Cortical changes in the brain of patients with hemifacial spasm.

OBJECTIVE: Hemifacial spasm (HFS) is a movement disorder characterized by involuntary muscle contractions on one side of the face. It is associated with disturbances in the brain's functional architecture. Despite this, the structural alterations in the brain related to HFS remain poorly understood. In this study, we investigated the cortical morphology changes in patients with HFS compared to healthy controls (HCs). METHODS: We analyzed 3D T1-weighted MRI images from 33 patients with left-sided primary HFS and 33 age- and sex-matched HCs. Measurements of cortical thickness (CTh), sulcal depth, local gyrification index (lGI), and fractal dimension were taken using a computational anatomy toolbox. A general linear model, accounting for age, gender, and total brain volume, was applied for statistical analyses. Significant clusters were then assessed for correlations with clinical parameters. RESULTS: The HFS patients displayed several cortical abnormalities when compared to HCs, including reduced CTh in the contralateral precentral gyrus and left orbitofrontal cortex, decreased sulcal depth in the left orbitofrontal cortex, and increased lGI in the right insula and superior temporal cortex. However, fractal dimension did not differ significantly between the groups. Additionally, in HFS patients, a notable negative correlation was found between the sulcal depth in the left orbitofrontal cortex and the Beck Depression Inventory-II scores. CONCLUSIONS: Our findings reveal that HFS is associated with specific surface-based morphological changes in the brain. These alterations contribute to a deeper understanding of the neurophysiological mechanisms involved in HFS and may have implications for future research and treatment strategies.

Deceptive Appearances - Spastic Paretic Hemifacial Contractures in Cases of Seventh Nerve Involvement 'Inside' and 'Outside' the Brainstem.

Spastic paretic hemifacial contracture (SPHC) is a rare clinical phenomenon characterized by facial weakness and simultaneous well-sustained contraction of the unilateral half of the face, mimicking a paresis of the normal contralateral side on casual inspection. We present three cases with such phenomenon and have postulated the underlying mechanisms. One patient had intrinsic brainstem glioma, and the others were operated for extra-axial lesions compressing the pons. The former presented with SPHC, whereas the latter two gradually developed this phenomenon following postoperative facial paresis. This condition is possibly due to denervation hyper-excitability of the facial supranuclear pathway or an aberrant regeneration secondary to nerve injury leading to functional facial-nerve nuclear reorganization. SPHC occurrence is not limited to intra-axial lesions but can also be seen after partial injury to the facial nerve beyond its exit from the brainstem.

Alterations of white matter tracts and topological properties of structural networks in hemifacial spasm.

Hemifacial spasm (HFS) is characterized by involuntary and paroxysmal muscle contractions on the hemiface. It is generally believed that HFS is caused by neurovascular compression at the root exit zone of the facial nerve. In recent years, the structural alterations of brains with HFS have aroused growing concern. However, little attention has been directed towards the possible involvement of specific white matter (WM) tracts and the topological properties of structural networks in HFS. In the present study, diffusion magnetic resonance imaging tractography was utilized to construct structural networks and perform tractometric analysis. The diffusion tensor imaging scalar parameters along with the WM tracts, and the topological parameters of global networks and subnetworks, were assessed in 62 HFS patients and 57 demographically matched healthy controls (HCs). Moreover, we investigated the correlation of these parameters with disease-clinical-level (DCL) and disease-duration-time (DDT) of HFS patients. Compared with HCs, HFS patients had additional hub regions including the amygdala, ventromedial putamen, lateral occipital cortex, and rostral cuneus gyrus. Furthermore, HFS patients showed significant alternations with specific topological properties in some structural subnetworks, including the limbic, default mode, dorsal attention, somato-motor, and control networks, as well as diffusion properties in some WM tracts, including the superior longitudinal fasciculus, cingulum bundle, thalamo-frontal, and corpus callosum. These subnetworks and tracts were associated with the regulation of emotion, motor function, vision, and attention. Notably, we also found that the parameters with subnetworks and tracts exhibited correlations with DCL and DDT. In addition to corroborating previous findings in HFS, this study demonstrates the changed microstructures in specific locations along with the fiber tracts and changed topological properties in structural subnetworks.

Prevalence and severity of neurovascular compression in hemifacial spasm patients.

Hemifacial spasm is typically caused by vascular compression of the proximal intracranial facial nerve. Although the prevalence of neurovascular compression has been investigated in a cohort of patients with classical trigeminal neuralgia, the prevalence and severity of neurovascular compression has not been well characterized in patients with hemifacial spasm. We aimed to investigate whether presence and severity of neurovascular compression are correlated to the symptomatic side in patients with hemifacial spasm. All patients in our study were evaluated by a physician who specializes in the management of cranial nerve disorders. Once hemifacial spasm was diagnosed on physical exam, the patient underwent a dedicated cranial nerve protocol magnetic resonance imaging study on a 3 T scanner. Exams were retrospectively reviewed by a neuroradiologist blinded to the symptomatic side. The presence, severity, vessel type, and location of neurovascular compression along the facial nerve was recorded. Neurovascular compression was graded as contact alone (vessel touching the facial nerve) versus deformity (indentation or deviation of the nerve by the culprit vessel). A total of 330 patients with hemifacial spasm were included. The majority (232) were female while the minority (98) were male. The average age was 55.7 years. Neurovascular compression (arterial) was identified on both the symptomatic (97.88%) and asymptomatic sides (38.79%) frequently. Neurovascular compression from an artery along the susceptible/proximal portion of the nerve was much more common on the symptomatic side (96.36%) than on the asymptomatic side (12.73%), odds ratio = 93.00, P < 0.0001. When we assessed severity of arterial compression, the more severe form of neurovascular compression, deformity, was noted on the symptomatic side (70.3%) much more frequently than on the asymptomatic side (1.82%) (odds ratio = 114.00 P < 0.0001). We conclude that neurovascular compression that results in deformity of the susceptible portion of the facial nerve is highly associated with the symptomatic side in hemifacial spasm.

Two-dimensional structure analysis of hemifacial spasms and surgical outcomes of microvascular decompression.

BACKGROUND: Although studies have indicated that the small posterior fossa plays a role in hemifacial spasm (HFS), few studies have denoted the correlations between local measurements of the cerebellopontine angle and the incidence of HFS and surgical outcomes. METHODS: We retrospectively analyzed the demographic and clinical data of HSF patients who underwent microvascular decompression at our institution. Healthy controls were recruited. The divergent prognosis of HFS was defined as an ordinal variable. A multivariable ordinal regression model was generated to estimate the relationship between the variables and outcomes of HFS. RESULTS: Between 2013 and 2018, 180 patients who were enrolled in our study met the inclusion criteria. Compared with the control group (n = 94), HFS patients had a smaller internal acousticmeatus-brainstem distance (P < 0.001) on the unaffected side and a larger facial nerve-brainstem angle (P < 0.001). The regression analysis demonstrated that subgroups with more severe facial nerve compression (mild vs severe, OR = 0.269, P = 0.018; moderate vs severe, OR = 0.215, P < 0.001) and a thinner brainstem (OR = 2.368, P = 0.014) were more likely to experience better short-term outcomes, while subgroups with a thinner brainstem (OR = 5.583, P = 0.007) were more likely to experience better long-term outcomes. DISCUSSION: Structural changes occurring in patients are risk factors for HFS. The patient's local neurovascular structure and brainstem volume are factors that significantly influence short-term and long-term surgical outcomes.

Morphological characteristics of the vertebrobasilar artery system in patients with hemifacial spasm and measurement of bending length for evaluation of tortuosity.

OBJECTIVE: Dominant vertebral artery (DVA), tortuosity, and elongation of the vertebrobasilar artery system are frequently observed in hemifacial spasm (HFS). However, the morphological characteristics of the tortuosity of vertebrobasilar artery system have not yet been elucidated. In this study, we presented a novel method for the measurement of the bending length (BL) of the basilar artery(BA) or vertebral artery (VA) to assess the tortuosity of vertebrobasilar artery system in HFS patients. METHODS: The demographic and morphological characteristics of 135 patients with HFS admitted to the neurosurgical department to undergo microvascular decompression (MVD) were analyzed in this retrospective study. The BL was defined to appraise the tortuous degree of the vertebrobasilar artery system, and according to the BL value, the patients with HFS were divided into two groups: tortuous vertebrobasilar artery (TVA) and non-TVA groups. Additionally, the vessels responsible for HFS were analyzed based on the results of magnetic resonance imaging (MRI) of the two groups. The patients were followed up for 2-6 years post-discharge, and the effect of MVD operation was compared between the two groups. RESULTS: DVA was detected in 60.2% of HFS patients; the incidence of left-sided HFS in the TVA group was significantly higher than that in the non-TVA group (P = 0.013). The proportion of multiple responsible vessels in the TVA group was 68.4% (54/79), while that in the non-TVA group was 4.1% (2/49). The complication rate of the two groups was different, and that of the tortuous group was higher than that of the non-tortuous group. CONCLUSIONS: The morphological characteristics of the vertebrobasilar artery system in patients with HFS were complex. The measurement of BL is an easy and reliable tool to assess the tortuosity of the vertebrobasilar artery system in HFS patients.

Flat-shaped posterior cranial fossa was associated with poor outcomes of microvascular decompression for primary hemifacial spasm.

BACKGROUND: Numerous factors have been investigated on affecting the outcomes of primary hemifacial spasm (HFS) after microvascular decompression (MVD). It is well established that anatomical differences of the posterior cranial fossa (PCF) plays an important role in the occurrence of HFS. However, it is still not clear whether morphological characteristics of PCF affect the surgical outcomes of HFS after MVD. Our study aims to investigate the prognostic factors for surgical outcomes of MVD for primary HFS, with a particular focus on the morphological characteristics of PCF. METHODS: Between January 2014 and November 2017, a total of 152 HFS patients who underwent MVD treatment in our department were included in this study. The clinical data were retrospectively reviewed. The outcomes of MVD were classified into success and failure groups according to the short- and long-term postoperative responses. Particularly, we established an ellipsoid model for PCF. The related length (Y), width (X) and height (Z) of the PCF were measured and the volume of PCF was calculated employing a formula of [Formula: see text]XYZ. The relationship between PCF volume and surgical outcomes was statistically analysed. RESULTS: The severity of neurovascular compression (NVC) (p = 0.010), type of NVC (p = 0.001) and lateral spread response (LSR) (p < 0.0001) significantly influenced the long-term surgical outcomes of MVD for primary HFS. In particular, for the first time, we demonstrated that a flat-shaped PCF was associated with poor long-term outcome and postoperative recurrence. CONCLUSIONS: Our current study suggests that mild NVC, small vessel compression, intraoperative LSR persistence and flat-shaped PCF are independent factors predicting poor prognosis of MVD for primary HFS.

Microvascular decompression for hemifacial spasm associated with distinct offending vessels: A retrospective clinical study.

OBJECTIVE: To investigate the surgical effects and complications of microvascular decompression (MVD) for hemifacial spasm (HFS) based on different offending vessels, and report our surgical experience of HFS patients related to vertebral artery. PATIENTS AND METHODS: MVDs performed in Nanjing Drum Tower Hospital between January 1, 2014 and December 31, 2017 were retrospectively studied, and 1152 patients with HFS were split into two groups in accordance with the offending vessels. RESULTS: 954 patients with HFS caused by small vascular compression were classified as Group A. 849 patients got cured immediately after MVD, while delayed resolution was identified in 101 patients. 4 patients were not relieved and 4 were relapsed during the follow-up period. We observed 76 cases of delayed facial paralysis, 7 cases of hearing loss, 2 hoarseness and 3 cases of CSF leakage after surgery. In Group B, 198 patients displayed HFS associated with the vertebral artery (VA). 144 cases were spasm free after surgery and 51 patients had a delayed resolution. 3 patients were not significantly ameliorated and 2 were relapsed during the follow-up period. The major postoperative complications included facial paralysis in 28 patients, hearing loss in 4 and hoarseness and dysphagia in 3. The two groups showed no operative death. CONCLUSIONS: For the patients with HFS related to VA, the delayed cure rate and the incidence of postoperative cranial nerve complications were higher than HFS attributed to small vascular compression. And the two groups were not different in the long-term outcome and the incidence of permanent cranial nerve complications.

Loop characteristics and audio-vestibular symptoms or hemifacial spasm: is there a correlation? A multiplanar MRI study.

AIM: We investigated if loop characteristics correlate with audio-vestibular symptoms or hemifacial spasm in patients with a vascular loop in the root entry zone (VII and VIII) and in the internal auditory canal. MATERIALS AND METHODS: A retrospective, multicenter study analyzed 2622 consecutive magnetic resonance imaging (MRI) scans of the cerebellopontine angle of patients with asymmetric audio-vestibular symptom or hemifacial spasm; patients' symptoms were confirmed by clinical tests. MRIs displaying vascular loops visible in the axial view were analyzed using multiplanar reconstruction. We evaluated (1) depth of penetration of the loop into the internal auditory canal (IAC); (2) largest diameter of the vessel; (3) nerve(s) involved in the vascular impingement, position of the loop relative to such nerve(s) and number of contacts between vessel and nerve(s); (4) length of such contact. The loop metrics described above were correlated with the patients' audio-vestibular symptoms and hemifacial spasm. RESULTS: Three hundred ninety-nine patients displayed a loop visible in the MRI axial view and out of them only 118 displayed a direct contact between loop and nerve. The cochlear nerve was involved in a contact in 57.7%. Loops in direct nerve contact had a calibre > 0.85 mm, were located in the middle portion of the IAC, and correlated with vertigo (p = 0.002), tinnitus (p = 0.003), and hemifacial spasm (p < 0.001). Asymmetric sensorineural hearing loss (SNHL) correlated with number of contacts (p < 0.001) and length of contact (p < 0.05). The contact was asymptomatic in 41.5% of patients. CONCLUSION: Loop characteristics may help predict whether a vascular impingement is responsible for a symptom and guide the physician to select the best treatment. KEY POINTS: • A vascular loop in the internal auditory canal was observed in 18-20% of the patients in this study; whether a loop can be responsible for a compressive syndrome is still unclear in particular referred to the vestibulocochlear nerve. • Compression by a loop on the facial nerve causes hemifacial spasm; compression by a loop on the cochlear or vestibular nerve may cause audio-vestibular symptoms. • In patients with a loop, the loop calibre, the loop position, and the number of loop-nerve(s) assessed via the multiplanar MRI reconstruction technique may help assess whether the patient will manifest audio-vestibular symptoms or hemifacial spasm.

Clinical Outcome After Microvascular Decompression According to the Progression Rates of Hemifacial Spasm.

OBJECTIVE: The progression rate for clinical manifestations in hemifacial spasm (HFS) varies; however, little is known about the factors contributing to this. The purpose of this study was to identify independent factors affecting the rate of symptom progression and to evaluate clinical outcomes according to progression rates. METHODS: The study enrolled 1335 patients who underwent microvascular decompression for HFS between July 2004 and January 2015. We assessed detailed history, clinical manifestations, and outcomes. Based on the duration and severity of symptoms, patients were classified into rapidly progressive and slowly progressive groups. We identified predisposing factors affecting the differences between the 2 groups and evaluated the clinical outcome in each group. RESULTS: Of 1335 patients with HFS, 825 (61.8%) were classified as rapidly progressive, and 510 (38.2%) were classified as slowly progressive. In univariable and multivariable analyses, younger age at surgery, older age at symptom onset, and absence of intraoperative facial nerve indentation were significant predisposing factors for rapid progression. The rapidly progressive group had worse outcomes than the slowly progressive group following microvascular decompression. CONCLUSIONS: In this study, patients with rapidly progressive HFS had worse clinical outcomes. Therefore, patients with rapidly progressive symptoms should be warned in advance that the prognosis may be worse after microvascular decompression. This study is also useful to understand the differences in symptom progression rates in HFS in order to inform patients about symptom progression.

The Rhomboid Lip: Anatomy, Pathology, and Clinical Consideration in Neurosurgery.

OBJECTIVE: To describe microsurgical anatomy of the rhomboid lip (RL) and to consider its role by presenting histology and clinical cases. METHODS: We examined 10 (20 sides) formalin-fixed adult human cadaveric heads injected with colored silicone. A total of 20 RLs were examined posteriorly. We examined the expansion of the RL in the cerebellopontine cistern. We assessed the relationship between the RL and choroid plexus and the RL and cranial nerve IX and classified the RL. We also observed these relationships in clinical cases during surgeries and examined histologic assessments of the RL. RESULTS: The RL was divided into 3 types, non-extension type, lateral extension type, and jugular foramen type, according to the relationship between the RL and choroid plexus. There were many variations of the extension of the RL. The jugular foramen type was rare. CONCLUSIONS: Histologically, the RL is the remnant of the fourth ventricle covered with ependymal cells. Knowledge of the detailed anatomy and proper dissection of the RL may help surgeons to obtain good visualization of structures around the foramen of Luschka.

Epidemiology of benign essential blepharospasm: A nationwide population-based retrospective study in Taiwan.

IMPORTANCE: This study provides a nationwide, population-based data on the incidence of benign essential blepharospasm in Asian adults. BACKGROUND: To describe the incidence, patient demographics, and risk factors associated with benign essential blepharospasm. DESIGN: Population-based retrospective study. PARTICIPANTS AND SAMPLES: A total of 1325 patients with benign essential blepharospasm were identified. METHODS: Patients with diagnosis of blepharopsasm between January 2000 and December 2013 were sampled using the Longitudinal Health Insurance Database 2000. Secondary blepharospasm that may be related to neurological, trauma, and ocular surface disease were excluded. MAIN OUTCOME MEASURED: Multivariate conditional logistic regression was used to estimate the odds ratios for potential risk factors of benign essential blepharospasm. RESULTS: The mean annual incidence was 0.10‰ (0.07‰ for males, and 0.12‰ for females). The peak incidence was in the 50 to 59-year-old age group (0.19‰). People living in urban regions have more risk of developing blepharospasm comparing to people living in less urban regions (p <0.01). White-collar workers also have higher chance of having blepharospasm (p<0.001). Significant difference between control group and case group in hyperlipidemia (p <0.001), sleep disorders (p <0.001), mental disorders (depression, anxiety, obsessive compulsive disorder) (p <0.001), dry eye-related diseases (dry eye, Sjögren's syndrome) (p <0.001), Parkinson's disease (p <0.004), and rosacea (p <0.021) were also identified. CONCLUSIONS AND RELEVANCE: Higher level of urbanization, white-collar work, sleep disorders, mental health diseases, dry eye-related diseases, Parkinsonism, and rosacea are possible risk factors for benign essential blepharospasm.

Imaging of hemifacial spasm.

Almost all primary hemifacial spasms are associated with one or more neurovascular conflicts, most often at the root exit zone in the immediate vicinity of the brainstem. Imaging has first to exclude a secondary hemifacial spasm and secondly to search for and characterize the responsible neurovascular conflict(s). Magnetic resonance imaging should include high-resolution anatomical hyper T2-weighted sequences and magnetic resonance angiography by using 1.5 or even better 3 Tesla magnets. The most frequent vascular compressions are from the anterior-inferior cerebellar artery, the posterior-inferior cerebellar artery and the vertebrobasilar artery; venous conflicts are very rare. Conflicts are often multiple; also, the same vessel may compress the facial nerve in two places. Also, conflicts may be aided by particular anatomical circumstances, including arterial dolichoectasia, posterior fossa with a small volume or bony malformations.

New insights into the pathophysiology of primary hemifacial spasm.

Primary hemifacial spasm (pHFS) is due to a benign compression of the facial motor nerve by an offending vessel, leading to increased nerve excitability. Facial nerve hyperexcitability presents two different aspects. First, there is a spontaneous and ectopic generation of action potentials on the incriminated nerve and then this ectopic impulse can propagate and spread "laterally" from one facial nerve branch to another. This results in spontaneous and synkinetic spasms affecting one hemiface. Although the increase in excitability certainly concerns the nucleus of the facial motor nerve in the brainstem, it seems unlikely that the primary origin of this hyperexcitability and the associated phenomenon of lateral spreading strictly originate at the nuclear level. In fact, the mechanisms causing facial nerve hyperexcitability per se remain unknown. The leading implication of a structural nerve lesion, such as segmental demyelination, induced by vessel compression, is also unconvincing. In contrast, a functional mechanical factor increasing nerve excitability is extremely probable, that it is either due to compression or stretch resulting from the neurovascular conflict. Axonal ion channel changes are obviously associated with this mechanism. Then the lateral spreading of nerve fibre hyperexcitability probably results from an ephaptic process, the "cross-talk" between axons being located in the region of the conflict or in the transition zone between central and peripheral myelin, at the end of the facial nerve root exit zone. In any event, pHFS is due to a functional increase in facial nerve excitability triggered by an offending vessel and this clearly explains the remarkable and rapid efficacy of surgical microvascular decompression.

Facial Myokymia and Hemifacial Spasm in Multiple Sclerosis: A Descriptive Study on Clinical Features and Treatment Outcomes.

OBJECTIVE: Facial Myokymia and hemifacial spasm have been associated with multiple sclerosis; however, their etiology and clinical outcome is uncertain. Here, we describe the clinical, radiologic features, and treatment outcomes of a cohort of patients with multiple sclerosis and history of facial myokymia or hemifacial spasm. METHODOLOGY: We reviewed the clinical features, radiologic features, and treatment outcomes of 35 patients with a diagnosis of multiple sclerosis and facial myokymia (28) or hemifacial spasm (7) seen at Mayo Clinic (Rochester, MN). RESULTS: Facial myokymia was associated with a clinical or radiologic relapse in 11 of 28 patients. In 27 of 28 patients with facial myokymia, symptom resolution occurred within months regardless of treatment. An ipsilateral pontine lesion was found in 3 of 7 cases with hemifacial spasm. Hemifacial spasm was associated with a clinical or radiologic relapse in 3 of 7 cases. Hemifacial spasm resolved within 4 years in 5 of 7 cases, with the remaining cases persisting up to 9 years despite treatment. CONCLUSIONS: Facial myokymia and hemifacial spasm occurring in patients with multiple sclerosis is associated with an ipsilateral pontine MRI lesion in a minority of patients. Facial myokymia is a self-limited process while hemifacial spasm can be persistent in a minority of patients, despite treatment.

Reorganization of sensory input at brainstem in hemifacial spasm and postparalytic facial syndrome.

We hypothesized the filtering of sensory input from face and hand at brainstem may reorganize in hemifacial spasm (HFS) and postparalytic facial syndrome (PFS). Thus, we examined the prepulse inhibition of blink reflex (BR-PPI) in HFS and PFS. We included 12 healthy subjects, 13 patients with HFS, and 11 patients with PFS. Baseline BR, BR recovery at interstimulus interval (ISI) of 300 ms and BR-PPI at ISI of 100 ms were performed on the right sides of healthy subjects and on both sides of patients. Within-subject analysis showed baseline BR and BR-PPI were similar between asymptomatic and symptomatic sides of patients with HFS whereas BR recovery was higher on the symptomatic side. In the PFS group, latency of R2 during baseline BR recording was longer (p = 0.022) and R2 amplitude (p = 0.046) was reduced on the symptomatic side compared to asymptomatic side. Reduction of R2 area in BR-PPI recordings was also the lowest in HFS compared to other two groups (p = 0.000); however, it was also lower in patients with PFS compared to healthy subjects (p = 0.018). BR-PPI was decreased on both sides of patients. The mean R2 recovery was higher on both sides of patients with HFS and PFS (p = 0.007). Filtering of facial sensory input is decreased probably to monitor and to correct the sequence of facial movements in these disorders.

Vascular tortuosity in relationship with hypertension and posterior fossa volume in hemifacial spasm.

BACKGROUND: Hemifacial spasm (HFS) is a disabling neurological condition. Vascular tortuosity in HFS patients has not been quantified objectively and its relationship with hypertension and posterior fossa volume (PF) is unknown. In a case control magnetic resonance imaging and angiographic (MRI/A) study, we quantified and compared the vascular tortuosity in HFS and controls, and evaluated its relationship with hypertension and PF. METHODS: Using a commercially available vessel probe tool, an index of tortuosity based on vessel over chord length was employed to quantify vascular tortuosity of the vertebral (VA) and basilar arteries (BA) in 79 subjects (40 HFS, 39 controls). RESULTS: The tortuosity index of the BA (1.09 vs 1.16, p = 0.26, 95 % CI 1.07, 1.23), RVA (1.15 vs 1.15, p = 0.83, 95 % CI 1.06, 1.38) and LVA (1.14 vs 1.288, p = 0.16, 95 % CI 1.14, 1.44) was not different between HFS and controls, with adjustments for PF volume and hypertension. CONCLUSIONS: Contrary to popular belief, our study showed that taking into account hypertension and PF volume, vascular tortuosity of the vertebrobasilar arteries is unlikely to be a major etiologic factor in HFS, though its role in select individuals cannot be excluded. The complex interplay of facial nerve hyperexcitability, genetic predisposition, vascular tortuosity, posterior fossa volume and hypertension needs to be further evaluated.