued due to exacerbation of the MS. In addition, we discuss 3 more AS cases with neurological symptoms and atypical white matter demyelinating MRI lesions after anti-TNFα treatment. Discussion: Given the fact that anti-TNFα drugs can potentially exacerbate a latent MS or induce atypical demyelination in the central nervous system, they should be discouraged or discontinued in relevant cases. The remaining effective therapeutic options for MS are either contradictory for AS (interferon-β), have no definite data regarding their safety/efficacy in AS (glatiramer acetate, azathioprine, natalizumab, fingolimod), or their efficacy in MS-AS is associated with increased treatment risks (rituximab). Any of these proposed treatments may require active patients informed consent.