Recent Advances in Cerebellar Ischemic Stroke Syndromes Causing Vertigo and Hearing Loss

Cerebellar ischemic stroke is one of the common causes of vascular vertigo. It usually accompanies other neurological symptoms or signs, but a small infarct in the cerebellum can present with vertigo without other localizing symptoms. Approximately 11 % of the patients with isolated cerebellar infarction simulated acute peripheral vestibulopathy, and most patients had an infarct in the territory of the medial branch of the posterior inferior cerebellar artery (PICA). A head impulse test can differentiate acute isolated vertigo associated with PICA territory cerebellar infarction from more benign disorders involving the inner ear. Acute hearing loss (AHL) of a vascular cause is mostly associated with cerebellar infarction in the territory of the anterior inferior cerebellar artery (AICA), but PICA territory cerebellar infarction rarely causes AHL. To date, at least eight subgroups of AICA territory infarction have been identified according to the pattern of neurotological presentations, among which the most common pattern of audiovestibular dysfunction is the combined loss of auditory and vestibular functions. Sometimes acute isolated audiovestibular loss can be the initial symptom of impending posterior circulation ischemic stroke (particularly within the territory of the AICA). Audiovestibular loss from cerebellar infarction has a good long-term outcome than previously thought. Approximately half of patients with superior cerebellar artery territory (SCA) cerebellar infarction experienced true vertigo, suggesting that the vertigo and nystagmus in the SCA territory cerebellar infarctions are more common than previously thought. In this article, recent findings on clinical features of vertigo and hearing loss from cerebellar ischemic stroke syndrome are summarized.     

The Usefulness of the TOAST Classification and Prognostic Significance of Pyramidal Symptoms During the Acute Phase of Cerebellar Ischemic Stroke

Cerebellar stroke is a rare condition with very nonspecific clinical features. The symptoms in the acute phase could imitate acute peripheral vestibular disorders or a brainstem lesion. The aim of this study was to assess the usefulness of the Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification in cerebellar stroke and the impact of clinical features on the prognosis. We retrospectively analyzed 107 patients with diagnosed ischemic cerebellar infarction. We studied the clinical features and compared them based on the location of the ischemic lesion and its distribution in the posterior interior cerebellar artery (PICA), superior cerebellar artery (SCA), and anterior inferior cerebellar artery (AICA) territories. According to the TOAST classification, stroke was more prevalent in atrial fibrillation (26/107) and when the lesion was in the PICA territory (39/107). Pyramidal signs occurred in 29/107 of patients and were more prevalent when the lesion was distributed in more than two vascular regions (p?=?0.00640). Mortality was higher among patients with ischemic lesion caused by cardiac sources (p?=?0.00094) and with pyramidal signs (p?=?0.00640). The TOAST classification is less useful in assessing supratentorial ischemic infarcts. Cardioembolic etiology, location of the ischemic lesion, and pyramidal signs support a negative prognosis.     

Impaired Tilt Suppression of Post-Rotatory Nystagmus and Cross-Coupled Head-Shaking Nystagmus in Cerebellar Lesions: Image Mapping Study

We sought to determine the cerebellar structures responsible for tilt suppression of post-rotatory nystagmus. We investigated ocular motor findings and MRI lesions in 73 patients with isolated cerebellar lesions who underwent recording of the vestibulo-ocular reflex (VOR) using rotatory chair tests. Tilt suppression of post-rotatory nystagmus was diminished in 27 patients (27/73, 37.0 %). The gains of the VOR and the TCs of per- and post-rotatory nystagmus did not differ between the patients with diminished and with normal tilt suppression. The patients with impaired tilt suppression showed perverted (“cross-coupled”) head-shaking nystagmus (pHSN) and central positional nystagmus (CPN) more frequently than those with normal responses. Tilt suppression was impaired in five (71.4 %) of the seven patients with isolated nodulus and uvular infarction. Probabilistic lesion-mapping analysis showed that the nodulus and uvula are responsible for tilt suppression. Impaired tilt suppression may be ascribed to disruption of cerebellar contribution to the vestibular velocity-storage mechanism, which integrates information from the semicircular canals and otolith organs to help derive the brain’s estimate of the head orientation relative to the pull of gravity.     

The Diagnosis and Natural History of Multiple System Atrophy,Cerebellar Type

The objective of this study was to identify key features differentiating multiple system atrophy cerebellar type (MSA-C) from idiopathic late-onset cerebellar ataxia (ILOCA). We reviewed records of patients seen in the Massachusetts General Hospital Ataxia Unit between 1992 and 2013 with consensus criteria diagnoses of MSA-C or ILOCA. Twelve patients had definite MSA-C, 53 had possible/probable MSA-C, and 12 had ILOCA. Autonomic features, specifically urinary urgency, frequency, and incontinence with erectile dysfunction in males, differentiated MSA-C from ILOCA throughout the disease course (p?=?0.005). Orthostatic hypotension developed later and differentiated MSA-C from ILOCA (p?<?0.01). REM sleep behavior disorder (RBD) occurred early in possible/probable MSA-C (p?<?0.01). Late MSA-C included pathologic laughing and crying (PLC, p?<?0.01), bradykinesia (p?=?0.01), and corticospinal findings (p?=?0.01). MRI distinguished MSA-C from ILOCA by atrophy of the brainstem (p?<?0.01) and middle cerebellar peduncles (MCP, p?=?0.02). MSA-C progressed faster than ILOCA: by 6 years, MSA-C walker dependency was 100 % and ILOCA 33 %. MSA-C survival was 8.4?±?2.5 years. Mean length of ILOCA illness to date is 15.9?±?6.4 years. A sporadic onset, insidiously developing cerebellar syndrome in midlife, with autonomic features of otherwise unexplained bladder dysfunction with or without erectile dysfunction in males, and atrophy of the cerebellum, brainstem, and MCP points strongly to MSA-C. RBD and postural hypotension confirm the diagnosis. Extrapyramidal findings, corticospinal tract signs, and PLC are helpful but not necessary for diagnosis. Clarity in early MSA-C diagnosis can prevent unnecessary investigations and facilitate therapeutic trials.     

Infarcts in the territory of the lateral branch of the posterior inferior cerebellar artery.

The territory of the lateral branch of the posterior inferior cerebellar artery (1PICA) supplies the anterolateral region of the caudal part of the cerebellar hemisphere. Because infarcts in the territory of the 1PICA have rarely been studied specifically, 10 patients with this type of infarct are reported. An 1PICA infarct was isolated in only three patients, whereas it was associated with brainstem infarct in four, with occipital infarct in one, and with multiple infarcts in two patients. The most common symptom at onset was acute unsteadiness and gait ataxia without rotatory vertigo (six patients). Unilateral cerebellar dysfunction was found in all patients, with limb ataxia (nine patients), dysdiadochokinesia (five patients), and ipsilateral body sway (four patients), but dysarthria and primary position nystagmus were notably absent. In the patients with a coexisting infarct in the brainstem, cranial nerve and sensorimotor dysfunction was prominent and often masked the signs of cerebellar dysfunction. Unlike other infarcts in the PICA territory, 1PICA territory infarcts were mainly associated with vertebral artery atherosclerosis (six patients), whereas cardiac embolism was less common (three patients). Unilateral limb ataxia without dysarthria or vestibular signs suggests isolated 1PICA territory infarction and should allow its differentiation from other cerebellar infarcts.     

Perverted Head-Shaking and Positional Downbeat Nystagmus in Essential Tremor

Even though the pathophysiology is not completely understood, cerebellar dysfunction has been invoked in essential tremor (ET). We evaluated cerebellar dysfunction in ET with the presence of perverted head-shaking (pHSN) and positional downbeat nystagmus (pDBN) which are known to reflect cerebellar dysfunction. First, we reviewed the videooculography (VOG) of 185 patients with ET from March 2007 to April 2010. Seventeen of 28 patients with pHSN and pDBN were followed up for at least a 1.8-year interval from baseline to determine the clinical course. And then, we recruited 52 consecutive patients with ET and compared their ocular motor findings with 51 normal controls using VOG. Among the 185 patients with ET, 28 (15.1 %) showed pHSN (n?=?23, 12.4 %) or pDBN (n?=?8, 4.3 %). Seventeen of them who were followed up did not develop Parkinsonism or other neurologic deficits during the observation period. The subsequent case-control study showed a higher prevalence of pHSN or pDBN (11/52, 21.2 %, pHSN in nine and pDBN in five) in patients with ET than in the normal controls (2/51, 3.9 %, pHSN only, P?=?0.015). The tremor rating scale or involved body sites did not differ between the patients with and without pHSN/pDBN. pHSN and pDBN were more common in patients with ET than in the normal controls. This result supports that cerebellar dysfunction is associated with ET.     

Non-motor and Extracerebellar Features in Spinocerebellar Ataxia Type 2

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant degenerative disease. Pathological studies have demonstrated not only cerebellar and brainstem atrophy, but substantia nigra, motoneurons, basal ganglia, thalamus, and peripheral nerves involvement. These findings may explain non-motor and extra-cerebellar features in SCA2. We accessed the non-motor symptoms and extra-cerebellar signs in SCA2 patients in order to provide a better understanding on pathophysiological mechanisms and natural history of brain degeneration in the disease. Thirty-three SCA2 patients were evaluated and compared with 26 healthy subjects. We investigated the following variables: sleep disorders, cognitive deficit, olfactory impairment, urinary dysfunction, psychiatric symptoms, cramps, pain, movement disorders, and weight loss. SCA2 had a high frequency of REM sleep behavior disorder (48.48 %, N?=?16) as well as excessive daytime sleepiness (42.42 %, N?=?14). Chorea was present in 15.15 % (N?=?5), dystonia in 27.27 % (N?=?9), and parkinsonism in 27.27 % (N?=?9). Slow saccadic pursuit was present in 87.87 % (N?=?29) and ophtalmoparesis in 78.78 % (N?=?26) of patients. Regarding sleep disorders, 18.18 % (N?=?6) of patients had restless leg syndrome. Dysphagia was present in 39.39 % (N?=?13), weight loss 24.24 % (N?=?8), and urinary dysfunction 27.27 % (N?=?9). Cramps was present in only 6 % of patients (N?=?2). This study highlighted the high frequency of non-motor symptoms and extra-cerebellar signs in SCA2. Our findings demonstrate the widespread of nervous system involvement in SCA2 patients and contribute to better understand the natural history of brain degeneration in this genetic condition.     

以眩晕为首发症状的小脑梗死临床类型及供血区分布

目的:探讨以眩晕为首发症状的小脑梗死临床类型及病灶供血区分布特征。方法:对26例经MRI确诊、以眩晕为首发症状的小脑梗死患者的临床资料进行回顾性分析。结果:将眩晕为首发症状的小脑梗死分为2种临床类型:①稳定型:单纯自发性持续性眩晕伴平衡失调(19/26例,73.1%);②进展型:以持续性眩晕、平衡失调为首发症状,起病2d后伴有延迟神经功能受累症状(7/26例,26.9%)。梗死病灶以小脑后下动脉内侧支(16/26例,61.5%)受累最为常见;其次为小脑前下动脉区(6/26例,23.1%)及小脑上动脉区(2/26例,7.7%)。未见多发小脑供血动脉区梗死患者以单纯眩晕为首发症状。结论:以眩晕为首发症状的小脑梗死以小脑后下动脉内侧支受累最为常见,绝大多数患者呈良性病程,但需警惕可能出现的延迟神经功能受累症状和体征。     

Mastication-induced vertigo and nystagmus

Even though trigeminovestibular connections are well established in animals, mastication-induced dizziness has been described only as a vascular steal phenomenon in humans. We determined induction or modulation of nystagmus in two index patients with mastication-induced vertigo, 12 normal controls, and 52 additional patients with peripheral (n = 38, 26 with vestibular neuritis/labyrinthitis and 12 with Meniere’s disease) or central (n = 14, 11 with Wallenberg syndrome, two with cerebellar infarction, and one with pontine infarction) vestibulopathy during their acute or compensated phase. Both index patients developed mastication-induced vertigo after near complete resolution of the spontaneous vertigo from presumed acute unilateral peripheral vestibulopathy. The nystagmus and vertigo gradually built up during mastication and dissipated slowly after cessation of mastication. Brain MRI and cerebral angiography were normal in these patients. Mastication did not induce nystagmus in normal controls. However, mastication induced nystagmus in five (24 %) of the 21 patients without spontaneous nystagmus (SN) but with a previous history of a vestibular syndrome, and either increased (21/31, 68 %) or decreased (7/31, 23 %) the SN in almost all the patients (28/31, 90 %) with SN. Mastication may induce significant vertigo and nystagmus in patients with a prior history of acute vestibulopathy. The induction or modulation of nystagmus by mastication in both peripheral and central vestibulopathies supports trigeminal modulation of the vestibular system in human. The gradual build-up and dissipation suggest a role of the velocity storage mechanism in the generation of mastication-induced vertigo and nystagmus.     

Deciphering the causes of sporadic late-onset cerebellar ataxias: a prospective study with implications for diagnostic work

The management of sporadic late-onset cerebellar ataxias represents a very heterogeneous group of patients and remains a challenge for neurologist in clinical practice. We aimed at describing the different causes of sporadic late-onset cerebellar ataxias that were diagnosed following standardized, exhaustive investigations and the population characteristics according to the aetiologies as well as at evaluating the relevance of these investigations. All patients consecutively referred to our centre due to sporadic, progressive cerebellar ataxia occurring after 40 years of age were included in the prospective, observational study. 80 patients were included over a 2 year period. A diagnosis was established for 52 patients (65%) corresponding to 18 distinct causes, the most frequent being cerebellar variant of multiple system atrophy (n = 29). The second most frequent cause was inherited diseases (including spinocerebellar ataxias, late-onset Friedreich’s disease, SLC20A2 mutations, FXTAS, MELAS, and other mitochondrial diseases) (n = 9), followed by immune-mediated or other acquired causes. The group of patient without diagnosis showed a slower worsening of ataxia (p < 0.05) than patients with multiple system atrophy. Patients with later age at onset experienced faster progression of ataxia (p = 0.001) and more frequently parkinsonism (p < 0.05) than patients with earlier onset. Brain MRI, DaT scan, genetic analysis and to some extent muscle biopsy, thoracic-abdominal-pelvic tomodensitometry, and cerebrospinal fluid analysis were the most relevant investigations to explore sporadic late-onset cerebellar ataxia. Sporadic late-onset cerebellar ataxias should be exhaustively investigated to identify the underlying causes that are numerous, including inherited causes, but dominated by multiple system atrophy.     

Blood brain barrier impairment is associated with cerebrospinal fluid markers of neuronal damage in HIV-positive patients

Blood brain barrier impairment occurs early in the course of infection by HIV and it may persist in a subset of patients despite effective antiretroviral treatment. We tested the hypothesis that HIV-positive patients with dysfunctional blood brain barrier may have altered biomarkers of neuronal damage. In adult HIV-positive highly active antiretroviral treatment (HAART)-treated patients (without central nervous system infections and undergoing lumbar punctures for clinical reasons) cerebrospinal fluid albumin to serum ratios (CSAR), total tau, phosphorylated tau, 1–42 beta amyloid, and neopterin were measured. In 101 adult patients, cerebrospinal fluid-to-serum albumin ratios were 4.8 (3.7–6.1) with 12 patients (11.9 %) presenting age-defined impaired blood brain barrier. A significant correlation was observed between CSAR and total tau (p?=?0.005), phosphorylated tau (p?=?0.008), and 1–42 beta amyloid (p?=?0.040). Patients with impaired blood brain barrier showed significantly higher total tau (201.6 vs. 87.3 pg/mL, p?=?0.010), phosphorylated tau (35.3 vs. 32.1 ng/mL, p?=?0.035), and 1–42 beta amyloid (1134 vs. 830 pg/mL, p?=?0.045). Despite effective antiretroviral treatment, blood brain barrier impairment persists in some HIV-positive patients: it is associated with markers of neuronal damage and it was not associated with CSF neopterin concentrations.     

Impaired Modulation of the Otolithic Function in Acute Unilateral Cerebellar Infarction

To define the cerebellar contribution in modulating the otolithic signals, we investigated the otolithic function in 27 patients with acute unilateral cerebellar infarctions in the territory of the posterior inferior cerebellar artery (PICA, n?=?17, 63 %), combined PICA and superior cerebellar artery (SCA) (n?=?7, 30 %), SCA (n?=?2, 7 %), and anterior inferior cerebellar artery (n?=?1, 4 %) from 2010 to 2012. The patients had evaluation of the ocular tilt reaction [head tilt, ocular torsion (OT), and skew deviation], tilt of the subjective visual vertical (SVV), cervical vestibular evoked myogenic potentials (VEMPs) in response to air conducted tone bursts, and ocular VEMPs induced by tapping the head at AFz. The evaluation was completed within 2 weeks after symptom onset. Patients often showed OT or SVV tilt (15/27, 55.6 %) that was either ipsi- (n?=?6) or contraversive (n?=?9). Overall, there were no differences in the amplitudes and latencies of cervical and ocular VEMPs between the ipsi- and contralesional sides. However, individual analyses revealed frequent abnormalities of cervical (11/27, 41 %) and/or ocular (9/27, 33 %) VEMPs. While 11 (73 %) of the 15 patients with the OTR/SVV tilt showed abnormalities of cervical (n?=?9) and/or ocular (n?=?7) VEMP responses, only three (25 %) of the 12 patients without the OTR/SVV tilt had abnormal cervical (n?=?2) and/or ocular (n?=?2) VEMPs (73 % vs. 25 %, Fisher’s exact test, p?=?0.021). The concordance rate in the results of cervical and ocular VEMPs was marginally significant (19/27, 70 %, p?=?0.052, binominal). Unilateral cerebellar lesions may generate otolithic imbalances, as evidenced by the OTR/SVV tilt and asymmetric ocular or cervical VEMP responses, but without directionality according to the lesion side. Patients with the OTR/SVV tilt had abnormal VEMPs more often than those without.     

Psychosis in Spinocerebellar Ataxias: a Case Series and Study of Tyrosine Hydroxylase in Substantia Nigra

Spinocerebellar ataxias are a genetically heterogeneous group of degenerative diseases typically characterized by progressive ataxia and to various degrees, neuropathy, amyotrophy, and ocular abnormalities. There is increasing evidence for non-motor manifestations associated with cerebellar syndromes including cognitive and psychiatric features. We studied a retrospective clinical case series of eight subjects with spinocerebellar ataxias (SCAs) 2, 3, 7, and 17, all displaying features of psychosis, and also measured tyrosine hydroxylase (TH) staining of the substantia nigra (SN) at autopsy, among four of the subjects. We hypothesized that increased dopamine production in the SN may underlie the pathophysiology of psychosis in SCAs, given evidence of increased dopamine production in the SN in schizophrenia, as measured by TH staining. We analyzed differences in TH staining between the SCA psychosis cohort (n = 4), a heterogeneous ataxic cohort without psychosis (n = 22), and non-diseased age- and sex-matched control group (n = 12). SCA subjects with psychosis did not differ significantly in TH staining versus ataxic cases without psychosis. There was, however, increased TH staining in the ataxic cohort with and without psychosis (n = 26), compared to non-diseased controls (n = 12). Psychotic features were similar across subjects, with the presence of delusions, paranoia, and auditory hallucinations. Our findings are preliminary because of small numbers of subjects and variable neuropathology; however, they suggest that psychosis is a clinical feature of SCAs and may be under-recognized. While the underlying pathophysiology remains to be fully established, it may be related to extra-cerebellar pathology, including a possible propensity for increased dopamine activity in the SN.     

Anatomy of the cerebellar arteries

Origin, course and distribution of the cerebellar arteries and of their branches are described. Anatomical drawings of the territory of these arteries are presented. They are based on a neuropathological study of 64 cases of cerebellar infarctions, the detailed study of which is reported elsewhere. The superior cerebellar artery (SCA) supplies a small brain stem territory, located on the dorsal tegmentum and the tectum of the upper part of the pons. The superior part of the cerebellum supplied by this artery includes the following lobules: lobulus anterior, lobulus simplex, lobulus semilunaris superior, and, in the vermis, lobulus centralis, culmen and clivus. The dentate nucleus belongs to this territory. The anterior inferior cerebellar artery (AICA) irrigates a ponto-cerebellar territory. It usually supplies the lateral territory of the lower part of the pons, the middle cerebellar peduncle, the flocculus and the neighbouring lobules of cerebellum. When the posterior inferior cerebellar artery (PICA) is hypoplastic, AICA takes over the territory usually supplied by the lateral branch of the PICA. The PICA always gives rami to the group of arteries supplying the dorsal medullary territory, but rarely participates to the supply of the lateral medullary territory. It supplies the lobulus semilunaris inferior, the lobulus gracilis, the lobulus biventer, the tonsilla cerebelli, and, in the vermis, the clivus, the tuber, the pyramis, the uvula and the nodulus. PICA never supplies the dentate nucleus. The flocculo-nodular lobe is usually supplied by 2 arteries: the flocculus is supplied by the AICA and the nodulus is supplied by the PICA.     

Association of post stroke depression with social factors,insomnia, and neurological status in Chinese elderly population

The purpose of this study was to investigate the association of post stroke depression (PSD) with social factors, insomnia, and neurological status among elderly Chinese patients with ischemic stroke. Six hundred and eight patients over 60 years of age, who had suffered from a first episode of ischemic stroke within 7 days, were enrolled into the study. They were divided into PSD and non-PSD groups according to the Self-rating Depression Scale (SDS) scores. The association of PSD with social factors, insomnia, and neurological status was analyzed using multivariable logistic regression analysis. Compared with the patients who did not develop PSD, those with PSD reported adverse life events more frequently, and more subjects with PSD lived alone, had left carotid artery infarction and cortical infarction (P < 0.05), history of insomnia, and high National Institute of Health Stroke Scale (NIHSS) scores and low Barthel Index (BI) scores (P < 0.01). The multivariable logistic regression analysis showed that the occurrence of PSD was associated with a history of insomnia (HR = 1.59, 95 % CI 1.12–2.36, P < 0.01), NIHSS scores (HR = 2.45, 95 % CI 1.42–3.91, P < 0.01) and BI scores (HR = 2.56, 95 % CI 1.39–4.25, P < 0.01). Insomnia and the degree of neurological deficit were associated with PSD in an elderly population of Chinese people.     

Fast Progression of Cerebellar Atrophy in PLA2G6-Associated Infantile Neuronal Axonal Dystrophy

Infantile neuronal axonal dystrophy (INAD) is characterized by progressive cerebellar atrophy. MRI has been recommended as a marker of disease progression in cerebellar diseases. We performed a longitudinal brain volumetry study in a couple of bicorial twins with PLA2G6-positive INAD. Brain volumetry was calculated with FreeSurfer software on 3T T1-weighted images acquired at age 28 (t ₀) and 36 months (t ₁) in patient 1 and at age 22 (t ₀) and 31 months (t ₁) in patient 2. Data at t ₀ were compared to those obtained in 18 control children aged 14–44 months with normal MRI. At t ₀, both patients showed markedly lower cerebellar volume compared to controls. At t ₁, both patients exhibited a remarkable decrease of cerebellar volume (?25.8% in patient 1; ?16.5% in patient 2) and of frontal (?6.8% in patient 1 and ?3.3% in patient 2) and occipital (?9.8% in patient 1 and ?9.1% in patient 2) cortical GM volume. Our MRI morphometry study indicates that INAD is characterized by a remarkably fast progression of cerebellar atrophy and mild atrophy of the frontal and occipital cortex presumably secondary to deafferentation in the cortical-pons-cerebellum-rubro-thalamus-cortical circuit and visual pathways.     

Efficacy of mastoid oscillation and the Gufoni maneuver for treating apogeotropic horizontal benign positional vertigo: a randomized controlled study

To determine the immediate and short-term efficacies of mastoid oscillation vs. Gufoni maneuver in treating the apogeotropic type of horizontal canal benign paroxysmal positional vertigo (HC-BPPV), we designed a randomized, prospective, sham-controlled study. In eight dizziness clinics in Korea, 209 consecutive patients with apogeotropic HC-BPPV were enrolled. The patients were randomly assigned to receive a single application of Gufoni (n = 70), mastoid oscillation (n = 67), or sham maneuver (n = 72). Immediate and second-day responses were determined based on the results within 1 h after a single trial of each maneuver and the following day, respectively. Second-day response was assessed in patients who were non-responders on the first day. The short-term response was determined based on the cumulative response for 2 days. Successful treatment was defined as a resolution of positional nystagmus or as a transition into geotropic horizontal nystagmus (not requires vertigo symptom resolution). The immediate responses of the Gufoni maneuver (33/70, 47.1%) and mastoid oscillation (32/67, 47.8%) were better than the sham maneuver (14/72, 19.4%) (p = 0.00). The second-day results did not differ among the three groups (p = 0.76). The short-term responses showed better efficacies with the Gufoni maneuver (51/70, 76.1%) and mastoid oscillation (46/67, 71.9%) than with the sham maneuver (38/72, 53.5%) (p = 0.02). Therapeutic efficacies did not differ between the Gufoni and mastoid oscillation groups in terms of both immediate and short-term outcomes (p = 0.94, 0.57). Both the Gufoni maneuver and mastoid oscillation are valid methods for treating apogeotropic HC-BPPV, with a success rate of approximately 70% for a single maneuver during the short-term follow-up. Trial registration: clinicaltrials.gov identifier number: NCT02046980.     

Effect of Intraventricular Hemorrhage on Cerebellar Growth in Preterm Neonates

The aim of this study is to evaluate cerebellar growth of preterm infants with intraventricular hemorrhage. Vermis height (VH) and transverse cerebellar diameter (TCD) were measured by cranial ultrasound in 18 preterm infants (26–30 weeks) with intraventricular hemorrhage (IVH) at first 3 days of life and at term equivalent age (TEA). IVH was diagnosed by ultrasonography and scaled in accordance with the definitions by Papile et al. Measurements were compared with 18 preterm (26–30 weeks) infants without IVH. Both VH and TCD of preterm infants with IVH were significantly lower than those of preterm ones without IVH at TEA (p?<?0.001). No significant difference was found for head circumference (p?=?0.158) and weight (p?=?0.092). In subgroup analysis, preterm infants with grades 3–4 IVH had significantly lower TCD (p?=?0.008) and head circumference (p?=?0.033) than the ones with grades 1–2 IVH. However, VH (p?=?0.102) and weight (p?=?0.480) did not show any difference between these subgroups. IVH may have a significant impact on cerebellar growth on preterm infants at TEA, specially those with a severe IVH. TCD is affected more than VH.     

Clinical and CN-SFEMG evaluation of neostigmine test in myasthenia gravis

Neostigmine test (NT) is a pharmacological test, demonstrating a clinical improvement in patients affected by myasthenia gravis (MG). We aim to compare clinical evaluation and neurophysiological recordings by concentric-needle single-fiber electromyography (CN-SFEMG) in response to acute administration of neostigmine in ocular and generalized MG patients. Twenty-three MG patients (10 with ocular MG and 13 with generalized MG) were evaluated before and after 90 min neostigmine 0.5-mg administration. Clinical responsiveness was assessed by MG composite (MGC) scale. Neurophysiological evaluation by CN-SFEMG considered analysis of mean value of consecutive differences (MCD), single-pair jitter, and blocks. MGC scores significantly improved after NT in generalized MG patients (MGC 11.1?±?7.6 vs 9.1?±?6.7, p?=?0.02), whereas the improvement was not significant in the ocular group. CN-SFEMG recordings significantly improved after NT in generalized MG patients (MCD 58.9?±?18.8 vs 45.9?±?23.2 μs, p?=?0.003; single-pair jitter 49.8?±?26.9 vs 24.1?±?26.7%, p?=?0.0001; blocks 6.2?±?9.5 vs 2.6?±?7.4%, p?=?0.03) as well as in ocular MG patients (MCD 50.8?±?22.7 vs 40.1?±?22.9 μs, p?=?0.01; single-pair jitter 35.9?±?23.7 vs 20.0?±?25.1%, p?=?0.001). CN-SFEMG is a reliable tool to evaluate responsiveness to acute administration of neostigmine in MG. Moreover, neurophysiological modifications to NT could show subclinical improvement in ocular MG better than that of the clinical scale.     

Association between the growth rate of subependymal giant cell astrocytoma and age in patients with tuberous sclerosis complex

Purpose

The most common neurological complications associated with tuberous sclerosis complex (TSC) include intractable seizures that begin in infancy and subependymal giant cell astrocytoma (SEGA) complicated by hydrocephalus with increasing age. Information on SEGA growth of TSC patients is limited. This study aimed to examine the TSC-SEGA growth rates by periodic neuroimaging.

Methods

This study evaluated the TSC-SEGA growth rates by serial neuroimaging. Fifty-eight patients with TSC underwent systematic evaluation, including a review of medical history and serial brain neuroimaging.

Results

While magnetic resonance imaging was more sensitive in detecting cortical tubers than computed tomography (73.1 vs. 0 %, p?<?0.001), its efficacy in identifying intracranial lesions was comparable to that of computed tomography (96.2 vs. 100 %, p?=?0.658). Significant tumor growth was observed in children (p?=?0.012) and adults (p?=?0.028) during follow-up periods, respectively (median for children 23.5 months, interquartile range 18–40 months and median for adults 23 months, interquartile range 12–34 months). Further, the SEGA growth rate in children was significantly higher than that in adults (75.6 vs. 16.5 %, p?=?0.03).

Conclusions

The results of the study show that SEGA has a significantly higher growth rate in children using serial follow-up brain imaging, suggesting the importance of performing follow-up neuroimaging at yearly intervals in childhood to identify and prevent potential comorbidities.