The field of neurointerventional surgery has grown in recent years. Endovascular therapies for both ischemic stroke and intracranial aneurysms have become important components in the multimodal treatment of these conditions. Familiarity with these treatment options by general neurologists is important for patient care. This article reviews recent trials and devices representing important advances in the field.

Cerebral microbleeds (CMBs) reflect an underlying angiopathy currently thought to result mainly from hypertension or from the deposition of beta-amyloid in small and micro vessel walls. This chapter describes the prevalence of, and risk factors for, CMBs and methodological issues related to their study. CMBs can be present in up to 80% of a clinical hemorrhagic stroke sample. Most studies with a reasonable distribution of subject age and sample size show CMB prevalence increases with age. There is robust evidence that high blood pressure, measured in different ways, is a risk factor for CMB. Most genetic diseases with increased susceptibility to CMBs are rare. The only candidate susceptibility gene identified as risk modifying is APOE. Research on risk factors for CMBs will bring into better focus issues related to comorbidity with other vascular and neurodegenerative lesions and location and number of lesions.

DEVELOPMENT OF MESIAL TEMPORAL LOBE EPILEPSY IN CHOREA-ACANTHOCYTOSIS To the Editor: I read with interest the article by Scheid et al.,1 who describe 3 patients with choreaacanthocytosis (ChAc) whose initial symptomatology included partial complex seizures (PCS). The brain MRI, in addition to caudate atrophy, showed mesial temporal sclerosis (MTS) in all 3 cases. As the authors suggest, it is intriguing that a pathologic process with a predilection for the basal ganglia should cause focal abnormalities in the hippocampal region. The authors posit several theories but do not mention genetic abnormalities, which have been theorized as a contributing factor in MTS. In addition, since MTS is the most common pathology in temporal lobe seizures, its occurrence in ChAc may be coincidental. The authors doubt the significance of the seizures themselves as a culprit behind MTS in their patients. However, seizures— particularly when prolonged—are known to cause MTS. Did the authors document the duration of their patients’ seizure episodes? In addition, were nasopharyngeal electrodes utilized in any of the EEG recordings to confirm the mesial temporal region as the primary epileptogenic zone? Video-EEG would indicate a hemispheric concordance between the topography of the ictal onset and the location of the MTS, as occasional discord may occur between these 2 parameters in PCS. Recurrent seizures may have contributed to the development of MTS in the present series, perhaps through the kindling process. The absence of status epilepticus history in these patients does not weaken this theory. Since the pathogenesis of MTS in ChAc is unclear, pharmacotherapy may have been preferable to epilepsy surgery.

Background: There are many unresolved issues in the diagnosis and treatment of persons with traumatic brain injury (TBI) in its post-acute and chronic phases. This article deals with two problems of clinical importance: (i) the interrelationships between structural brain damage, brain function, and clinical outcome, and (ii) post-traumatic epilepsy.Methods: Exploratory, retrospective analysis of clinical, neuroradiological (MRI), and neuropsychological data of all patients with TBI who were treated in a cognitive neurology outpatient clinic of a German university hospital over a period of 12 years (n=320).Results: 156 patients (48.8%) had brain contusions, 83 of them (25.9%) as the sole neuroradiological abnormality. Traumatic micro-hemorrhages were seen in 148 patients (46.2%) and were the sole neuroradiological abnormality in 79 of them (24.7%). 49 patients (15.3%) had no structural brain lesion. There was no obvious correlation between the neuroradiological findings and the clinical outcome, as measured either by a general outcome parameter such as the extended Glasgow Outcome Scale (GOSE) or by neuropsychological testing. 47 patients (14.7%) had post-traumatic epilepsy; its occurrence was positively correlated with the presence of brain contusions, but not with an isolated diagnosis of diffuse axonal injury (DAI).Conclusion: A comparison of the findings of neuroradiological studies and neuropsychological tests among patients in the chronic phase of traumatic brain injury does not reveal any simple relationship between structural and functional brain abnormalities. Diffuse axonal injury is often present in combination with other findings, and it may well be the only structural abnormality in many cases; therefore, all symptomatic patients should undergo MRI of the brain. Patients with isolated DAI seem to be less prone to post-traumatic epilepsy than those with brain contusions.

Behavioral and executive dysfunctions are typical symptoms of frontotemporal lobar degeneration, associated with its subtypes frontotemporal and semantic dementia. Although both functions depend on the frontal lobes, no study has yet compared their neural correlates in frontotemporal lobar degeneration. Accordingly, we correlated clinical scores of behavioral and executive deficits with glucose utilization as measured by [18F]fluorodeoxyglucose positron emission tomography in 17 patients with frontotemporal lobar degeneration and 9 age- and sex-matched control subjects. Impairment in executive functions was measured by the Behavioral Assessment of the Dysexecutive Syndrome, a modified Stroop paradigm and/or the Tower of Toronto Test. Behavioral deficits were examined with the Neuropsychiatric Inventory. Executive dysfunction was correlated with diminished glucose utilization in frontomedial and frontolateral cortices. Brain regions included the anterior cingulate and midcingulate gyri, anterior medial frontal cortex, and left frontolateral cortex. Behavioral deficits were associated with mainly frontomedial networks, particularly the anterior medial frontal cortex, gyrus rectus, and area subcallosa. Our pilot study reveals partially overlapping neural correlates of executive and behavioral dysfunction in frontotemporal lobar degeneration. The results suggest that some behavioral deficits, namely disinhibition and appetite and eating abnormalities, are particularly related to executive dysfunction. This hypothesis might be further explored in studies involving larger patient groups.

Sir, We would like to bring to your attention our experience with a 47-year-old female patient who presented acutely with a left temporal haemorrhage for which no cause was identified on CT-angiography (Fig. 1). Four months later, MRI with TOF-MRA was also normal (Fig. 2) but surprisingly, an MRI, a further 4 months later (Fig. 3), was highly suspicious of a DAVF and this diagnosis was confirmed by DSA (Fig. 4). She was scheduled for treatment by embolisation but when DSA was repeated 3 months later, immediately prior to treatment, the DAVF was no more. This finding was confirmed on further MRI/ MRA scans. We concluded that the contradictory imaging findings in our patient most likely reflected a dynamic process of an initial closure, re-opening, and repeat spontaneous closure of a symptomaticDAVF during the follow-up period of 12months. The results can be regarded reliable, since identical parameters were used for repeat MRI and MRA, and because MRI/MRA findings matched the DSA findings when the procedures were performed in close temporal relationship. An alternative hypothesis—namely, that there originally might have been a venous congestive bleeding after a thrombosis of a major cortical vein draining into the transverse sinus and a secondary evolvement of a DAVF—seems unlikely since (1) there was an unremarkable initial CTA; (2) in retrospect the patient reported a left-sided pulsatile tinnitus, which had started several months prior to the haemorrhage. Nevertheless, the possibility that the negative first MRI/ MRA reflects an insufficient sensitivity of the imaging technique must be considered. The potential invisibility of slow flow DAVFs in the TOF modality has been discussed previously; however, the heightened sensitivity of the respective source images has been emphasized [1, 2]. In our patient, both TOF-MRA and the source images were unremarkable. Missing or insufficient visualization of DAVFs using TOF-MRA in the subacute phase of cerebral haemorrhages is usually attributed to the T1-shortening effect due to haemorrhage or the mass effect of the haemorrhage itself [3]. In our patient, the T1-shortening Neuroradiology (2009) 51:131–133 DOI 10.1007/s00234-008-0481-8

Steroids have been suggested as a therapeutic option for superficial siderosis of the central nervous system (SSCNS) without identifiable bleeding source. Longitudinal observational data of a patient with idiopathic SSCNS who was repeatedly treated with methylprednisolone over a course of 2 years are reported. The case history is critically discussed on the background of the sparse literature. In conclusion, if at all, there is only a limited and temporary, mostly subjective clinical response to steroids in SSCNS. Systematic studies of this medication in SSCNS do not seem warranted. Pathophysiological considerations hopefully may lead to more helpful medications for this chronic and debilitating disorder.

Mutations in the Notch3 gene in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) usually involve cysteine residues.1 We report a cysteine-sparing mutation (A1020P) in association with a CADASIL-compatible phenotype in two German families. ### Case histories. The index patient 1 is a 49-year-old woman with a medical history of sensorineural hearing loss of adolescent onset. At the age of 34, arterial hypertension was diagnosed. Since her 40s she reported episodes of unilateral headache, vertigo, and nausea. In addition, the patient had intermittent paresthesias and weakness of the left extremities, reduced fine motor skills, cognitive slowing, and impaired memory. MRI in 2003 revealed widespread T2-hyperintense white matter lesions (WMLs) (figure, A). Electron microscopic examination of a skin biopsy showed deposits of osmiophilic material between the smooth-muscle cells of small arteries (figure e-1 on the Neurology ® Web site at www.neurology.org), and immunostaining with a Notch3 monoclonal antibody showed granular labeling of vascular smooth-muscle cells (figure e-2). Figure Axial T2-weighted (A, C, E) and fluid-attenuated inversion recovery MRIs (B, D) of the index patient (A), the index patient’s mother (B), and patient 2 (D), compatible with widespread ischemic leukoaraiosis Note especially the absence of white matter lesions (WMLs) in the anterior temporal lobes in all three individuals. The MRI of the index patient’s uncle (C), although characterized as a mutation carrier, only showed a slight pallor of the white matter and dilated Virchow Robin spaces. (E) MRI of the father of the …

Chorea-acanthocytosis (ChAc) is a hereditary movement disorder that is caused by recessive mutations in the VPS13A gene on chromosome 9q21, encoding for chorein.1 Epilepsy is currently not considered a core clinical feature of ChAc, although approximately 40% of patients are affected by seizures. We report on 3 patients with mesial temporal lobe epilepsy as the first, predominant clinical indication, and in 2 of the patients so far the sole clinical symptom of the disease. For the first time in this context, a pathologic process in the medial temporal lobes, leading to hippocampal atrophy, is unequivocally documented. ### Case histories. Patient 1 was a 14-year-old boy when, after repeat epigastric sensations, a first seemingly generalized tonic-clonic seizure led to hospital admission. Several weeks prior, he had started to complain about repetitive deja vu. Repeat EEG studies most frequently showed normal 10/second alpha-, but also paroxysmal generalized theta-, right hemispheric delta-, and right temporo-occipital spike- and sharp-wave activity. Until the present, 4 years after the initial onset, 2 more tonic-clonic seizures have occurred, and, with variable frequencies, the patient continues to report rising epigastric sensations, deja vu, and amnesic auras. Medical treatment currently consists of the administration of topiramate (300 mg/day). Patient 2, the older brother of patient 1, had a first complex partial seizure at 23 years of age. His history was unremarkable; no febrile seizures had been reported. Lately, however, he has also reported repeat episodes of deja vu. EEG findings ranged from normal 9/second alpha- to intermittent and continuous spike- and sharp-wave activity over both frontotemporal regions. Apart from rare epigastric auras, the patient is presently (i.e., 8 months after his first epileptic seizure) seizure-free under levetiracetam (2,500 mg/day). Patient 3 is a 39-year-old man …

The etiology of abnormal belching is not known. Currently, it is being subsumed under “functional gastroduodenal disorders.” Here, we report the unusual case history of a patient who developed aerophagia and consecutive excessive belching in association with herpes simplex encephalitis. The case report adds to the limited information about potential organic geneses of belching. Implications for possible medical therapies are discussed.

Toxocariasis of the central nervous system is usually characterized by an eosinophilic meningitis, encephalitis or myelitis. We here report a patient with an at least 7 years history of unexplained neurologic signs and symptoms. MRI showed a cystic lesion in the left thalamus, compatible with a parasitic infection. Blood and CSF analyses were positive for Toxocara canis IgG Western-blot, but were otherwise unremarkable. The case report raises the question whether there are chronic or late variants of this disease.

A patient with ulcerative colitis (UC) is reported who developed a severe neurologic disorder involving the peripheral and the central nervous system. Atypical antineutrophil cytoplasmatic autoantibodies (ANCA) were found that were positive for proteinase 3 (PR3). Cerebral vasculitis and a demyelinating disorder like acute disseminated encephalomyelitis (ADEM) were the main differential diagnoses for the central nervous system abnormalities. Although there is no proof of a causal relationship, the case history supports the assumption that UC may lead to (auto)immunologically triggered neurologic disease.

Traumatic microbleeds (TMBs) can be regarded as a radiological marker of diffuse axonal injury (DAI). We sought to investigate the impact of the field strengths on the depiction of TMBs by T2*-weighted gradient echo magnetic resonance imaging (MRI). By the use of comparative MRI of 14 patients (age range, 22-62 years) on 1.5- and a 3 T (Tesla) systems at a median time interval of 61 months after traumatic brain injury (TBI), we found 239 (range 0.5-48.5, median 7.5) TMBs at 1.5 T, and 470 (range 2-118, median 18.5) TMBs at 3 T, respectively (p=0.001). However, in all but one patients MRI at 1.5 T also clearly showed TMBs. A significant negative correlation between the number of TMBs and the time interval TBI-MRI was observed, which was weaker for the imaging at 3 T (r(s)=-0.798; p=0.001; and r(s)=-0.649; p=0.012, respectively). In conclusion, T2*-weighted gradient-echo MRI at 3 T is superior as compared to MRI at 1.5 T for the detection of TMBs. Nevertheless, in clinical practice, MRI at 1.5 T seems to be sufficient for this purpose. MRI at 3 T may be appropriate if there is a strong clinical suspicion of DAI, despite unremarkable routine MRI, and possibly also if evidence of DAI is sought after a long interval from trauma.

Wir berichten einen Patienten mit langjährig bestehender Colitis ulcerosa (CU), der im Anschluss an einen akuten Schub der Kolitis eine schwere neurologische Erkrankung unter Einschluss des peripheren und zentralen Nervensystems entwickelte. Proteinase-3(PR-3)-positive atypische antineutrophile zytoplasmatische Autoantikörper (ANCA) waren im Serum des Patienten präsent. ZNS-Vaskulitis und demyelinisierende Erkrankungen wie die akute demyelinisierende Enzephalomyelitis (ADEM) sind die wichtigsten Differenzialdiagnosen für die ZNS-Manifestation. Die Kasuistik ist nicht beweisend, kann aber die Annahme der Existenz (auto-)immun getriggerter neurologischer Manifestationen bei CU weiterunterstützen.

It is well known that traumatic brain injury particularly affects the frontal lobes. Consequently, patients often suffer from executive dysfunction and behavioral disturbances. Accordingly, our study aimed at investigating patients after traumatic brain injury with two tasks involving different functional processes and structural networks supported by the frontal lobes. Two paradigms were applied: the Stroop color-word task and a task in which subjects had to inhibit imitative response tendencies. We selected a patient group solely with diffuse axonal injury, as this type of injury is homogenous and is correlated with cognitive dysfunction more than focal contusions. To evaluate long-term effects most relevant for rehabilitation, we selected a patient group whose brain injuries dated back several years. Our results show that patients with diffuse axonal injury inhibited imitative responses more successfully than control subjects, whereas executive processes examined with the Stroop task were unaltered. Interestingly, impairments were tightly correlated both with the length of the post-traumatic amnesia predicting outcome in traumatic brain injury and with behavioral disturbances. Impairments in the imitation-inhibition task may indicate alterations in an anterior frontomedian neural network even years after traumatic brain injury.