Headache in cerebral venous thrombosis.

Headache has been consistently reported as the most common symptom of cerebral venous thrombosis and as the most frequent presenting feature. It is often the heralding symptom, preceding other manifestations of the disease by days or even weeks. This aspect highlights the importance of recognizing headache due to cerebral venous thrombosis, as early recognition of the disease can lead to a rapid diagnosis with appropriate imaging techniques and as early treatment with heparin can dramatically change the course of the disease and alter the prognosis. Unfortunately, although common, the headache has no specific features, and the clinical presentation of CVT is highly variable, making the correct diagnosis in the emergency setting a challenging task for clinicians, even in the case of highly specialized ones such as neurologists. In this review, we will briefly summarize the epidemiology and physiopathology of CVT, and then we will discuss in more details the causes, features, and course of headache, focusing on its relevance for differential diagnosis and on red flags that should suggest the possibility of CVT as the cause of the headache.

Psychological features and quality of life in 50 adult patients with epilepsy and their caregivers from the Lecco epilepsy center, Italy.

Epilepsy is one of the most common neurological disorders. To the best of our knowledge, in Italy, the relationship between patients' and caregivers' psychological state has rarely been analyzed. Thus, we sought to evaluate both the psychological state of patients with epilepsy and that of their caregivers and the interrelationship between them. We also assessed the existing relation between psychological features and some clinical and demographic information, such as number of antiepileptic drugs (AEDs), epilepsy duration and education level of patients and their caregivers. We enrolled in the study 50 consecutive adult patients attending the epilepsy clinic of "A. Manzoni" Hospital and their caregivers. Both patients and their caregivers were administered Hospital Anxiety and Depression Scale (HADS) and 36-item Short-Form Health Survey (SF-36). Anxiety, depression and quality of life values of both patients and their caregivers did not differ significantly from the normative samples. No statistically significant correlation between epilepsy duration and patients' and caregivers' psychological features was found. Patients which took more than one AED reported lower values of "Vitality" (p <.05) and "Social Functioning" (p <.05) than their own caregivers. Caregivers with higher education level presented lower "Vitality" values than caregivers with lower education level (p <.05). Patients with pharmacoresistant seizures reported lower values of "Mental Health" than patients with non-pharmacoresistant seizures (p <.05). In this context, the role of coping mechanisms by patients and caregivers may explain apparently unexpected findings and suggests that strategies aimed at reinforcing them may be effective in selected cases. Therefore, while the severity of epilepsy may have an impact on the psychological state of adult patients with epilepsy and their caregivers, our results highlight the role of multidimensional determinants, including stigma. Further studies are needed to identify the factors related to epilepsy, patients, caregivers, treatments, and the environment that may be modifiable in order to improve self-perceived QoL.

Neurophysiology versus clinical genetics in Rett syndrome: A multicenter study.

Many studies have attempted to establish the genotype-phenotype correlation in Rett syndrome (RTT). Cardiorespiratory measurements provide robust objective data, to correlate with each of the different clinical phenotypes. It has important implications for the management and treatment of this syndrome. The aim of this study was to correlate the genotype with the quantitative cardiorespiratory data obtained by neurophysiological measurement combined with a clinical severity score. This international multicenter study was conducted in four European countries from 1999 to 2012. The study cohort consisted of a group of 132 well-defined RTT females aged between 2 and 43 years with extended clinical, molecular, and neurophysiological assessments. Diagnosis of RTT was based on the consensus criteria for RTT and molecular confirmation. Genotype-phenotype analyses of clinical features and cardiorespiratory data were performed after grouping mutations by the same type and localization or having the same putative biological effect on the MeCP2 protein, and subsequently on eight single recurrent mutations. A less severe phenotype was seen in females with CTS, p.R133C, and p.R294X mutations. Autonomic disturbances were present in all females, and not restricted to nor influenced by one specific group or any single recurrent mutation. The objective information from non-invasive neurophysiological evaluation of the disturbed central autonomic control is of great importance in helping to organize the lifelong care for females with RTT. Further research is needed to provide insights into the pathogenesis of autonomic dysfunction, and to develop evidence-based management in RTT. © 2016 Wiley Periodicals, Inc.

Persistent Interictal Musical Hallucination in a Patient With Mesial Temporal Sclerosis-Related Epilepsy: First Case Report and Etiopathological Hypothesis.

Musical hallucination is a disorder of complex sound processing of instrumental music, songs, choirs, chants, etc. The underlying pathologies include moderate to severe acquired hearing loss (the auditory equivalent of Charles Bonnet syndrome), psychiatric illnesses (depression, schizophrenia), drug intoxication (benzodiazepines, salicylate, pentoxifylline, propranolol), traumatic lesions along the acoustic pathways, and epilepsy. The hallucinations are most likely to begin late in life; 70% of patients are women. Musical hallucination has no known specific therapy. Treating the underlying cause is the most effective approach; neuroleptic and antidepressant medications have only rarely succeeded.Musical hallucination in epilepsy typically presents as simple partial seizures originating in the lateral temporal cortex. To our knowledge, no formal report of musical hallucination in the interictal state has been published before. In contrast, other interictal psychotic features are a relatively common complication, especially in patients with long-standing drug-resistant epilepsy.We describe a 62-year-old woman with a long history of mesial temporal lobe epilepsy whose musical hallucination was solely interictal. We speculate on the possible link between temporal epilepsy and her hallucination. We hypothesize that, as a result of her epileptic activity-induced damage, an imbalance developed between the excitatory and inhibitory projections connecting the mesial temporal cortex to the other auditory structures. These structures may have generated hyperactivity in the lateral temporal cortex through a "release" mechanism that eventually resulted in musical hallucination.

Neuropathy and levodopa in Parkinson's disease: evidence from a multicenter study.

The objectives of this study were to evaluate the risk of neuropathy in patients with Parkinson's disease (PD) and to evaluate the role of levodopa exposure as a potential risk factor. A multicenter study of 330 patients with PD and 137 healthy controls with a comparable age distribution was performed. With respect to levodopa exposure, 144 patients had long exposure (≥ 3 years) to levodopa (LELD), 103 patients had short exposure (<3 years) to levodopa (SELD), and 83 patients had no exposure to levodopa (NOLD). Nerve function was evaluated using the reduced total neuropathy score. Right sural sensory antidromic and peroneal motor nerve conduction studies were performed by neurophysiologists who were blinded to the existence of neuropathy clinical features or PD treatment. Overall, 19.40% of patients in the LELD group, 6.80% in the SELD group, 4.82% in the NOLD group, and 8.76% in the control group were diagnosed with neuropathy (axonal, predominantly sensory). Multivariate logistic analysis indicated that the risk of neuropathy was not influenced by disease duration, severity, or sex. The risk of neuropathy increased by approximately 8% for each year of age (P < 0.001; odds ratio [OR], 1.08; 95% confidence interval [CI], 1.037-1.128). The risk of neuropathy was 2.38 higher in the LELD group than in the control group (P = 0.022; OR, 2.38; 95% CI, 1.130-5.014). In a comparison between patients with and without neuropathy (Student's t test), the levodopa dose was higher (P < 0.0001), serum vitamin B12 levels were lower (P = 0.0102), and homocysteine levels were higher (P < 0.001) in the patients with neuropathy. Our results demonstrate that the duration of exposure to levodopa, along with age, is the main risk factor for the development of neuropathy. Screening for homocysteine and vitamin B12 levels and clinical-neurophysiological monitoring for neuropathy may be advisable in patients with PD who are receiving treatment with levodopa.

How aware are migraineurs of their triggers?

BACKGROUND: Broad discrepancies in the number of migraine triggers have been reported in several studies. Migraineurs do not seem to recognize easily headache triggers in clinical practice. OBJECTIVE: To evaluate how aware migraineurs are about their headache triggers. MATERIALS AND METHODS: We recruited 120 consecutive migraineurs. Each patient was first asked to report spontaneously any migraine trigger. Subsequently, the patient selected from a list of commonly known triggers. RESULTS: Ninety-seven patients (72.5%) spontaneously reported at least 1 migraine trigger, and 120 patients (100%) reported at least 1 migraine trigger selecting from a specific list of precipitants. The mean number of spontaneously identified triggers was 1.5 (±1.5), and the total number of triggers identified was 7.20 (±3.9). CONCLUSIONS: A relevant discrepancy between the number of spontaneously recognized triggers and the total number of triggers was found. This may suggest that migraineurs display poor awareness about headache triggers.

Triggers in allodynic and non-allodynic migraineurs. A clinic setting study.

BACKGROUND: Allodynia is considered a phenomenon of central sensitization that may lead to migraine transformation, lowering the attack threshold. Migraine triggers are factors that may induce headache attacks in susceptible individuals. We hypothesize that because allodynia decreases the migraine-attack threshold, allodynic migraineurs are more susceptible to triggers than the non-allodynic ones. OBJECTIVE: To determine if the number of headache triggers differs between migraineurs with no/mild allodynia and those with moderate/severe allodynia. MATERIALS AND METHODS: We recruited 120 consecutive migraineurs. Other primary headache comorbidity and migraine prophylaxis were exclusion criteria. Each patient was interviewed according a structured questionnaire including general features about migraine, depression, and anxiety symptoms. Patients reported any migraine trigger both spontaneously and by selecting from a specific list. Allodynia during the migraine attack was measured using the Allodynia Symptoms Check-List 12 (ASC-12): a cut-off of ASC-12 score of >2 defined allodynic patients; 3-5 score indicated mild allodynia; a 6-8 score moderate allodynia; and score of >8 severe allodynia. RESULTS: The total number of identified triggers was significantly and positively related to allodynia measured with ASC-12 (ρ(s) 0.33; P < .001). In a logistic regression model, allodynia independently influenced the risk to have a higher number of triggers. Moderate/severe allodynic patients had an odds ratio of 2.8 to report a number of triggers >7 in respect to non-/mild allodynic ones. CONCLUSIONS: Migraineurs with moderate/severe allodynia had more triggers than those with no/mild allodynia. It is unknown if those with moderate/severe allodynia are more susceptible to triggers, or repetitive stimulation of the trigeminal system by triggers resulted in moderate/severe allodynia.

Cognitive correlates of negative symptoms in behavioral variant frontotemporal dementia: implications for the frontal lobe syndrome.

Although both behavioral disturbances and executive impairments of patients with the behavioral variant frontotemporal dementia (bvFTD) seem to depend on early neurodegenerative damages to the prefrontal cortex, the relationship between these two distinct clinical features has been only partially established and represents the focus of the current preliminary neuropsychological study. Ten subsequent bvFTD patients underwent a neuropsychiatric assessment with the Frontal Behavior Inventory and a neuropsychological battery focused on prefrontal functions. Significant correlations were found only between negative symptoms and measures of prevalent medial prefrontal functioning, i.e. decision making under ambiguity (Iowa gambling task) (r = -0.887; p = 0.018) and affective theory of mind (reading the mind in the eyes task) (r = -0.982; p = 0.017). This finding could preliminary support a "frontal lobe syndrome" hypothesis for negative symptoms of bvFTD patients, as proposed for negative symptoms of schizophrenia; the small sample size represents a limit and empirical findings need replication in larger samples of bvFTD patients.

Variant of Rett syndrome and CDKL5 gene: clinical and autonomic description of 10 cases.

UNLABELLED: Rett syndrome (RTT) is a severe neurodevelopmental disorder affecting almost exclusively females. The Hanefeld variant, or early-onset seizure variant, has been associated with mutations in CDKL5 gene. AIMS: In recent years more than 60 patients with mutations in the CDKL5 gene have been described in the literature, but the cardiorespiratory phenotype has not been reported. Our aim is to describe clinical and autonomic features of these girls. METHODS: 10 girls with CDKL5 mutations and a diagnosis of Hanefeld variant have been evaluated on axiological and clinical aspects. In all subjects an evaluation of the autonomic system was performed using the Neuroscope. RESULTS: Common features were gaze avoidance, repetitive head movements and hand stereotypies. The autonomic evaluation disclosed eight cases with the Forceful breather cardiorespiratory phenotype and two cases with the Apneustic breather phenotype. CONCLUSIONS: The clinical picture remains within the RTT spectrum but some symptoms are more pronounced in addition to the very early onset of seizures. The cardiorespiratory phenotype was dominated by Forceful breathers, while Feeble breathers were not found, differently from the general Rett population, suggesting a specific behavioral and cardiorespiratory phenotype of the RTT the Hanefeld variant.

Peri-ictal prolonged atrial fibrillation after generalized seizures: description of a case and etiopathological considerations.

Cardiac rhythm changes are not uncommon in connection with seizures and should be considered and recognized given their potentially harmful consequences including Sudden Unexpected Death in Epilepsy (SUDEP). The most well known are ictal tachycardia and bradycardia. However, other potentially dangerous peri-ictal arrhythmias have been reported. Brief atrial fibrillation episodes, never longer than 2 min, have rarely been described in connection with seizures. We report the case of a patient who presented with two generalized tonic-clonic seizures associated with prolonged atrial fibrillation. Extensive non-invasive cardiac investigations failed to disclose cardiac abnormalities, and after proper antiepileptic drug treatment the patient had neither further seizures nor cardiac events in an 18-month follow-up. Our case, to our knowledge, is the first report of prolonged (more than 1 h) peri-ictal atrial fibrillation.

Bilateral thalamic infarct caused by spontaneous vertebral artery dissection in pre-eclampsia with HELLP syndrome: a previously unreported association.

Cerebrovascular accidents are not rare during pregnancy and the postpartum period. Pre-eclampsia is a common condition that is characterized by proteinuria and de novo hypertension that may be complicated by hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Spontaneous cervical artery dissection has been rarely reported in the postpartum period but never in association with HELLP syndrome. We describe a case of pre-eclampsia and HELLP syndrome complicated in the postpartum period by bilateral thalamic infarct as result of left vertebral artery dissection. We speculated about the possible common etiopathologic mechanisms involved in this previously unreported association.

Postpartum headache due to spontaneous cervical artery dissection.

Postpartum headache is quite common and often related to potentially ominous cerebrovascular accidents. As illustrated in previously published reports, spontaneous cervical artery dissection is a rare but possible cause of headache in the postpartum. We provide 2 additional cases to the 19 described so far, including the first ever report of migraine with aura-like symptoms. Additionally, we summarize the literature and we speculate about the possible etiopathological mechanism underlying this condition.

A case of monofocal motor neuropathy with GM1 and GD1b antibodies improved with intravenous immunoglobulin.

Multifocal motor neuropathy is a purely motor neuropathy with a probably dysimmune pathogenesis, supported by the presence of anti-GM1 IgM antibodies in about half of the cases. Single nerve involvement allows for diagnosis of possible multifocal motor neuropathy. We present the case of a middle age man presenting with progressive weakness and hypotrophy in the left leg and difficulty in walking, in which we have diagnosed a dysimmune mononeuropathy. Treatment with IVIg was performed with substantial improvement. Although only one nerve is involved, early diagnosis of dysimmune mononeuropathy is important to start IVIg treatment that is often decisive.

Extratemporal ictal clinical features in hippocampal sclerosis: their relationship to the degree of hippocampal volume loss and to the outcome of temporal lobectomy.

PURPOSE: Since extratemporal clinical features in patients with unilateral hippocampal sclerosis (HS) are likely to indicate aberrant ictal spread or a more extensive epileptogenic zone, we asked whether such features are associated with more severe HS and a worse outcome following temporal lobectomy. PATIENTS AND METHODS: We reviewed all patients (174) who had undergone temporal lobectomy for histologically proven unilateral HS related temporal lobe epilepsy between 1997-2005 at the National Hospital for Neurology and Neurosurgery. We divided patients into those with severe HS (side-to-side ratio < 0.6) and those with mild HS (side-to-side ratio > 0.75). We examined all seizures recorded on electroencephalography (EEG) video telemetry in these patients for clinical features of temporal lobe epilepsy. The postsurgical outcome was classified using the Engel classification at the time of follow up (median 4.7 years, range 1-9 years). RESULTS: Patients (28 out 39) with severe HS had atypical features compared to 7 out of 27 in the mild HS [Chi square (chi(2)) test, p = 0.0013]. The mean number of atypical clinical features was 2.2 in the severe HS group and 0.62 in the mild HS group (Mann Whitney U Test, p < 0.001). The percentage of postsurgery seizure freedom (class 1 Engel classification) was 87%, and there was no significant effect of the presence of atypical clinical features. CONCLUSIONS: This study shows that atypical (extratemporal) clinical features tend to occur more frequently in patients with severe HS and do not correlate with worse surgical outcome.

[Combination of endovenous laser treatment and a surgical approach for venous disease]. / EVLT (EndoVenous Laser Treatment) combinato con approccio chirurgico per la malattia venosa.

The aim of our study was to report our experience with endovenous laser treatment (EVLT), initiated with an exclusively US-guided approach, but then combined with a surgical approach. Over the period from September 2003 to December 2005 in the Sondrio Hospital General Surgery Unit 61 patients were submitted to EVLT. In the first 13 cases the procedure was performed under ultrasonographic guidance, whereas later we opted for a combined technique in which the use of the laser was preceded by high ligation of the saphenous vein ("crossectomy") together with complete sectioning of the venous collaterals (48 cases). We observed no complications related to the saphenous vein ligation, whilst EVLT proved safe and easy to perform. Considering successful EVLT as ultrasonographically documented absence of flow in the saphenous vein, a statistically significant difference (p < 0.01) was found between the patients treated using the combined strategy (absence of flow in 92.0% of cases) and those treated with EVLT alone (absence of flow in 54.6% of cases). If EVLT is combined with high ligation of the saphenous vein, it is easier to obtain complete sclerosis of the saphenous trunk, avoiding possible recurrences, amongst other things thanks to the ligation of all the collateral veins that flow into the cross. Moreover, particular selection of patients to be submitted to EVLT proves less necessary, thus substantially increasing the indications for the procedure.

Immediate loading implants: a clinical report of 1301 implants.

The purpose of this study is to suggest 4 immediate load of implants techniques that are alternative and/or complementary to the traditional submersion technique. These techniques meet the criteria for implant immobilization during the entire period of osteointegration, and are based on the principles of splinting and load sharing. One hundred fifty-three maxillary and 309 mandibular arches were treated with 1301 implants. Only implants that satisfied the primary retention were immediately loaded with a provisional crown with wings (94), provisional plastic prosthesis (478), provisional plastic prosthesis with metal frame (293), and intraoral welding (436). Facies morphology, type of occlusion, size and function of the tongue, bone density, number, and length of implants appeared to have an influence on the results. Success rates achieved over 21 years are 99.3% with the intraoral welding machine, 98.3% with the provisional plastic prosthesis with metal frame, 97.9% with metal wings, and 88.02% with provisional plastic prosthesis. This last percentage is reflective of the years 1974 to 1984 when only blade-forms and root-forms with unscrewable abutments were available and a provisional plastic prosthesis was the only immobilization technique known. Guidelines are proposed for a treatment plan indicating when and why immediate loading implants can be suggested.