Francesca Romana Pezzella

Prevalence and clinical features of hedonistic homeostatic dysregulation in Parkinson's disease

Hedonistic homeostatic dysregulation (HHD) is a neuropsychiatric disorder recently described in Parkinsons disease (PD), which is characterized by self‐medication and addiction to dopaminergic drugs. To understand the prevalence of this disorder, we screened 202 PD patients attending our movement disorder unit for HHD. The clinical features of the patients identified as affected by this syndrome were then compared with those of control PD patients in an attempt to ascertain the possible risk factors for HHD. Results showed 7 subjects who fulfilled the HHD criteria. The analysis of a case–control study showed a significant correlation between HHD and a previous history of mood disorders, and the use of dopamine agonists, either in monotherapy or in combination. The prevalence of HHD in our study is similar to the one reported in the United Kingdom by the authors who first described this syndrome in PD. Of interest, our patients showed a somewhat different pattern of the disorder, suggesting that cultural and environmental factors may play a role in the phenomenology of HHD.

3D reconstruction techniques made easy: know-how and pictures.

Three-dimensional reconstructions represent a visual-based tool for illustrating the basis of three-dimensional post-processing such as interpolation, ray-casting, segmentation, percentage classification, gradient calculation, shading and illumination. The knowledge of the optimal scanning and reconstruction parameters facilitates the use of three-dimensional reconstruction techniques in clinical practise. The aim of this article is to explain the principles of multidimensional image processing in a pictorial way and the advantages and limitations of the different possibilities of 3D visualisation.

Hedonistic homeostatic dysregulation in Parkinson's disease: a short screening questionnaire.

Behavioural abnormalities and cognitive impairment frequently complicate long-standing Parkinson’s disease (PD), a neurodegenerative disorder usually characterized by its movement abnormalities. Several factors are implicated in the development of non-motor symptoms of PD, including the underlying neurodegenerative process related to the disease, psychological reactions to the physical illness, and the effect of medications. Hedonistic homeostatic dysregulation (HHD) is neuropsychiatric disorder recently described in PD [1]. It occurs mainly in young-onset male patients with PD, and it is linked to substance misuse and addiction to dopaminergic replacement therapy (DRT) [2]. The core clinical features of HHD are self-medication, drug hoarding, and alteration of the perception of the on state; these patients may develop violent and disabling but surprisingly well-tolerated drug-induced dyskinesia, which does not act as a deterrent to further increases in the level of DRT. Walkabouts are common in these patients, who also present a wide range of mood disorders, including depression, anxiety, hypomanic state, euphoria, and psychosis. Behavioral disorders, such as pathological gambling [3], obsessional shopping, hypersexuality, aggression, and social isolation make the management of these patients more difficult and urgent, since they significantly affect their own and their families’ quality of life. HHD is not common and the only prevalence figure reported in literature (4%) [1] is probably biased, as the studied population belonged to tertiary and quaternary referral clinics. However, since milder forms with many features of the dysregulation may occur in PD, the real prevalence is difficult to ascertain. Hence, in order to study the prevalence and the characteristic features of HHD, we have designed a brief screening questionnaire (Table 1) that was administered for 6 months to consecutive PD patients attending our movement disorder unit. The questionnaire consists of three parts: demographic data (name, surname, sex, age, duration of disease, Hoehn and Yahr score); presence of dyskinesias and percentage of the day taken up with dyskinetic periods; five questions (yes or no) about (1) self-medication and extra doses of DRT in the last 3 months, (2) mood disorders, (3) behavioral disorders with emphasis on aggressive and violent behavior, (4) compulsive behaviors, such as compulsive Neurol Sci (2003) 24:205–206 DOI 10.1007/s10072-003-0132-0

Anterior Choroidal Artery Territory Infarction

The anterior choroidal artery (AChA) originates from the posterior wall of the internal carotid distal to the posterior communicating artery and proximal to the intracerebral carotid bifurcation. This thin artery is rarely the cause of aneurysm and only accounts for 2-5% of all aneurysms. Even though the AChA territory shows large variations among individuals, it supplies crucial motor and sensory structures, such as the internal capsule, optic tract, the posterior limb of the internal capsule, the cerebral peduncle, and the choroid plexus.

Transient Ischemic Attack Fast-track and Long-Term Stroke Risk: Role of Diffusion-Weighted Magnetic Resonance Imaging

BACKGROUND Acute ischemic lesions on diffusion-weighted magnetic resonance imaging (DWI-MRI) are reliable predictors of recurrent stroke at 90 days. However, to date, limited information on transient ischemic attack (TIA) patients with positive DWI lesions for stroke risk from 1 to 5 years is available. In this study, we evaluated the role of positive DWI lesions and vascular risk factors on stroke, cardiovascular death, and mortality at 90 days (T0), 1 year (T1), and 5 years (T2). Moreover, we also evaluated the association between stroke risk and the presence of DWI lesions. METHODS We performed an observational study on consecutive patients admitted to the emergency department of San Camillo-Forlanini Hospital, Rome, Italy, from January 2007 to November 2012. Over the study period, 4300 patients with TIA or ischemic stroke were examined by stroke specialists in an emergency room setting within 1 hour from admittance. RESULTS In 510 of 4300 patients (11.86%), a TIA was diagnosed, and 445 patients satisfy the study inclusion criteria. For all 445 patients, the mean ABCD2 score was 4.35 ± 1.30. Using DWI-MRI, we identified acute ischemic lesions in 185 patients (41.57%). We did not observe any correlation between duration of symptoms, ABCD2 score, and positive or negative DWI lesions. Positivity for DWI was not associated with the presence of diabetes mellitus, hypertension, smoking habit, or age; however, an association with weakness was observed. We documented a time-dependent increase in the absolute risk of stroke: T0: 1.35% (95% confidence interval [CI], .81-2.8); T1: 4.78% (95% CI, 2.88-7.47); T2: 9.02% (95% CI, 4.66-5.70). We did not record any difference in stroke risk in patients with positive DWI lesions: T0: hazard ratio [HR], 1.43; 95% CI, .35-5.88; log-rank P = .60; T1: HR, 1.04; 95%CI, .42-2.61; log-rank P = .91; T2: HR, .83; 95% CI, .25-2.67; log-rank P = .86. CONCLUSIONS This long-term follow-up study in TIA patients documents that both positive and negative DWI patients treated with fast-track had similar long-term risks of stroke.

Progressive sensorineural hearing loss, ataxia and anosmia as manifestation of superficial siderosis in post traumatic brain injury

Superficial siderosis (SS) of the central nervous system (CNS) is a rare condition, caused by deposition of hemosiderin in the superficial layers of the CNS due to repeated chronic subarachnoid or intraventricular hemorrhage. The ability of the brain to bio-synthesize ferritin in response to prolonged contact with hemoglobin iron is important in the pathogenesis of SS. The hindbrain structures, in particular the cerebellum, are usually most affected. Adult-onset slowly progressive gait ataxia, with cerebellar dysarthria and sensorineural hearing impairment, is the typical clinical presentation of SS. Despite extensive neuroimaging, often the bleeding source is not detected and a prior history of trauma or intradural surgery may be present. It is highly possible that a long time (decades) may elapse between the presumed inciting event and the development of symptoms, which can be attributed to SS. We report the case of a 52-year-old man, referred for brain magnetic resonance (MR) examination: over the past 3 years, the man suffered from progressive sensorineural hearing loss—with an almost total bilateral deafness, with sudden onset anosmia and mild gait unsteadiness, suggesting ataxia condition. Cognitive examination was normal. He referred a ten-year-before, mild traumatic brain injury, with no intraparenchymal brain lesions or subarachnoid bleed. Brain MRI was performed at 1.5T imager (SIEMENS Magneton) with a standardized imaging protocol including gradient-recalled-echo T2*-weighted MR images and gadolinium i.v injection. On axial and coronal T2-weighted MR images a variably thick hypointense rim completely outlined the whole brainstem, the cerebellum and basal cisterns, medulla oblongata and inferior cerebellar peduncles, and the seventh and eighth cranial nerve complexes (Fig. 1a–d). On axial T1-weighted MR images a variably thick hyperintense rim partially outlined the mesencephalon (Fig. 1e, f). The extent of involvement was more conspicuous on the axial gradientrecalled-echo T2*-weighted MR images (Fig. 2), which displayed a symmetric hypointense rim that delineated the brain stem, cerebellum, some of the lower cranial nerves, the frontopolar, fronto-basal and temporo-polar subarachnoid spaces. The involved nerves demonstrated thickened appearance on the volumetric T2-weighted MR images, as well (Fig. 3). On axial and coronal T2 images a moderate enlargement of subarachnoid spaces at the level of the vermis and cerebellar hemispheres was evident (Fig 1b–d). Axial and coronal T1-weighted MR images showed no meaningful enhancement after gadolinium administration. Total spine MR imaging, performed with a standardized imaging protocol including gradient-recalled-echo T2*-weighted MR images, ruled out the presence of spinal bleeding.

Cerebrospinal fluid analysis for Whipple's disease in patients with progressive supranuclear palsy.

The clinical picture of neurological involvement in Whipples disease (WD) may resemble progressive supranuclear palsy (PSP). We looked for WD pathogen DNA in the cerebrospinal fluid of 9 patients with a clinical diagnosis of PSP. The analysis was negative for all samples, showing that WD is not commonly involved in the aetiopathogenesis of PSP.

Reversible cerebral vasoconstriction syndrome after intercontinental airplane travel

A 51-year-old man was admitted to our Stroke Unit because of confusion, sleepiness, and weakness of the right limbs a few days after a recurrent thunderclap occipital headaches occurred for the first time during the descent phase of an intercontinental airplane flight lasting 12-h. He had no history of prior illness, and he did not take any medications or illicit drugs. He also denied any history of migraine. Clinical examination revealed normal blood pressure, normal heart rate and rhythm, and normal oxygenation. Emesis, photosensitivity, nystagmus, right upper and lower limb hemiparesis were noted, and the patient was uncooperative during the clinical examination. He reported having a recurrent severe headache occurring since the air travel. No hemorrhagic or ischemic lesions were seen on the admission head CT scan. Brain MRI–DWI showed multifocal restricted diffusion areas in the right cerebellar hemisphere, subcortical white matter of the centrum semiovale, knee and splenius of the corpus callosum, and anterior thalamus of the left hemisphere. An MRA demonstrated multiple narrowings of the basilar artery, posterior cerebral arteries and intracranial tract of the vertebral arteries. Similarly, we found multifocal segmental vasoconstrictions of the left internal carotid, right internal carotid and middle cerebral arteries (M1 tract) (Fig. 1a, c). Routine laboratory examinations revealed the presence of increased PCR values (1.83 mg/ dl) and high ESR (50 mm). Chest X-ray study, echocardiography and ultrasound examinations of the arteries of the neck were unremarkable. The patient was started on intravenous nimodipine, methylprednisolone and analgesic treatment for headaches, and after 11 days was discharged free of the signs or symptoms presented on admission. During follow-up, the patient underwent a new MRI–DWI and MRA that showed, after 20 days from acute event, complete regression of the multifocal segmental vasoconstriction of the intracerebral arteries (Fig. 1b, d). Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by a reversible and multifocal vasoconstriction of cerebral arteries and severe headaches with or without focal neurological deficits or seizures [1]. Thunderclap headache is the most common clinical feature together with nausea, vomiting, confusion and blurred vision while subarachnoid, intracerebral hemorrhage or ischemic stroke represents possible major complications. The pathogenesis remains poorly understood, but an alteration of cerebral vascular tone that occurs spontaneously or is triggered by endogenous/exogenous substances seems to be plausible [2]. Putative precipitants of RCVS are the postpartum state, vasoactive drugs, immunosuppressants or blood products, catecholamine-secreting tumors, cervical and cerebral largeartery lesions, and head trauma. Recently, a case of RCVS precipitated by airplane descent has been reported [3]. The diagnosis of RCVS is based on vascular imaging (CT, MRI or digital angiography), and the abnormalities are usually multiple and bilateral resulting in severe narrowing of intracranial arteries (classical ‘‘sausage on a string’’) [1] Treatment of RCVS consists of calcium channel blockers (nimodipine or verapamil), glucocorticoids and analgesics for headaches. To our knowledge this is the second case of RCVS associated with air flight. It suggests that RCVS may be precipitated by air travel, and that milder forms of RCVS occurring after flying may & Maria Cristina Bravi mcbravi@tiscali.it

Women Stroke Association statement on stroke

We describe the current and future objectives of the Women Stroke Association, a nonprofit multidisciplinary organization promoting research awareness on medical, psychological, and social issues concerning women affected by cerebrocardiovascular disease. In this paper, we deal with only cerebrovascular disease, whereas cardiovascular disorders will be addressed in a future paper. Gender differences in the clinical presentation of cerebrovascular diseases have been repeatedly suggested, and some treatment options may not be as effective and safe in men and women. For many years, women have either been underrepresented or excluded from randomized clinical trials, and the majority of therapeutic research has been carried on predominantly male populations. Furthermore, gender differences have been shown to contribute to different responses to cerebrovascular drugs in women when compared with men, regarding pharmacokinetics, pharmacodynamics, and physiology. In this statement, we discuss main research fields relevant to Women Stroke Associations mission and commitment, highlighting opportunities and critical from the womens health perspective. Future directions and goals of the Women Stroke Association arise from these considerations and represent the associations commitment to combating stroke.

Vascular Parkinsonism: Motor and Non-Motor Response on Treatmentwith Rotigotine

To date dopaminergic therapy has shown unsatisfactory effect on vascular Parkinsonism symptoms and related disturbances. In this case series we describe the effect of rotigotine treatment in patients with subacute onset of Parkinson’s like movement (3-6 months) after ischemic stroke of thalamus or internal capsule areas with concomitant leukoencephalopathy. Rotigotine treatment seems to improve both motor and cognitive symptoms of our sample; further studies are needed to clarify the effect of continuous dopaminergic stimulation on nigro-striatal functions in vascular patients with Parkinsonism like disturbances.