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: The intrinsic lesions of the brainstem in children lead to a variety of diseases. Although their differentiation is important; however, their diagnosis is difficult via neuroradiological imaging alone. Herein, we report a pediatric case of a developmental venous anomaly (DVA) sited at the brain stem. The patient’s initial head MRI revealed an intrinsic lesion with ill-defined and brush-like contrast enhancements, which was primarily suspected to be a brain tumor. However, the atypical clinical course of the patient and neuroimaging findings such as the rapid exacerbations and remissions in a short time period, and rack of lesion volume effect observed in MRI, refrain us from proceeding with highly invasive examination and initiating any type of treatment including chemotherapy, radiotherapy and ／ or administration of steroids. Consequently, a complete recovery of symptoms and improvement in neuroimaging findings were achieved during the natural course of the disease. Retrospectively, MRI with contrast mater at the initial visit revealed a DVA traversing across the medulla oblongata, suggesting that the this case’s pathophysiology was based on a venous malformation of the medulla oblongata with stagnant outflow and stenosis, causing increased venous pressure and contributing to the clinical symptoms and findings on neuroradiological imaging. In this study, we report this extremely rare case of DVA with a literature review. [ABSTRACT FROM AUTHOR]

Cisternography using heavy T2-weighted images from 3-Tesla magnetic resonance imaging(MRI)and three-dimensional time-of-flight MR angiography(3D TOF MRA)is useful for identifying conflicting vessels in primary hemifacial spasm(HFS). Cisternography provides high-signal images of the cerebrospinal fluid and low-signal images of the cranial nerves and cerebral blood vessels, whereas 3D TOF MRA provides high-signal images with only vascular information. The combination of these two methods increases the identification rate of conflicting vessels. The neurovascular conflict(NVC)site in HFS is where the facial nerve exits the brainstem. However, on MRI, the true NVC site is often more proximal than the facial nerve attachment to the brainstem. On preoperative MRI, it is important to not miss the blood vessels surrounding the proximal portion of the facial nerve. If multiple compression vessels or deep vessels are located in the supraolivary fossette, they may be missed. Coronal section imaging and multiplanar reconstruction(MPR)minimize the chances of missing a compression vessel. Preoperative MRI and CT can also provide various other information, such as volume of the cerebellum, presence of emissary veins, shape of the petrosal bone, and size of the flocculus.

In microvascular decompression surgery for trigeminal neuralgia, the veins are essential as an anatomical frame for the microsurgical approach and as an offending vessel to compress the trigeminal nerve. Thorough arachnoid dissection of the superior petrosal vein and its tributaries provides surgical corridors to the trigeminal nerve root and enables the mobilization of the bridging, brainstem, and deep cerebellar veins. It is necessary to protect the trigeminal nerve by coagulating and cutting the offending vein. We reviewed the clinical features of trigeminal neuralgia caused by venous decompression and its outcomes after microvascular decompression. Among patients with trigeminal neuralgia, 4%-14% have sole venous compression. Atypical or type 2 trigeminal neuralgia may occur in 60%-80% of cases of sole venous compression. Three-dimensional MR cisternography and CT venography can help in detecting the offending vein. The transverse pontine vein is the common offending vein. The surgical cure and recurrence rates of trigeminal neuralgia with venous compression are 64%-75% and 23%, respectively. Sole venous compression is a unique form of trigeminal neuralgia. Its clinical characteristics differ from those of trigeminal neuralgia caused by arterial compression. Surgical procedures to resolve venous compression include nuances in safely handling venous structures.

Human speech and vocal communication of nonhuman primates share many features in semantic ability such as referentiality of calls or pragmatic inference. By contrast, a large gap is found in the phonological ability; vocal learning or volitional vocal control are hardly observed in nonhuman primates. However, recent studies have shown similarity between speech and the vocal, facial communication of nonhuman primates used in low-arousal situations. Here I focused on such vocalizations, called contact calls and close calls. These vocalizations are widely seen among primate taxa. Contact calls function to keep group cohesiveness. Close calls are soft vocalizations used in face-to-face communication and function to signal the benign intent of the caller. These vocalizations exhibit the subcomponent abilities that enables human speech as follows. First, they show some plasticity in usage and acoustic features. The use of close calls becomes partner-specific as individuals mature, and this process is influenced by other group members. The acoustic features of contact calls are modified not only developmentally through interactions with parents but also temporally during vocal exchanges. Second, just as turn-taking in human conversation, timing adjustment has been found in both contact calls and close calls, indicating a vocal coordination ability in nonhuman primates. Third, the facial movements of close calls or lip-smacking have been revealed to be putative precursors of speech rhythm. Finally, some studies have shown that monkeys can acquire volitional vocal control through training. Although primates lack direct projection from the cortex to the vocal control region in the brainstem, initiation of such vocal utterance or lip-smacking involves similar brain areas as in human speech. Further investigation of vocal control during contact and close calls in social, natural settings as well as in experimental settings, would contribute to a better understanding of the evolutionary course leading to speech. [ABSTRACT FROM AUTHOR]

Chiari malformation type I (CM-1) is a disease in which part of the cerebellum and brainstem invades into the spinal canal beyond the foramen magnum. Patients with CM-1 can present with various symptoms; however, most cases of CM-1 are asymptomatic. Symptomatic cases are distributed bimodally in children and middle-aged adults, but occur very rarely in elderly individuals. We experienced a case of CM-1 onset after the age of 60 years that followed a favorable postoperative course. We report the potential mechanism of asymptomatic CM-1 in elderly patients along with a review of the literature.

Background: Diffuse midline glioma, H3K27M mutant is a glioma located in the thalamus, brainstem, or spine with the H3K27M mutation, which is a new entity in the 2016 revised WHO classification. The treatment of thalamic glioma(TG)and brainstem glioma(BSG), which includes diffuse midline gliomas, the H3K27M mutant is challenging, and there are no standard therapeutic strategies. It is important to determine the characteristics of these brain tumors. Here, we retrospectively reviewed 31 consecutive patients with TG and BSG who were treated at our institute between January 1994 and May 2018, including methionine-positron emission tomography(MET-PET)data., Results: Fourteen patients had TG, while 17 patients had BSG. Six patients were children, and 25 were adults. Nine patients with TGs and seven with BSG were enhanced by gadolinium. Twenty-seven patients were treated with radiotherapy, and 20 patients were treated with chemotherapy. All 21 tumors that underwent surgery showed wild-type IDH. The H3K27M mutation was present in four TG and two BSG. There was no statistically significant association between methionine uptake and gadolinium contrast enhancement and tumor grade. The median overall survival period(OS)of all cases was 16.9 months, whereas those of TG and BSG were 22.8 and 10.0 months, respectively., Conclusion: Because TG and BSG still have poor prognoses, it is necessary to elucidate the pathology of the disease and establish its standard therapy.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a brainstem predominant lymphocytic inflammatory disease, which often relapses without oral immunosuppressants. This report describes a 37-year-old male case of CLIPPERS without relapse for 1 year after early steroid treatment. He was admitted to our hospital because of sensory disturbance in the left side of his body and ataxic gait. Gadolinium-enhanced T 1 -weighted MRI revealed multiple punctate and curvilinear enhancements in the pons and right middle cerebellar peduncle. We started treatment with high-dose intravenous methylprednisolone (IVMP) therapy on the 20th day of the illness. His neurological symptoms dramatically improved. Follow-up MRI showed that the enhancing lesions disappeared. We diagnosed him with CLIPPERS based on the clinical course, radiological findings, and steroid response. He did not take any oral immunosuppressant after discharge. However, there was no clinical and radiological relapse for 1 year after the IVMP therapy. Although this case requires careful follow-up because of recurrence risk, early steroid treatment was possibly related to 1-year remission.

Most cases of the primary brainstem injuries(PBSI)are fatal, and disturbance of consciousness is often prolonged even if lifesaving is obtained. The mechanisms of PBSI are as follows: diffuse axonal injury from acceleration/deceleration, shear strain at the midbrain, direct injury of neurovascular structures by tentorial margin, and lower brainstem injury by hyperextension of the cervical vertebrae. Though we can use both CT and MRI to diagnose, MRI is more helpful than CT in detecting, localizing, and characterizing PBSI. When the location of PBSI is limited in the ventral side of pons, it may occasionally result in locked in syndrome(LIS). Generally it is difficult to diagnose LIS with severe trauma due to the rarity of this syndrome caused by head injury. Here, we report a case of an elderly man with traumatic brainstem hemorrhage, who transiently presented LIS and finally improved.

A 33-year-old woman was admitted due to disturbance of consciousness, dysarthria, dysphagia, sensory disturbances and weakness of the left upper limb after mycoplasma infection. She was treated with intravenous immunoglobulin and intravenous high-dose methylprednisolone as Bickerstaff brainstem encephalitis (BBE). On the 15th hospital day, reflex myoclonus appeared on her face, neck, body and limbs induced by techniques of jaw jerk reflex and patellar tendon reflex. The myoclonus was disappeared after two weeks in accordance with improvement of BBE. The transient reflex myoclonus may be originated from brainstem lesion which was affected by BBE. Reflex myoclonus is thought to be rare symptom in patient with BBE.

Objective: Endovascular coil embolization of intracranial aneurysms is associated with better outcomes and a lower mortality rate compared with surgical clip occlusion. However, a principal disadvantage of endovascular therapy is the higher rate of retreatment compared with neurosurgical clipping. Self-expandable hydrogel-coated coils were developed to reduce recanalization rates of cerebral aneurysms by promoting complete volumetric aneurysm occlusion. Herein, we report a case of brainstem hemorrhage following coil embolization of a large basilar aneurysm with hydrogel-coated coils., Case Presentation: A 65-year-old female with a history of hypertension, who presented with worsening headaches, right hemiplegia, and left oculomotor palsy, underwent endovascular treatment for a large basilar aneurysm. The aneurysm was treated with both hydrogel-coated coils and bare platinum coils. Hydrogel-coated coils represented 46% of the coil length in the aneurysm. The patient was discharged from the hospital with improvement of neurological deficits 6 days after the procedure. However, the patient was readmitted with perianeurysmal edema in the midbrain 23 days after coil embolization. Follow-up angiography 26 days after the procedure showed complete obliteration of the aneurysm. Two weeks later, the patient presented with a large brainstem hemorrhage and died. Pathological findings revealed intraparenchymal hemorrhage in the pons without rupture of the aneurysm., Conclusion: Hydrogel-coated coils may cause a marked inflammatory response that may result in intracerebral hemorrhage.

Several previous reports have established the Pentax Airwayscope (Pentax AWS, S-100, HOYA-PENTAX, Tokyo, Japan) as an efficient tool for tracheal intubation in adult patients. The Pentax AWS is often successfully used with an INTLOCK blade; to date, however, INTLOCK blades have been released for neonatal and pediatric patients only. In this case, we performed tracheal intubation using a Pentax AWS attached to a pediatric-type INTLOCK blade (ITL-P) in an adult patient fitted with a Leksell Stereotactic frame (Elekta, Sweden). The patient weighed 45 kg and was 154 cm tall, and was scheduled for a tumor biopsy due to glioblastoma in the brain stem. The patient was preoperatively fitted with a Leksell frame on her head. The patient was not premedicated and was monitored with electrocardiography (ECG), noninvasive blood pressure, and pulse oximetry. Following pre-oxygenation, general anesthesia was induced using propofol 4.0 microg x ml with target-controlled infusion and remifentanil 0.25 microg x kg(-1) hr(-1). After loss of consciousness, we administered 30-mg rocuronium boluses. We initially attempted tracheal intubation first using a Macintosh laryngoscope and then a Pentax AWS, but we could not achieve tracheal intubation with either of these instruments. Upon switching to a Pentax AWS with an ITL-P, we successfully achieved tracheal intubation without any complications. Anesthesia was maintained uneventfully with 3.0 microg x ml(-1) propofol and remifentanil 0.10 to 0.25 microg x kg(-1) x hr(-1) in oxygen and air. Further study is needed to facilitate the effective use of the Pentax AWS and the ITL-P in such cases.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare central nervous system inflammatory disease characterized by the punctate gadolinium enhancement peppering the pons and the cerebellar peduncles as neuroimaging. We report the case of a 66-year-old woman who presented with CLIPPERS associated with swelling in the brainstem. She was hospitalized because of gait ataxia and consciousness disturbance. MRI of the brain showed FLAIR hyperintense lesions in the pons, cerebellar peduncles, cerebellum and the subcortical white matter lesion in the right occipital lobe with significant swelling in the brainstem. Diffusion-weighted MRI did not show an abnormal signal, indicating vasogenic edema. Post-contrast T1-weighted MRI showed enhanced area in the right occipital lobe and panctate gadolinium enhancement peppering brainstem. Treatment with steroids led to rapid improvement. However, she showed exacerbation of clinical and radiological findings during the tapering schedule of steroid. The biopsy from the occipital lobe revealed intense perivascular and parenchymal lymphocytic infiltrates composed of primarily T cells, B cells and macrophages. The patient was diagnosed with CLIPPERS, and treatment with increased dose of corticosteroid induced a clinical improvement. Previous reports well described a characteristic MRI finding of punctate enhancement peppering the pons. In addition, the pons and cerebellar peduncles swelling can occur in this disorder.

Fisher syndrome has been regarded peculiar inflammatory neuropathy with ophthalmoplegia, ataxia, and areflexia, whereas Bickerstaff brainstem encephalitis has been considered pure central nervous system disease characterized with ophthalmoplegia, ataxia, and consciousness disturbance. Both disorder share common features including preceding infection, albumin-cytological dissociation, and association with Guillain-Barre syndrome. The discovery of anti-GQ1b IgG antibodies further supports the view that the two disorder represent a single disease spectrum. Currently Bickerstaff brainstem encephalitis can be regarded as a variant of Fisher syndrome with central nervous system involvement.

Bickerstaff brainstem encephalitis (BBE) is characterized by acutely progressive bilateral ophthalmoparesis and ataxia with impaired consciousness or pyramidal signs, or both; all of which are followed by a monophasic course with good recovery. Alike Guillain-Barré syndrome (GBS), BBE is proposed to have an autoimmune mechanism triggered by antecedent infection. The nationwide epidemiologic survey for BBE, which the author had performed in Japan, suggests that BBE consists of typical and atypical cases. Typical BBE has similar neurological and serological features to Fisher syndrome and shows good recovery, whereas atypical BBE is characterized by delayed recovery, negative anti-GQ1b antibodies, and abnormal cerebrospinal fluid and brain MRI findings with other possible pathogeneses.

The incidence of brain abscess remains high, despite the development of novel antibiotics. Vancomycin or carbapenems, which are third-generation cephems, are recommended as standard therapy for bacterial meningitis or brain abscess. The effectiveness of the high-dose meropenem therapy on brain abscess has occasionally been reported. We experienced 2 consecutive cases of brain abscess in adults. The first patient was a 67-year-old man with diplopia, dizziness, and dysesthesia on the left upper and lower extremities. Images of T1-weighted magnetic resonance imaging (MRI) with contrast medium and diffusion-weighted MRI showed a ring enhancing cystic lesion and a high intensity lesion, respectively, in the right pons. The second patient was a 37-year-old man who complained of right hemiparesis. MRI revealed a ring-enhancing cystic mass in the left thalamus. On the basis of MRI findings, patients were diagnosed with brain abscess and were given high-dose meropenem (6g/day) continuously for 2 months. The abscess resolved completely after treatment with meropenem administered intravenously. Further, neurological deficits caused by abscess successfully improved. High-dose meropenem therapy should be considered as an effective treatment for brain abscess, even in the brain stem and basal ganglia, where it is quite difficult to achieve surgical access.

We report the case of a 30-year-old man who developed severe dysphagia owing to neuroborreliosis. He showed dysphagia, diplopia, hiccups, and walking difficulty Neurological examination revealed mild disturbance of consciousness, diplopia on left lateral gaze, left-side-dominant blephaloptosis, gaze-evoked horizontal nystagmus on left lateral gaze, mild bilateral muscle weakness, palatoplegia, dysphagia, dysarthria, and truncal ataxia An increased pharyngeal reflex caused dysphagia in this patient. An EEG revealed intermittent high amplitude slow wave activity. However, head MRI, blood count, serum chemistry, and cerebrospinal fluid examination showed no abnormality. Initially, brainstem encephalitis with unknown etiology was diagnosed. The hiccups, diplopia, and ptosis were improved by corticosteroid therapy, but other symptoms were refractory to corticosteroid therapy and IVIg. After these immunotherapies, anti-Borrelia IgG and IgM antibodies were found to be positive, and symptoms, including dysphagia, were improved by doxycycline and cefotaxime. Because the clinical symptoms of Borrelia infection are widely variable, neuroborreliosis should be considered in patients with brainstem encephalitis refractory to conventional immunotherapies.

A rare case of a brainstem variant of reversible posterior leukoencephalopathy syndrome (RPLS) is reported. A 34-year-old man with a past history of untreated chronic renal failure and hypertension was admitted to our hospital complaining of severe nuchal headache lasting for about four days. His neurological examination was normal, however physical examination revealed his blood pressure was 216/120 mmHg. Computed tomography imaging (CT) showed his brain stem with a low attenuation. Magnetic resonance imaging (MRI) revealed extensive hyperintensity and enlargement of the midbrain and pons on T2 weighted and fluid attenuated inversion-recovery (FLAIR) image. However, there was no abnormal lesion seen in either bilateral occipital lobe. Diffusion-weighted image (DWI) was normal at the brainstem, but apparent diffusion coefficient (ADC) values were slightly elevated at the left midbrain. There was no contrast enhancement. His symptom and radiological finding improved soon after his blood pressure was controlled. A repeated MRI taken two weeks later showed complete resolution of the lesion. RPLS associated with predominant involvement of the brainstem and sparing of the supratentorial region is rare, but it should be differentiated from brain stem infarction, pontine glioma, central pontine myelinolysis and infective encephalitis, since the neulological consequences are potentially fully reversible after adequate and prompt treatment.

A 62-year-old man was admitted to our hospital because of rapidly progressive dysarthria, truncal ataxia, and gait disturbance. High titers of the ProGRP and anti-P/Q-type VGCC antibody were detected in the serum. High accumulation of [18F] was detected at the hilus of the left lung on [18F]-FDG-PET scan. A high-frequency repetitive stimulation test of the median nerve yielded an incremental response. On the basis of these findings, a diagnosis of paraneoplastic cerebellar degeneration (PCD) and Lambert-Eaton myasthenic syndrome (LEMS) associated with small cell lung carcinoma (SCLC) was diagnosed. After intravenous immunoglobulin therapy (IVIg), methylprednisolone (m-PSL) pulse therapy, and other multidisciplinary concurrent treatments, a partial regression of the SCLC and a significant improvement in neurological symptoms were observed. However, ataxia relapsed and brainstem encephalitis developed 6 months later. A marginal improvement in neurological symptoms was observed with IVIg, m-PSL pulse therapy, and intravenous cyclophosphamide pulse therapy (IVCY). SCLC also recurred later. We hypothesized that VGCC of the brainstem was damaged by anti-P/Q-type VGCC antibody.

While interest in headache research started early in Japan, headache cares did not develop until recently. Patients with migraine did not visit doctors for headache, and physicians were unaware of the prevalence and disability of migraine in Japan for a long time. Studies in Japan on migraine epidemiology revealed that the prevalence of migraine was 8.4% of the population, demonstrating 8.4 million people are suffering from migraine. These results gave impact to young scientists in Japan and encouraged them to do clinical and experimental studies on headache. One of the barriers for studying headache was a difficulty in communication to obtain enough information by the physicians. Patients usually suffered in silence. New medication for migraine, triptan, increased the number of patients visiting physicians. Physicians also developed such communication tools as headache diary, migraine screening tools helping physicians to obtain good amount of information on headache. The purpose of this communication is to present the author's experience in the study of mechanism and treatment of headache based on headache diary. The importance for future progress of migrainology was emphasized.

A 39-year-old man acutely developed diplopia, vertigo, unsteady gait, and disturbance of consciousness following an upper respiratory infection. Neurological examination showed ophthalmoplegia, facial paralysis, tetraplegia and loss of deep tendon reflexes. Babinski reflex was positive on the left and there were bilateral flexor withdrawal reflexes. He also developed ballism-like involuntary movements in all extremities, loss of proprioception predominantly on the left, and severe truncal ataxia. Anti-GQ1b IgG antibody was selectively elevated in serum, and CSF protein was elevated to 53 mg/dl with cell count of 12/mm3. Nerve conduction study showed decreased amplitude of compound motor action potentials in all extremities, and no response in facial muscles. Cranial MRI showed no abnormalities whereas EEG was severely abnormal with lack of posterior dominant rhythm and the presence of continuous diffuse theta-waves. This case presented clinical characteristics of three syndromes concurrently-Fisher syndrome, Bickerstaff brainstem encephalitis, and Guillain-Barré syndrome-that may be collectively called 'anti-GQ1b IgG antibody syndrome'. The unique feature of the present case was development of deep coma and ballism-like movements, associated with selective increase of serum anti-GQ1b IgG antibody. It is thus conceivable that anti-GQ1b IgG antibody might underlie the pathogenesis of all three conditions.

A 72-year-old male with liver cirrhosis and hepatocellular carcinoma experienced general fatigue. Four days later he was admitted to our hospital because of dizziness, dysbasia and left facial palsy (day 1). On day 6, a neurological examination revealed left trigeminal neuralgia, left medial longitudinal fasciculus (MLF) syndrome, skew deviation, hypacusia, tongue deviation and left limb ataxia. Magnetic resonance imaging of the brain including diffusion-weighted imaging showed previous lacunar infarctions at the left thalamus and pons. The immunological investigation for viral infection in his serum samples showed high titers of IgM antibody against cytomegalovirus (CMV). Cerebrospinal fluid (CSF) investigation revealed mononuclear pleocytosis, elevated protein levels and high titers of IgG antibody against the varicella-zoster virus (VZV). Anti-CMV antibody measurement and CMV-DNA detection by the polymerase chain reaction in CSF revealed that the central nervous system (CNS) was not infected by CMV. We diagnosed this case as brainstem encephalitis following multiple cranial neuropathy associated with CMV and VZV infections. The neurological symptoms gradually improved with aciclovir and prednisolone therapy. The titers of antibody for CMV in his serum samples normalized 4 months later after onset. Although there was no evidence of CMV infection in the CNS was obtained, parainfection or autoimmune mediated responses followed by viral infections might have led to brainstem encephalitis with multiple cranial nerve involvements in our patient.

We report a case of a dissecting vertebral aneurysm with subarachnoid hemorrhage (SAH) after ischemic onset on the same day. A 48-year-old man had abrupt vertigo and nausea. CT & MRI on admission showed no abnormality, but he complained of left hemiparesis after admission. Twelve hours after the ischemic onset he suddenly complained of severe headache and his consciousness deteriorated. The follow-up CT showed diffuse SAH. Cerebral angiography showed occlusion of the right vertebral artery at the origin of the posterior inferior cerebellar artery (PICA) and segmental stenosis of the left vertebral artery at the portion distal to the vertebral PICA junction. We treated the patient conservatively. Four days later, he suddenly fell into a coma, but CT showed no bleeding. Because of this we suspected brain stem ischemia due to deterioration of vertebral dissection. The patient died 8 hours after the ischemic reattack. We report difficulty of treatment of a dissecting vertebral aneurysm with simultaneous ischemia and subarachnoid hemorrhage.

This paper reviews a variety of abnormal eye movements which include abnormal ocular positions, restricted eye motions, impairment of conjugated eye movements, abnormal smooth pursuit, abnormal saccade, gaze-evoked nystagmus, down-beat nystagmus, internuclear ophthalmoplegia, supranuclear ophthalmoplegia, square wave jerks, roving eye movement, ocular bobbing, ocular dipping, reverse ocular bobbing, and ping-pong gaze. Abnormal eye movements occur from stroke, spinocerebellar degeneration, Parkinson disease, multiple system atrophy, progressive supranuclear palsy, multiple sclerosis, Miller Fisher syndrome, myasthenia gravis, opsoclonus-polymyoclonia syndrome, and Creutzfeldt-Jakob disease. In neurological practice, it is important to observe abnormal eye movements accurately and enthusiastically, to make appropriate anatomical and etiological diagnosis.

Brainstem encephalitis is a rare form of encephalitis which should be differentiated from cerebrovascular and neoplastic diseases. The authors report a case of viral brainstem encephalitis mimicking malignant lymphoma. A 55-year-old female was admitted to our hospital with gradually progressive diplopia and left hemiplegia. CT scan revealed low density lesions in the right globus pallidus and the anterior limb of the internal capsule. MRI demonstrated high intensity signals extending into the right cerebral peduncle, temporal lobe, thalamus and the contralateral thalamus on FLAIR images. Petechial hemorrhages were seen in the affected lesions, but no enhancement was observed following administration of a contrast material. CSF examination revealed mild mononuclear cell dominant pleocytosis. Both early and delayed images of 123I-IMP SPECT revealed marked hot spots corresponding to the lesions on FLAIR images. CT-guided stereotactic biopsy was useful for early diagnosis.

A 28-year-old woman initially suffered high fever and headache (day 1). Aseptic meningitis was diagnosed on day 3. Limb ataxia, however, appeared on day 7, and external ophthalmoplegia and drowsiness were recognized on day 8. Urinary disturbance and orthostatic hypotension appeared on day 13. Cerebrospinal fluid showed moderately high cell counts(mononuclear cells, 51/microliter; polynuclear cells, 9/microliter). MRI T2-weighted images showed high intensity lesions in the pons and mesencephalon. No serum anti-GQ1b IgG antibody was detected on day 4. Epstein-Barr virus (EBV) viral capsid antigen-IgG antibody was positive, and EBV determined nuclear antigen antibody was seroconverted. EBV-DNA was detected in the CSF by PCR. These findings indicate prior infection by EBV. After intravenous dexamethasone therapy, these symptoms rapidly disappeared. Our patient showed external ophthalmoplegia, ataxia, and disturbance of consciousness, which are the cardinal signs in Bickerstaff's brainstem encephalitis. The time course of her neurological symptoms, the presence of meningitis, and the MRI findings, however, indicated the pathogenesis of acute disseminated encephalomyelitis rather than Bickerstaff's brainstem encephalitis. We diagnosed this patient as a brainstem encephalitis associated with EBV infection.

Patient was an 18-year-old female student. After she had symptoms of common cold for 3 days, she developed somnolence, diplopia, dysarthria, urinary disturbance and ataxia. On admission neurological examination revealed coma with mydriasis, ophthalmoplegia, ptosis and weakness of the upper limbs. Light reflex, corneal reflex and oculocephalic test were all negative. Deep tendon reflexes were brisk and extensor toe signs were positive bilaterally. She did not have nuchal rigidity. Laboratory test revealed normal cerebrospinal fluid with negative myelin basic protein. Brain MRI, brainstem evoked potentials presented no abnormality. EMG revealed normal conduction velocity and no conduction block. EEG had diffuse theta and delta slowing. Culture of the stool represented no Campylobacter jejuni. At the fifth day of admission consciousness level improved, and other neurological findings disappeared in about 6 weeks. She had anti GQ 1 b, GT 1 a(IgG, IgM) and anti GM 1(IgM) antibodies in the serum. We made a diagnosis of Bickerstaff's brainstem encephalitis from these neurological symptoms and clinical course. The main lesion was present in the brainstem from midbrain to medulla oblongata in the midline. High titer of anti GT 1a antibody may be related to the ophthalmoplegia as noted in Miller Fisher syndrome. As a result of EMG and stool culture, it denied the complication of Guillain-Barré syndrome. We had no proof of the reason of the presentation of anti GM 1 antibody.

We report here a case of acute necrotizing encephalopathy (ANE) showing brainstem hemorrhage. A 5-year-11-month-old boy with a history of febrile seizures was admitted to our hospital because of high fever and coma. Laboratory studies on admission demonstrated elevated serum transaminase and blood glucose. Brain CT was normal on admission, but 9 hours later, it showed low density areas in the bilateral thalamus, putamen, midbrain, pons and cerebellum. Brain MRI revealed abnormal short T1 and long T2 signals in the same areas. Despite promptly performed intensive care, massive brainstem hemorrhage occurred on the fourth day. EEG showed a suppression-burst pattern followed by a flat pattern on the sixth day. The patient died on the eighth day of multiple organ failure. This is the first case of massive brainstem hemorrhage that occurred in association with ANE.

Here we present a case of 23-year-old woman with Bickerstaff's brainstem encephalitis (BBE) during 13 weeks of pregnancy. After symptoms of the upper respiratory tract infection, she developed somnolence, marked disturbance of extraocular eye movements, cerebellar ataxia, hyperreflexia and spasticity of the lower limbs. An electroencephalogram revealed slow waves and brainstem auditory evoked potential disclosed prolongation of III-V interpeak intervals. Serum IgG anti-GQ1b antibody was detected. Plasma exchanges (PE) were performed four times. Although no patient with BBE during pregnancy has ever been reported, in the literatures the pregnant patients with Guillain-Barré syndrome had four sessions of plasmapheresis with safe. Neurological symptoms gradually improved after the PE. No serious adverse effects were noted. Although no definite therapy for BBE has been established, PE may be one of the selections even if the patients is pregnant.

A 54-year-old female developed dysarthria, left limb ataxia, and crossed dissociated sensory impairment, followed by external ophthalmoplegia, severe bulbar palsy and bilateral pyramidal tract involvement. A high titer of anti-GQ1b IgG antibody was detected in her serum. Brain MRI (T2 weighted image, diffusion weighted image) and peripheral nerve conduction study were normal. We diagnosed her as having Bickerstaff's brainstem encephalitis. In the past literature, we could not find any case of Bickerstaff's brainstem encephalitis showing crossed dissociated sensory impairment.