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-ß), 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 patient's informed consent.

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. METHOD: We translated and administered the Greek version of the ACE-R to 95 individuals comprising two groups: a dementia group (n=35) and an age-matched and education-matched healthy control group (n=60). RESULTS: Our results suggest high internal consistency and very good sensitivity, specificity and negative predictive value, but only adequate positive predictive value for the ACE-R, with excellent diagnostic accuracy. In our sample, the ratio of verbal fluency plus language scores to orientation plus memory (VLOM) subscore failed to discriminate patients in the target dementia group. CONCLUSION: The present findings support the usefulness of the ACE-R as a brief dementia screening tool in clinical practice. Further neuropsychologic evaluation is required for the differential diagnosis of dementia.

Transient epileptic amnesia in a memory clinic setting: a report of three cases.

Transient epileptic amnesia (TEA) is a recently described, typically misdiagnosed at presentation, distinctive syndrome, characterized by recurrent, brief attacks of memory loss in middle-aged or elderly people, accompanied by significant interictal memory difficulties. We describe the clinical, neuroimaging, electrophysiological, and neuropsychological characteristics of three patients fulfilling the proposed criteria for TEA, initially referred to our memory clinic for evaluation and treatment of probable dementia. Neuropsychological performance on admission and 6 months after treatment demonstrated a broad range of performance on measures of executive function, lower than expected visuospatial perception, and poor autobiographical memory. TEA should be included in the differential diagnosis of dementia to avoid misdiagnosis and needless medication. It is a benign and treatable condition, yet the associated decline in autobiographical and remote memory despite antiepileptic therapy poses challenges for further research.

Acute paraplegia in painless aortic dissection. Rich imaging with poor outcome.

STUDY DESIGN: Case report. OBJECTIVE: To describe the clinical and imaging findings of a patient with painless aortic dissection. SETTING: University Neurology Department, Thessaloniki, Greece. PATIENT, METHODS, RESULTS: A 46-year-old man was transferred to our Department for emergent evaluation of paraplegia, from the local hospital of the nearby town, where he was admitted complaining from sudden, painless, bilateral leg weakness, 24 h earlier. He presented complete flaccid paraplegia with urinary retention, loss of pain and temperature sensation below the TH7 level and well-preserved vibration and position sense bilaterally. He had no pain and general physical examination was unremarkable. Chest X-rays first raised the suspicion of an aortic lesion. Thoracic MRI revealed cord dilation, with no enhancement on T1-weighted images (wi) and increased signal on T2-wi at the TH9-TH12 levels, suggesting cord ischemia. At the same MR sequences, the double lumen of the descending aorta indicated dissection in both sagittal and axial images. Later the same day, the patient died, and autopsy verified dissection of the descending aorta up to the aortic valve. CONCLUSION: The rapid evolution of our case further points out that radiologists, neurologists, as well as internal specialists should be vigilant for this emergency, which despite rich imaging could have a fatal outcome.

CADASIL presenting with spontaneous intracerebral hemorrhage: report of a case and description of the first family in Northern Greece.

INTRODUCTION: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), the most common inherited cause of stroke and dementia in adults, has been described in six Greek families. Common presenting manifestations include migraine with aura, brain ischemia, mood disorders and cognitive decline. Spontaneous intracerebral hemorrhage (SICH) rarely occurs in CADASIL and only exceptionally as the first clinical manifestation. CASE DESCRIPTION: We have previously reported the sixth Greek family with CADASIL and in particular, two brothers in whom the genetic testing documented a classic mutation of the NOTCH3 gene (Arg169Cys). In this report, we describe the 30-year-old son of the second brother, who suffered a thalamic SICH as the presenting manifestation of CADASIL, in the absence of arterial hypertension or antiplatelet drug use. CONCLUSION: Patients with acute subcortical infarcts, leukoencephalopathy, a history of migraine, mood disorders, and dementia, should always raise the suspicion of CADASIL. However, a SICH, even in the absence of classical risk factors for hemorrhage, is possible and should not exclude the diagnosis of CADACIL. Hippokratia 2016, 20(1): 76-79.

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.

The pharmacological effects of buflomedil in patients with multi-cerebral infarcts dementia: an open, preliminary assessment.

The influence of buflomedil on brain circulation was investigated in an open, preliminary trial in 12 patients with dementia caused by multi-cerebral infarcts. Patients were given 150 mg buflomedil 3-times daily for a period of 6 months. Assessments carried out before treatment included regional cerebral blood flow measurements, using a 133Xenon technique, as well as psychometric tests (Weschler and Benton), psychiatric and neurological evaluations, and routine clinical and laboratory (blood and urine) examinations. The tests and investigations were repeated on Days 90 and 180 of treatment. The results showed that mean regional cerebral blood flow increased by 15% to 22% and 16% to 24% in the right and left hemispheres, respectively, by the end of the trial, and marked improvements were noted in psychiatric symptoms and scores in the psychometric tests. A controlled, double-blind study is being planned to validate these preliminary findings.

Precocious puberty following head injury. Case report.

A 6-year-old girl developed secondary sexual characteristics 5 months after severe closed head injury. Endocrinological tests confirmed a pubertal sexual condition; there was also diminution of serum melatonin and disruption of the diurnal pattern. Magnetic resonance imaging demonstrated focal hypothalamic injury; this is believed to be the first time such a posttraumatic lesion has been demonstrated by imaging techniques. The pathophysiology of this condition is discussed.

Isovolemic hemodilution in experimental focal cerebral ischemia. Part 1: Effects on hemodynamics, hemorheology, and intracranial pressure.

A total of 76 splenectomized dogs were entered in a study of the value and effects of isovolemic hemodilution. Of these, seven were not included in the analysis because of technical errors. Of the remaining 69 dogs, 35 were treated with hemodilution; 28 were subjected to a 6-hour period of temporary occlusion of the distal internal carotid artery and the proximal middle cerebral artery, and seven underwent a sham operation only, with arterial manipulation but no occlusion. The other 34 dogs were not subjected to hemodilution; 26 of these underwent temporary arterial occlusion and eight had a sham operation only. In each group the animals were about equally divided into 1) an acute protocol with regional cerebral blood flow measurements by a radioactive microsphere technique and sacrifice at the end of the acute experiment, and 2) a chronic protocol with survival for 1 week to permit daily neurological assessment and final histopathological examination but without blood flow measurements. Isovolemic hemodilution was performed about 1 hour after the arterial occlusion or sham operation and was accomplished by phlebotomy and infusions of low molecular weight dextran to bring the hematocrit to a level of 30% to 32%. This treatment resulted in a very significant reduction in viscosity and fibrinogen levels. The decrease in hematocrit lasted throughout the week in the animals in the chronic protocol. The decrease in viscosity correlated almost linearly with the decrease in hematocrit. There was a slight decrease in systemic arterial pressure with hemodilution but there were no significant changes in central venous pressure or in pulmonary arterial or wedge pressure. There was a slight decrease in cardiac index in both the hemodilution and control groups, which may have been due to the effects of barbiturate anesthesia. There was a slight increase in the measured blood volume in both groups, which was probably artifactual and related to the method of calculation. Intracranial pressure increased significantly with time in all animals subjected to arterial occlusion, but this increase was less severe in the hemodilution group. There was no significant change in intracranial pressure in sham-operated animals, whether hemodiluted or not. The results of cerebral blood flow measurements, assessment of neurological conditions, and measurement of infarct size are given in Part 2 of this report.

Isovolemic hemodilution in experimental focal cerebral ischemia. Part 2: Effects on regional cerebral blood flow and size of infarction.

Seventy-six splenectomized dogs were entered in a study of the value and effects of isovolemic hemodilution. Of these, seven were not included in the analysis because of technical errors. Of the remaining 69 dogs, 35 were treated with hemodilution; 28 were subjected to a 6-hour period of temporary occlusion of the distal internal carotid artery and the proximal middle cerebral artery, and seven underwent a sham operation only, with arterial manipulation but no occlusion. The other 34 dogs were not subjected to hemodilution; 26 of these underwent temporary arterial occlusion and eight had a sham operation only. In each group the animals were about equally divided into 1) an acute protocol with regional cerebral blood flow measurements by a radioactive microsphere technique and sacrifice at the end of the acute experiment, and 2) a chronic protocol with survival for 1 week to permit daily neurological assessment and final histopathological examination but without blood flow measurements. The general experimental protocol, the hemodynamic and rheological measurements, and the changes in intracranial pressure are described in Part 1 of this report. In the animals with arterial occlusion, blood flow decreased significantly in the territory of the ischemic middle cerebral artery. This decrease was partially reversed by hemodilution in the animals so treated. When the changes in blood flow before and after hemodilution in treated animals are compared with the changes at equivalent times in animals without hemodilution, the increases in flow in the gray matter of the ischemic hemisphere brought about by hemodilution are statistically significant. The neurological condition of the animals in the chronic protocol (sacrificed 1 week after occlusion) with hemodilution, as evaluated by daily neurological assessment, was significantly better than that of the control animals. In the animals sacrificed acutely (8 hours after arterial occlusion), the volume of infarction as estimated by the tetrazolium chloride histochemical method was 7.36% of the total hemispheric volume in the control animals and 1.09% in the hemodiluted animals, showing a statistically significant difference (p less than 0.005). In the chronic animals these values were 9.84% and 1.26%, respectively (p less than 0.005), as calculated by fluorescein staining. By histopathological examination the volume of infarction in the chronic animals was calculated as 10.92% in the control animals and 1.20% in the hemodiluted animals (p less than 0.005).(ABSTRACT TRUNCATED AT 400 WORDS)

Cobalamin deficiency: MRI detection of posterior columns involvement and posttreatment resolution.

Cobalamin-deficient myelopathy is a neurologic disorder manifesting progressive symptoms of paresthesiae and spastic paralysis. The pathologic changes initially involve the posterior columns of the cervical and upper thoracic cord. The authors present the case of a patient with cobalamin deficiency with preferential posterior columns involvement, as evidenced by spinal magnetic resonance imaging findings. Marked clinical and radiographic resolution occurred after administration of vitamin B12. Early recognition of this specific magnetic resonance pattern is essential, because complete recovery can be achieved with prompt treatment at this reversible stage of the disease.

Case report: epidural and bilateral retroorbital hematomas complicating sickle cell anemia.

Early in the course of a painful crisis, a 19-year-old man with known sickle cell anemia (SCA) developed a clinical picture that resembled either early cavernous sinus thrombosis or retroorbital and bifrontal microinfarcts. A brain computer tomography scan demonstrated bilateral retroorbital hemorrhages along with a left frontal epidural hematoma. In the absence of trauma, thrombocytopenia, or any other detectable hemostatic defect, this type of hemorrhagic manifestation in the setting of SCA has not, to our knowledge, been previously reported in the literature.

Isolated ocular ischemic syndrome with no cerebral involvement in common carotid artery occlusion.

PURPOSE: The ocular ischemic syndrome (OIS) is sometimes a complication of common carotid artery (CCA) occlusion causing complete interruption of blood flow through both the internal and external carotid arteries we investigated a single case of an isolated OIS that remained undiagnosed for two years, because the underlying CCA pathology caused no cerebral involvement. CASE REPORT: A 57-year-old man presented with subacute painful visual loss in the right eye in a setting of hypertension, smoking and coronary artery disease. RESULTS. Neurological examination, a brain CT and MRI scan were all normal. Extensive laboratory work-up excluded small artery disease, inflammatory arteritis or cardiac causes of retinal embolism. Ophthalmologic evaluation and fluorescein angiography gave findings consistent with OIS, while vascular ultrasound evaluation and aortic arch angiography verified right CCA occlusion accompanied by an extensive collateral network. CONCLUSIONS: Had this patient been referred sooner for a simple carotid artery work-up, both the CCA occlusion and the OIS could probably had been prevented.

Beneficial effect of piracetam monotherapy on post-ischaemic palatal myoclonus.

A 70-year-old hypertensive woman suffered a subarachnoid haemorrhage followed by delayed vasospasm in the basal cerebral arteries. This resulted in multiple ischaemic lesions in the right middle cerebral artery region and contralateral post-ischaemic palatal myoclonus. In this setting, piracetam administered in high doses (24-36 g/day), abolished the myoclonus observed in this patient. Although there is evidence from case reports and clinical trials of the therapeutic efficacy of piracetam in patients with skeletal myoclonus of various causes, to our knowledge this is the first report indicating the beneficial effect of piracetam monotherapy on post-ischaemic palatal myoclonus.

Hemorrhagic cerebral infarct and ulcerative colitis. A case report.

We report the occurrence of a cerebral ischemic infarct in a young normotensive woman suffering from ulcerative colitis. One week later, clinical neurological deterioration corresponded with hemorrhagic transformation of the previously ischemic region and white matter edema. Extensive laboratory investigation excluded other possible causes for stroke mechanism and rather supported a cerebral vasculitic process, while significant improvement both on neurological and bowel function was observed under prednisone therapy. This report provides some additional support to the possible association between ulcerative colitis and cerebrovascular complications.

Bilateral thalamic infarcts presenting as acute pseudobulbar palsy.

A 28-year-old, previously healthy, normotensive woman suddenly developed an acute pseudobulbar palsy with dysarthria, dysphagia, hypernasal voice and mild right arm paresis. Extensive laboratory investigations excluded all other possible causes of acute pseudobulbar palsy (neoplastic, inflammatory, demyelinative, myasthenic) and an MRI study demonstrated bilateral isolated thalamic infarcts. Oral contraceptives and smoking were the only possible stroke risk factors found and cerebral diaschisis the most tenuous explanation proposed. To our knowledge, this is the first report indicating that bilateral thalamic infarction on specific nuclei could be manifested as acute pseudobulbar palsy.

The evaluation of blood flow Xe133 in different degrees experimentally induced carotid stenosis.

The present study was conducted in order to detect the possible relationship between the degree of carotid artery stenosis and the morphology, width of Xe133 curves and the absolute value of blood flow volume, obtained by the intra-carotid injection of Xe133 in 21 dogs (Groups A,B,C, n = 7 in each). In Group B the morphology of Xe133 curves revealed a double peak on the vertical part and the width a 57% decrease on the same part as compared to the control (Group A), while the per min blood flow value decreased by 47%. In Group C an elevation of the horizontal part was observed in all animals, the width of the vertical part could not be measured and the horizontal part showed a 10% increase as compared to the control Group A. Finally, the per minute blood flow value decreased by 75% compared to the controls.

Carotid compression test and supraorbital photoelectric plethysmography in patients with carotid TIAs.

Fifty-two patients with carotid transient ischemic attacks (TIAs) were subjected to subsequent compression of the common carotid and superficial temporal artery. An EEG recording was monitored simultaneously with plethysmograms from both supraorbital regions during the arterial compression period. All patients were angiographically classified as normal (Group A, n = 25) with less than 50% stenosis (Group B, n = 14) and with more than 50% stenosis (Group C, n = 13), according to the degree of internal carotid stenosis. The carotid compression test was positive in 6 (11%) of our 52 patients, corresponding to 1 (4%) patient from Group A, 1 (7%) from Group B and 4 (30%) from Group C. In contrast, supraorbital photoelectric plethysmography (SOPPL) yielded abnormal results in 17 (33%) of our 52 patients, corresponding to 3 (12%) patients from Group A, 4 (28%) from Group B and 10 (77%) from Group C. We conclude that in view of the false positive results (12%) obtained with the SOPPL technique, the clinician who interprets these findings should be careful and combine these tests with other tests, preferably hemodynamic, for a final decision about the patient's status.

The cortical activity in experimental subarachnoid hemorrhage.

Based on a new subarachnoid hemorrhage model in rabbits, the electrocortical activity was evaluated over a period of one week following two successive whole blood injections in the cisterna magna. A second group was injected with artificial cerebrospinal fluid, while a third group with hemolyzed autologous blood. All the other procedures being the same, it became evident that elevated intracranial pressure (CSF injection) is not able by itself to induce true epileptiform activity; however, it releases phenomena of brainstem reflexes. During whole blood injection, the cortical activity showed a peak in true epileptogenic discharges 5 days after the first injection. Almost identical polyspikes and slow wave complexes were reproduced immediately after the injection of hemolyzed blood. The possible mechanisms involved in the appearance of these phenomena are further discussed.

Delayed diagnosis of spinal vascular malformations presenting as intermittent myelopathy.

We report our recent experience of three male patients, whose clinical diagnosis and subsequent surgery was delayed for 9.25 and 15 months respectively. Initially, they all presented with vague, either sensory or motor lower limb disturbances and intermittency of symptoms was followed by bladder dysfunction or impotence. Extensive laboratory work-up was unrevealing and the non-specific diagnosis of myelopathy further delayed the recognition of the underlying vascular lesion. Finally spinal magnetic resonance imaging (MRI), supine myelography and selective spinal arteriography indicated the presence of a tumor in the first patient and vascular malformation in the other two. Neurosurgery and histology confirmed one cavernous angioma and two arteriovenous malformations, all thoracic and dural. We believe that both the cryptic nature of these lesions and our technical limitations (spinal arteriography and MRI not readily available), accounted for the delayed diagnosis in our three patients, who nevertheless, when treated by surgery showed a satisfactory outcome in the first case, while further deterioration was prevented in the other two.