Pasquale Marchione

Neurological Involvement in Primary Sjögren Syndrome: A Focus on Central Nervous System

Objectives Sjögren syndrome is an autoimmune disease involving mainly salivary and lacrimal glands. Beyond widely described PNS involvement, high variable prevalence of CNS manifestations ranging from 2.5 and 60% of all pSS patients has been reported, without specific syndrome definition. The aim of this cohort study was to evaluate the prevalence of CNS signs and symptoms in pSS patients and to identify possible biomarkers of CNS damage. Methods 120 patients with pSS diagnosis according to the 2002 American-European Consensus Group criteria were enrolled after exclusion of secondary causes. All patients underwent to a wide neurological, neuropsychological, psychiatric, neuroradiological and ultrasonographic evaluation. Results Central and peripheral nervous system involvement was observed in 81 patients with a prevalence of 67.5%. The prevalence of CNS involvement was significantly higher than PNS disease (p 0.001). 68 patients (84%) shown non-focal CNS symptoms and 64 (79%) focal CNS deficits with headache as the most common feature (46.9%), followed by cognitive (44.4%) and mood disorders (38.3%). Particularly, we observed a high prevalence of migraine without aura, subcortical frontal executive functions and verbal memory impairment and apathy/alexythimia. MR spectroscopy revealed a reduction of NAA levels or NAA/Cr ratio decrease in subcortical frontal and basal ganglia white matter, while ultrasonography showed an impairment of microvasculature response. At multivariate analysis, headache, cognitive disorders and psychiatric symptoms was significantly associated to serological markers (anti-SSA), MRS and ultrasonographic features. Conclusions The higher prevalence of MWO-mimic headache, cognitive dys-esecutive syndrome and mood disorders observed in this series confirmed previous evidences of a higher diffused CNS compromission rather than focal involvement such as SM-like clinical course or NMO-like syndrome. The association with immunological biomarkers, metabolic cerebral dysfunction and microvascular damage suggests a possible endothelial dysfunction of the cerebral microcirculation or a potential inflammation-mediated shift of the neurovascular coupling.

Ultrasonographic Evaluation of Cerebral Arterial and Venous Haemodynamics in Multiple Sclerosis: A Case-Control Study

Objective Although recent studies excluded an association between Chronic Cerebrospinal Venous Insufficiency and Multiple Sclerosis (MS), controversial results account for some cerebrovascular haemodynamic impairment suggesting a dysfunction of cerebral autoregulation mechanisms. The aim of this cross-sectional, case-control study is to evaluate cerebral arterial inflow and venous outflow by means of a non-invasive ultrasound procedure in Relapsing Remitting (RR), Primary Progressive (PP) Multiple Sclerosis and age and sex-matched controls subjects. Material and Methods All subjects underwent a complete extra-intracranial arterial and venous ultrasound assessment with a color-coded duplex sonography scanner and a transcranial doppler equipment, in both supine and sitting position by means of a tilting chair. Basal arterial and venous morphology and flow velocities, postural changes in mean flow velocities (MFV) of middle cerebral arteries (MCA), differences between cerebral venous outflow (CVF) in clinostatism and in the seated position (ΔCVF) and non-invasive cerebral perfusion pressure (CPP) were evaluated. Results 85 RR-MS, 83 PP-MS and 82 healthy controls were included. ΔCVF was negative in 45/85 (52.9%) RR-MS, 63/83 (75.9%) PP-MS (p = 0.01) and 11/82 (13.4%) controls (p<0.001), while MFVs on both MCAs in sitting position were significantly reduced in RR-MS and PP-MS patients than in control, particularly in EDSS≥5 subgroup (respectively, 42/50, 84% vs. 66/131, 50.3%, p<0.01 and 48.3±2 cm/s vs. 54.6±3 cm/s, p = 0.01). No significant differences in CPP were observed within and between groups. Conclusions The quantitative evaluation of cerebral blood flow (CBF) and CVF and their postural dependency may be related to a dysfunction of autonomic nervous system that seems to characterize more disabled MS patients. Its not clear whether the altered postural control of arterial inflow and venous outflow is a specific MS condition or simply an “epiphenomenon” of neurodegenerative events.

Reversible Pisa syndrome associated to subdural haematoma: case-report

BackgroundPisa Syndrome or Pleurothotonus is a relatively rare truncal dystonia, characterized by tonic flexion of the trunk and head to one side with slight rotation of the body. Since frequently associated to specific drugs such as antipsychotics and cholinesterase inhibitors or to Parkinson Disease, a pathophysiological role of cholinergic-dopaminergic imbalance has been suggested. We report here the first case of Pisa Syndrome due to an extracerebral pathology as subdural haematoma.Case presentationA hypertensive patient was admitted to Our Department for subacute onset of tonic flexion and slight rotation of the trunk associated to progressive motor deficit in left upper limb after a mild head trauma without loss of consciousness occurred around three month before. No previous or current pharmacological interventions with antidepressant, neuroleptic or anticholinergic drugs were anamnestically retrieved. Familiar and personal history was negative for neurological disorders other than acute cerebrovascular diseases. Acutely performed cerebral MRI with DWI showed a voluminous right subdural haematoma with mild shift of median line. After surgical evacuation, both motor deficit and truncal dystonia were dramatically resolved. At one-year follow up, the patient did not develop any extrapyramidal and cognitive signs or symptoms.ConclusionsAccording to many Authors, the occurrence of truncal dystonia during several pharmacologic treatments and neurodegenerative disorders (such as Alzheimer disease and parkinsonian syndromes) supported the hypothesis that a complex dysregulation of multiple neurotransmitter systems are involved. We suggest a possible role of basal ganglia compression in pathogenesis of truncal dystonia by means of thalamo-cortical trait functional disruption and loss of proprioceptive integration. A further contribution of the subcortical structure displacement that alters motor cortex connectivity to basal ganglia may be postulated.

Atorvastatin Treatment and Carotid Plaque Morphology in First-ever Atherosclerotic Transient Ischemic Attack/Stroke: A Case–Control Study

BACKGROUND A relationship between echolucency of carotid plaques and the consequent risk of ipsilateral ischemic stroke has been observed. An aggressive lipid-lowering therapy may increase the echogenicity of carotid plaque in patients with elevated low-density lipoprotein cholesterol levels. The aim of this study is to prospectively evaluate the long-term effect of high-dose atorvastatin on carotid plaque morphology in patients with first-ever transient ischemic attack or stroke. METHODS All patients with symptomatic first ischemic atherosclerotic cerebrovascular event occurred within the previous 10 days were enrolled. Carotid Doppler ultrasound of the neck vessels with 7-11 MHz probe for the definition of the atherosclerotic carotid framework was performed. The analysis of the gray-scale median (GSM) of each plate was carried out with image processing software. RESULTS A total of 240 symptomatic plaques were included and divided into 3 groups: 80 in group A (atorvastatin 80 mg), 80 in group B (atorvastatin 40 mg), and 80 to group C (no atorvastatin). GSM score increases significantly more extensive in group A than in group B (+48.65 vs. +39.46, P < .02) and group C (+48.65 vs. 19.3, P = .0002). An inverse association between reduction of low-density lipoprotein and the increase in the GSM score (r = -.456, P = .007) has been observed. Moreover, the reduction of high-sensitive C-reactive protein correlates inversely with the increase of the GSM (r = -.398, P = .021). CONCLUSIONS Dose-dependent effect of atorvastatin on symptomatic carotid plaque morphology may suggest a specific role of this drug in the atherosclerotic stroke prevention.

Hemiballismus in subthalamic haemorrhage: efficacy of levetiracetam

Sir, A 65-year-old man with uncontrolled hypertension presented with severe ataxia, dizziness and vomiting in January 2005. Magnetic resonance imaging (MRI) brain showed a hyperintense haemorrhagic lesion in the cerebellar vermis on T2weighted images (Fig. 1a) and heterogeneous popcorn-like lesions with a mixed signal core in fast field echo sequences, which were consistent with multiple cavernous angiomas (Fig. 1b). As cavernous angiomas are typically occult at angiography, digital subtraction angiography was deemed unnecessary. Upon admission, high arterial blood pressure values (ABP, mean values 210/110 mmHg) were rapidly controlled with quinalapril 20 mg/ day and hydrochlorothiazide 25 mg/day. Full blood count, erythrocyte sedimentation rate, serum electrolytes, routine autoantibodies, glycaemia, liver and kidney function tests were within normal limits. Three days after admission, the patient presented with two consecutive episodes of severe vomiting and marked increase in ABP (200/100 mmHg) followed by sudden onset of severe involuntary, forceful flailing and wide movements in the left arm and leg. Large, prevalently proximal, vigorous and kicking movements were subcontinuously present during the waking hours, enhanced by voluntary movement of the involved limb and reduced or absent during sleep. A second MRI brain showed a markedly hyperintense haemorrhagic area in fluid attenuated inversion recovery (FLAIR) and T2-weighted images in the right subthalamic nucleus (STN, Fig. 1c). A diagnosis of hemiballismus related to subthalamic haemorrhage was considered. The patient was treated with haloperidol (8 mg/day) and lorazepam (5 mg/day), which led to a rapid improvement in the involuntary movements over a few days and an increasing degree of sedation and stiffness. Treatment was thus discontinued after 1 week; although rigidity and somnolence remitted rapidly, involuntary movements relapsed at 2-week evaluation. Levetiracetam (LEV) was started at a dosage of 125 mg twice daily and increased by 125 mg every week. A significant improvement in the hemiballismus was observed at 4 weeks (1000 mg), followed by complete remission when a final dose of 2000 mg/day was reached. At the 3-month follow-up, LEV was decreased by 125 mg per week to assess a possible long-term spontaneous improvement. As hemiballismus relapsed after a reduction of 1000 mg, LEV dosage was again raised to the previous dose with subsequent improve of involuntary movements.

T05-O-13 Is sexual behaviour related to analgesic addiction in migraine? A psychobiological perspective

Objective Since recent findings suggested high sexual desire in migraineurs, we clinically evaluated the relationship between sexual behaviour and analgesic intake in patients with episodic migraine. Subjects and methods. 130 patients with episodic migraine and 60 sex and age matched subjects with episodic tension-type headache (TTH) participated in the study. After three diagnostic interviews, an expert sexologist administered Sexual Desire Inventory (SDI), Sexual Addiction Screening Test (SAST), Leeds Dependence Questionnaire (LDQ), Beck Depression Inventory (BDI) and Zung Self-rating Anxiety Scale (SAS). Results. Migraineurs reported higher SDI score than TTH (F = 4.62, P P P P P P P P = 0.000) and BDI and LDQ (r = 0,75, P