Isolated hemorrhage in the cerebellar vermis with vertigo and body lateropulsion to the contralesional side / 영남의대학술지

There have been several reports of patients with isolated lesions of the cerebellar vermis presenting with clinical features similar to those of peripheral vestibulopathy. We report a case of small, isolated hematoma in the cerebellar vermis in a patient who presented with vertigo, ipsilesional nystagmus, and body lateropulsion to the contralesional side without the usual signs or symptoms of cerebellar dysfunction. Although they present with symptoms that mimic those of peripheral vestibulopathy, and brain computed tomography shows no abnormality, as there may be a small, isolated hematoma or infarction in the cerebellar vermis. Thus, brain magnetic resonance imaging should be performed in elderly patients with vascular risk factors.

Romboencefalosinapsis como causa de ventriculomegalia severa. Reporte de dos casos / Rhomboencephalosynapsis as a cause of severe vetriculomegaly. Report of two cases

We report two cases of a rare pathology of the fetal cerebellum, rhomboencephalosynapsis, a defect characterized by the partial or complete absence of the cerebellar vermis, which is currently associated with severe ventriculomegaly due to defects in the formation of the cerebral aqueduct. These are the first cases reported in Peru

Reportamos dos casos de una patología rara del cerebelo fetal, la romboencefalosinapsis, defecto caracterizado por la ausencia parcial o completa del vermis cerebeloso, frecuentemente asociada a ventriculomegalia severa por defectos en la formación del acueducto de Silvio. Estos constituyen los primeros casos reportados en Perú.

Craniocerebral ultrasonography in evaluation on correlation between brain development with gestational age and birth weight of neonatal brain / 中国医学影像技术

Objective To explore the value of craniocerebral ultrasonography in evaluation on neonatal brain development.Methods Totally 135 newborns were collected in neonatal intensive care unit.The sagittal length of the corpus callosum and the sagittal length of the cerebellar vermis were measured on the day of birth.The impact of gestational age and birth weight on the development of the corpus callosum and cerebellar vermis were analyzed.Results The mean sagittal length of corpus callosum at birth in premature infants and term infants was (39.18 ± 2.53) mm and (41.62 ± 3.28) mm,respectively (t =4.87,P＜0.05).The sagittal length of cerebellar vermis in premature infants and term infants was (19.03 ±2.00)mm and (20.91 ±2.29)mm,respectively (t=5.05,P＜0.05).The length of the corpus callosum was positively correlated with gestational age and birth weight (rs =0.45,0.51,both P ＜ 0.05),while the sagittal length of cerebellar vermis was positively correlated with gestational age and birth weight (rs =0.42,0.46,both P＜0.05).The length of the corpus callosum was positively correlated with the length of cerebellar vermis (rs =0.43,P ＜0.05).Conclusion Craniocerebral ultrasonography can be used to dynamically observe the neonatal intracranial structure and the development of corpus callosum and cerebellar vermis,therefore providing reliable references for clinical intervention.

Wernicke Encephalopathy Associated with Acute Wet Beriberi

Wernicke encephalopathy is usually accompanied with peripheral neuropathy, known as dry beriberi. In contrast, wet beriberi presenting as cardiovascular symptoms rarely occurs. The acute type of wet beriberi can be fatal, if untreated quickly. It is reported that the cerebellar vermis has a role of the coordination and control of cardiovascular and autonomic reflex activities. We report a 58-year-old man showing acute wet beriberi in Wernicke encephalopathy with cerebellar vermis lesion.

Síndrome cerebeloso por mesalamina: reporte de un caso / Cerebellar syndrome for mesalamine: a case report

El cerebelo proviene del latín "pequeño cerebro", es aproximadamente una décima parte del cerebro en tamaño y peso, se encuentra en la fosa craneal posterior. está conectado directamente o indirectamente a una variedad de estructuras, incluyendo el tronco cerebral, la columna vertebral, las regiones corticales y subcorticales. El cerebelo contiene casi el 80 % del total de las neuronas del cerebro y se compone de formas diferenciadas de unidades neuronales, cada una compartiendo microcircuitos cerebelosos básicos. El cerebelo está formado por dos hemisferios laterales y una porción media o vermis . El cerebelo tiene varias funciones: coordinar los movimientos voluntarios especializados al influir en la actividad muscular y controlar el equilibrio, el tono muscular a través de conexiones con el sistema vestibular, la médula espinal y sus neuronas motoras alfa. Dentro de la corteza del cerebelo existe una organización somatotópica de las partes del cuerpo. El cerebelo es una pieza clave para el procesamiento de la información y participa en numerosas actividades motoras y no motoras, gracias a las características anatómicas de sus circuitos, las enormes capacidades de análisis y la alta conectividad con otras áreas del cerebro, puede verse afectado por diferentes factores como: anormalidades en el desarrollo embrionario, tóxicos, enfermedades autoinmunes, inflamatorias, vasculares y metabólicas, infecciones, tumores primarios y secundarios, traumas, iatrogénicos, enfermedades genéticas, esporádicas, dando origen a signos y síntomas que causan un síndrome cerebeloso de acuerdo al área anatómica comprometida. En la literatura científica no hay reportes de casos de síndrome cerebeloso por mesalamina (mesalazina). A continuación presentamos el primer caso.

Cerebellum is Latin for "little brain" is about a tenth of the brain in size and weight, is in the posterior cranial fossa. It connects directly or indirectly to a variety of structures, including brainstem, spinal cord, cortical and subcortical regions. The cerebellum contains almost 80% of all neurons in the brain and consists of different forms of neuronal units, each sharing basic cerebellar microcircuits. The cerebellum is formed by two lateral hemispheres and vermis or a middle portion . The cerebellum has several functions: coordinate movements specialized volunteers to influence muscle activity and control the balance, muscle tone through connections with the vestibular system, the spinal cord and alpha motor neurons. Within the cerebellar cortex somatotopic organization exists a body part. The cerebellum is a key to information processing and participates in numerous motor and non-motor activities, thanks to the anatomical characteristics of its routes, huge analysis capabilities and high connectivity with other brain areas, can be affected by different factors such as abnormalities in embryonic development, toxic, autoimmune, inflammatory, vascular and metabolic, infections, primary and secondary tumors, trauma, iatrogenic, genetic diseases, sporadic, giving rise to signs and symptoms that cause cerebellar syndrome according to compromised anatomical area. In the scientific literature no reported cases of cerebellar syndrome mesalamina (mesalazina), we report the first case.

Isolated Infarction of Anterior Cerebellar Vermis / 대한평형의학회지

The anterior cerebellar vermis has been known to act in coordination of gait and postural adjustment of the trunk and legs. However, oculomotor abnormalities in an isolated anterior vermian lesion have not been described in the literature. A 59-year-old man presented with acute non-rotatory dizziness and disequilibrium. Neuro-ophthalmologic examination found impaired smooth pursuit and hypometric saccades in the contralesional direction, and disconjugate ipsiversive ocular torsion, but without spontaneous or gaze-evoked nystagmus. Imaging study showed an infarction restricted to the rostral end of right cerebellar vermis involving the lingual and central lobules. The anterior cerebellar vermis participates in the maintenance of axial posture and gait, and also in the control of ocular motor and vestibular systems.

Effect of histamine H1 and H2 receptor antagonists, microinjected into cerebellar vermis, on emotional memory consolidation in mice

This study investigated the effects of histamine H1 or H2 receptor antagonists on emotional memory consolidation in mice submitted to the elevated plus maze (EPM). The cerebellar vermis of male mice (Swiss albino) was implanted using a cannula guide. Three days after recovery, behavioral tests were performed in the EPM on 2 consecutive days (T1 and T2). Immediately after exposure to the EPM (T1), animals received a microinjection of saline (SAL) or the H1 antagonist chlorpheniramine (CPA; 0.016, 0.052, or 0.16 nmol/0.1 µL) in Experiment 1, and SAL or the H2 antagonist ranitidine (RA; 0.57, 2.85, or 5.7 nmol/0.1 µL) in Experiment 2. Twenty-four hours later, mice were reexposed to the EPM (T2) under the same experimental conditions but they did not receive any injection. Data were analyzed using one-way ANOVA and the Duncan test. In Experiment 1, mice microinjected with SAL and with CPA entered the open arms less often (%OAE) and spent less time in the open arms (%OAT) in T2, and there was no difference among groups. The results of Experiment 2 demonstrated that the values of %OAE and %OAT in T2 were lower compared to T1 for the groups that were microinjected with SAL and 2.85 nmol/0.1 µL RA. However, when animals were microinjected with 5.7 nmol/0.1 µL RA, they did not show a reduction in %OAE and %OAT. These results demonstrate that CPA did not affect behavior at the doses used in this study, while 5.7 nmol/0.1 µL RA induced impairment of memory consolidation in the EPM.

Study on the fetal cerebellar vermis using three-dimensional ultrasound VCI-C imaging / 中国基层医药

Objective To study the application value of three-dimensional transabdominal ultrasound VCI-C imaging in detection of fetal cerebellar vermis.Methods Totally 300 normal fetuses at 18 ～ 36 gestation weeks were examined by three-dimensional ultrasound VCI-C imaging to observe the fetal cerebellar vermis.Results The view of three-dimensional ultrasound VCI-C imaging well evaluated the integrity and size of vermis morphous,identified characteristic signs of vermis:the fourth ventricle apex and vermis crack.The brain stem and vermis were almost parallel in normal fetus.The angle between them was (3.89 ± 1.58)°.There was no significant relation between the gestational age and the angle.The craniocaudal diameter,anteroposte diameter and acreage of the cerebellar vermis were positively correlated with gestational age.Conclusion The three-dimensional ultrasound VCI-C imaging could help evaluate the development of the cerebellar vermis and show the information of cerebellar vermis and its surrounding structure.It offers a new method to evaluate the fetal cerebellar vermis.

Brain Imaging Studies in Leber's Congenital Amaurosis: New Radiologic Findings Associated with the Complex Trait

PURPOSE: To report the incidence and new findings of abnormal brain imaging studies associated with patients initially diagnosed with Leber's congenital amaurosis (LCA) without definite systemic abnormalities and to determine the need for brain imaging studies in these patients. METHODS: A retrospective review of medical records was performed in 83 patients initially diagnosed as LCA and without definite systemic abnormalities before the age of 6 months in 2 tertiary referral centers. Brain magnetic resonance imaging was performed in 31 of 83 patients (37.3%). RESULTS: Six of 31 patients (19%) had radiologically documented brain abnormalities. Two patients had cerebellar vermis hypoplasia, 1 patient showed an absence of septum pellucidum, 2 subjects showed mild external hydrocephalus, and 1 patient was found to have a small cerebellum. CONCLUSIONS: Approximately one fifth of the LCA patients in whom brain imaging was performed were associated with brain abnormalities, including the absence of septum pellucidum, which has not been documented in the literature. Brain imaging is mandatory in patients primarily diagnosed with LCA, even without definite neurologic or systemic abnormalities.

Joubert Syndrome Associated with Leber's Congenital Amaurosis

PURPOSE: To report a case of Joubert syndrome associated with Leber's congenital amaurosis. CASE SUMMARY: A 7-month-old boy was referred for poor eye contact and esodeviation since birth. He could not fixate with his eyes or follow objects. Nystagmus in both eyes and moderate angle esotropia were present. Slit lamp examination and fundus examination showed no significant findings. Visual evoked potential and electroretinogram were extinguished in both eyes. The patient showed abnormal respiratory patterns, developmental delay, and hypotonia. Brain MRI showed aplasia of the cerebellar vermis, and he was diagnosed with Joubert syndrome. At 2 years of age, he was moderately able to fixate with his eyes and to crudely follow objects. Nystagmus and esotropia had diminished, but the visual evoked potential and electroretinogram still showed no response in both eyes. CONCLUSIONS: Leber's congenital amaurosis can be associated with Joubert syndrome, and children with Leber's congenital amaurosis should be evaluated for associated systemic abnormalities.

Uremic Encephalopathy Associated with Bilateral Basal Ganglia and Cerebellar Lesion in a Non-diabetic Hemodialysis Patient / 대한신장학회잡지

Involvement of central nervous system is a well- known compication in uremic patients. However, development of acute extrapyramidal symptoms with bilateral basal ganglia involvement (acute basal ganglia syndrome), especially in non-diabetic hemodialysis patient is very rare. We report a case of acute basal ganglia syndrome in a non-diabetic hemodialysis patient. A 45-year-old man with autosomal dominant polycystic kidney disease (ADPKD) on chronic hemodialysis treatment for the last 4 years was admitted due to generalized myalgia. On admission, the patient was found to have rhabdomyolysis and intractable metabolic acidosis. Nine days after admission, he suddenly developed dysarthria, lateralizing ataxia, and bradykinesia. Brain MRI demonstrated low and high signals in bilateral basal ganglia and cerebellar vermis in T1-weighted and T2-weighted images, respectively. Intensified hemodialysis treatment combined with general supportive therapy resolved the severe metabolic acidosis and the neurologic manifestations gradually improved. Follow up brain CT scan taken 3 months later showed decreased size of initial low attenuation lesions in bilateral basal ganglia and cerebellar vermis. Although no definite pathophysiology is yet established, severe metabolic disorder is believed to play an important role in development of acute basal ganglia syndrome. Correction of metabolic acidosis and hypoglycemia in our patient lead to improvement in neurologic manifestations and organic brain lesions. Our case suggests that severe metabolic acidosis and hypoglycemia in uremic patient may act as risk factors for acute basal ganglia syndrome even in non-diabetic patient.

A Case of Joubert Syndrome Associated with Nephrocalcinosis and Agenesis of Cerebellar Vermis

There are several diseases characterized by neurologic abnormalities and renal disease. Joubert syndrome is one of them. Joubert syndrome is a relatively rare autosomal recessive syndrome. The most significant and constant neurologic finding is hypoplasia of the cerebellar vermis. Joubert syndrome is associated with hypotonia, retinal dystrophy, abnormal eye movement, delayed development, abnormal respiratory pattern (neonatal episodic tachypnea or apnea) and nephronophthisis. We report a boy with Joubert syndrome associated with nephrocalcinosis and agenesis of the cerebellar vermis. This patient had also abnormal eye movement, hypotonia, abnormal respiratory pattern, delayed development and chronic renal failure.

Aging and gender effects on mid-sagittal structures of Korean brain / 대한해부학회지

The brain size is a useful parameter describing ontogenic characters and functions. Recent studies of brain size with medical imaging techniques such as MRI and CT made us understand the functions of brain and pathophysiology of various neurologic diseases. With these advances in medical imaging technique, study on Korean brain size needs more attention. In this study, neurologically intact brain MRIs of 66 females and 58 males (ages 19 through 80) were analyzed. Areas of corpus callosum, midbrain, pons, and cerebellar vermis were estimated from mid-sagittal plane of MRI films. Statistical analyses were performed to reveal the effects of aging and gender on these structures. Our results demonstrate statistically significant sexual difference of pons size in healthy adult Korean. Size of other structures examined were not different between female and male. Age-related atrophy was observed in midbrain of male and female midbrain and the corpus callosum of male. This is the first report of sexual difference of pons size in normal adult Korean. Sexual dimorphism of ageing response in copus callosum differences needs further investigation.

Chronic Encapsulated Intracerebellar Hematoma Simulating Brain Tumor during Infancy

We report a case of chronic encapsulated intracerebellar hematoma occurring during infancy. An 18 month-old boy was admitted because for 3 months, his head could not be held upright, but fell to the left. Computed tomographic(CT) scanning and magnetic resonance imaging(MRI) of the brain revealed a round mass at the cerebellar vermis ; the lesion simulated a cerebellar tumor such as a medulloblastoma. The mass was surgically removed and found to be a well-encapsulated organized intracerebral hematoma. The characteristic clinical and radiologic findings and histopathologic features are discussed, and a survey of the literature is included.

Clinical Study of Symptoms and Various Anomalies of Patients with Joubert Syndrome

PURPOSE: Joubert syndrome is known to be an autosomal recessive disorder characterized by cerebellar vermian dysgenesis with many symptoms and variety of other malformations. We studied the relevant symptoms and various malformations of seven patients diagnosed as Joubert syndrome at our institution. METHODS: Seven children with cerebellar vermian dysgenesis consistent with Joubert syndrome were included in our study. Each child was diagnosed at the Seoul National University Children's Hospital from Feb. 1991 to Feb. 1995. We studied the frequency of each symptom of Joubert syndrome and the associated malformations of the selected cases. RESULTS: All seven patients had no family members affected. Also their parental consanguinity were not found. Sex ratio was 1.3 : 1 (4 males and 3 females) showing no significant sex difference. Ages at diagnosis were less than one year old. Six patients in our study had developmental delay, which was the most frequent symptom. Four patients had ataxia; four patients had hypotonia; three patients had abnormal ocular movements such as nystagmus or saccadic palsy; only one patient had episodic tachypnea in neonatal period; and in one case there were tongue protrusion and seizure, respectively. In two cases there were occipital meningoceles, agenesis of corpus callosum, and cleft palates, respectively. In one case there were cystic renal disorder, neuronal heterotopia, absence of septum pellucidum, and polydactyly, respectively. None had retinal dystrophy or chorioretinal coloboma. CONCLUSIONS: The cardinal symptoms of Joubert syndrome are unexplainable episodic tachypnea alternating with apnea during neonatal period, abnormal ocular movement such as nystagmus or saccadic palsy, ataxia, hypotonia, and developmental delay. The presence of such symptoms should alert the clinician to apply appropriate test such as neuroradiologic study including brain MRI etc. Some of the inconstantly associated features of Joubert syndrome include congenital retinal dystrophy, chorioretinal coloboma, and cystic kidney disease. The patients should be examined routinely with electroretinogram, fundoscopy, and kidney ultrasonogram for early detection or exclusion of the associated anomalies. We stress the importance of genetic counselling for the families of Joubert syndrome as well as that of the prompt supportive therapy for the patient.

Congenital Ocular Motor Apraxia without Head Thrusts: A Case of Joubert Syndrome

Joubert syndrome is the very rare autosomal recessive disorder which is including agenesis of cerebellar vermis, respiratory discomfort, ocular motor apraxia, hereditary retinal dystrophy, ataxia and developmental retardation. To diagnose, the findings of electroretinography and visual evoked potential study can be useful and hypoplasia of cerebellar vermis in brain MRI can make certain diagnosis. We found ocular motor apraxia without head thrusts in 4-month little baby can't even control his head and neck, and then his electroretinography and visual evoked potential study was normal and there was agenesis of cerebellar vermis by MRI finding. So he was diagnosed as Joubert syndrome with ocular motor apraxia. Agenesis of cerebellar vermis is considered to be one of the causes of oculomotor apraxia, and the clinical presentation varies with the age and motor development of the child. So, we describe this case with a brief review of the literatures related to this disease.

Joubert Syndrome Presented with Ocular Motor Apraxia

Agenesis of the cerebellar vermis, episodic hyperpnea alternating with apnea, abnormal eye movement, ataxia, and psychomotor retardation are shown in Joubert syndrome. This rare syndrome is inherited as an autosomal recessive trait with variable expression. We report the first cases of Joubert syndrome in Korea, to our knowledge, which showed ocular motor apraxia and agenesis of the cerebellar vermis by Magnetic Resornance Image. From these two cases, we describe the correlation between the cerebellar vermian dysfunction and the ocular motor apraxia.

MRI Measurement of Normal Cerebellar Vermis / 实用放射学杂志

Objective To measure the dimention and area of the cerebellar vermis and determine the effects of age and sex on the size of the cerebellar vermis in healthy adults.Methods We estimated the dimention and area of the cerebellar vermis in 360 healthy volunteers,20～87 years old.Results We found a significant reduction in the anterior-posterior dimention and the superior-inferior dimention after 60 years old.The anterior lobule area and the total area of the cerebellar vermis were larger in male than that in female.However,the analysis of age trends in the verian lobules revealed differential age related declines.Conclusion The dimention and area of cerebellar vermis reduced with aging after 60 years old.The area of the anterior vermis is larger in men than that in women.

A Case of Cavernous Angioma of the Cerebellar Vermis

A case of cavernous angioma of the cerebellar vermis is described. Cevernous angioma is rare vascular malformation of the central nervous system and the cerebellar vermis is very unusual site. Exact preoperative diagnosis of this lesion is difficult because cavernous angioma is angiographically silent and generally accepted to be very rare. The relevant literature is reviewed.

A Case of Oligodendroglioma of Cerebellar Vermis in a Child

The incidence of oligodendroglioma in pediatric age is less than 1%, and about 90% of them are located supratentorially. The authors are reporting a case of oligodendroglioma at the cerebellar vermis in a 14 years old boy which was cured by surgical resection and shunt procedure.