Rotavirus associated acute encephalitis/encephalopathy and concurrent cerebellitis: report of two cases

It is known that rotavirus gastroenteritis can accompany some neurological manifestations, including encephalitis/encephalopathy or seizures. However, the detailed pathogenesis involved has not been fully understood. To date, acute cerebellitis associated rotavirus gastroenteritis has not been previously reported, except for one case. Herein, we describe two cases of acute encephalitis/encephalopathy and concurrent cerebellitis, associated rotavirus gastroenteritis. Following vomiting and diarrhea, case 1 experienced convulsions and consciousness disturbance and case 2, transient loss of consciousness with eye deviation. After these symptoms subsided, cerebellar signs became evident and a brain MRI showed cerebellar involvement in both cases. Both cases showed speech disturbances, such as mutism, slow speech and dysarthria. In this report, we will discuss the possible pathogenesis of rotavirus associated acute encephalitis/encephalopathy and concurrent cerebellitis.     

Cerebellar involvement as a rare complication of pneumococcal meningitis

A 4-year old girl with meningitis, caused by streptococcus pneumoniae, developed a subcoma with respiratory insufficiency, followed by a severe cerebellar syndrome. Cerebellar involvement after regaining consciousness consisted of a symmetrical ataxia and mutism. This mutism changed into dysarthria and finally into normal speech. Magnetic resonance imaging revealed lesions in both cerebellar hemispheres, suggesting cerebellitis. She recovered with prompt antibiotic treatment.     

Chronological diffusion-weighted imaging changes and mutism in the course of rotavirus-associated acute cerebellitis/cerebellopathy concurrent with encephalitis/encephalopathy

Rotavirus is one of the most common causes of gastroenteritis in children and is known to accompany some neurological disorders such as encephalitis/encephalopathy and seizures. Although cerebellar disorders sometime occur as a complication of rotavirus gastroenteritis in Japan, few reports have addressed these issues. Here, we report three cases of insulted cerebellums in addition to encephalitis/encephalopathy associated with rotavirus. Similar to posterior fossa syndrome after surgery, mutism was a notable symptom that lasted about 1 month. Brain diffusion-weighted imaging (DWI) revealed chronological changes, i.e., marked hyperintensity in the bilateral dentate nucleus followed by the vermis and cerebellar hemisphere. The bilateral dentate nucleus is known to be a key lesion site for mutism, and these clinical and radiological findings may be tightly connected in rotavirus-associated cerebellitis/cerebellopathy.     

Neuroimaging of Acute Cerebellitis

Acute cerebellitis is one of the main causes of acute cerebellar dysfunction in childhood and may be infectious, postinfectious, or postvaccination. The etiology of acute cerebellitis is usually viral. Varicella zoster, Epsten-Barr, rubeola, pertussis, diphtheria, and coxsackie viruses are the most frequently involved agents. Diagnosing of acute cerebellitis can sometimes be difficult because the patient may present only mild cerebellar signs and the examination of cerebrospinal fluid may be normal. The authors present the clinical and neuroimaging findings of 2 patients presenting with acute cerebellitis. Their magnetic resonance imaging showed hyperintense signal of cerebellar gray matter in T2-weighted sequences, which is a strong indication of a diagnosis of acute cerebellitis.     

Fulminant cerebellitis with radiological recurrence in an adult patient with Crohn's disease

There are few reports of cerebellitis in adults and reports of recurrent cerebellitis are extremely rare. This report highlights both the fulminant course that may follow cerebellitis and a potentially important association with Crohn's disease. A 41-year-old man presented with headache and cerebellar dysfunction a month after ileo-colonic resection for Crohn's disease. MRI demonstrated T2-signal abnormalities, pial enhancement and cerebellar enlargement. His course was complicated by obstructive hydrocephalus, intraventricular hemorrhage associated with external ventricular drain placement and left transverse sinus venous thrombosis. Treatment with high-dose intravenous steroids and anti-coagulation resulted in improvement. Asymptomatic radiological worsening 13 months after initial presentation occurred during a relapse of Crohn's disease. He was treated with intravenous steroids and maintenance azathioprine for his Crohn's and suspected immune mediated cerebellitis. At last follow-up 4 years after initial presentation his clinical status remained unchanged and radiological changes had stabilized. Adult onset cerebellitis may be fulminant and recurrent. Early immunotherapy in acute relapsing cerebellitis may speed recovery and prevent recurrence. Onset and radiological relapse of cerebellitis during Crohn's disease flare-ups as in our case possibly suggests a shared autoimmune pathogenesis.     

Acute cerebellar ataxia and consecutive cerebellitis produced by glutamate receptor delta2 autoantibody

Acute cerebellar ataxia is usually a self-limited benign disease, which may develop in children after certain viral infections or vaccinations. There are several reports of acute cerebellar ataxia associated with autoantibodies. Glutamate receptor delta2, a member of the glutamate receptor family, is predominantly expressed in cerebellar Purkinje cells and plays a crucial role in cerebellar functions. To date anti-GluRdelta2 autoantibody was detected in a patient with chronic cerebellitis. Herein, an 18-month-old boy presented with cerebellar ataxia 9 days following a mild respiratory tract infection. Although cerebellar ataxia gradually improved, it worsened yet again following mumps and varicella virus infection. Cerebro-spinal fluid examination and magnetic resonance imaging of the brain demonstrated pleocytosis and meningeal enhancement, respectively. Furthermore, glutamate receptor delta2 autoantibody was detected in serum and cerebro-spinal fluid. Thus, we believe that the glutamate receptor delta2 autoantibody may play a role in cerebellar ataxia and consecutive cerebellitis.     

Postinfectious hemicerebellitis

The clinical signs of cerebellitis are usually bilateral and symmetrical. We report the case of a 9-year-old girl who presented with acute hemiataxia and flaccid hemiparesis associated with a lesion in the ipsilateral cerebellar cortex two weeks after a viral upper respiratory tract infection. The clinical outcome was good without treatment. Imaging follow-up showed hemiatrophy of the cerebellar cortex.     

An unusual presentation of varicella zoster virus with acute cerebellitis and SIADH without a rash

We report a case of varicella-zoster virus (VZV) infection with acute cerebellitis and encephalitis with associated Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) in an elderly man presenting with acute cerebellar ataxia without antecedent rash. Cerebrospinal fluid examination (CSF) revealed a mononuclear pleocytosis, high protein, normal glucose, positive for VZV polymerase chain reaction (PCR). Early acyclovir treatment is beneficial for acute VZV cerebellitis. Clinicians should consider infectious Central Nervous System (CNS) causes for presentations of acute cerebellar ataxia in adult patients, particularly if there is an accompanying clouded sensorium.     

Nonsurgical cerebellar mutism (anarthria) in two children

Cerebellar mutism (anarthria) is a well-described complication of posterior fossa tumor resection. It is accompanied by a characteristic behavior including irritability and autistic features. This syndrome is typically reversible within days to months. Underlying pathophysiology is unknown. We describe two children who presented with a similar clinical finding after nonsurgical cerebellar involvement, hemolytic-uremic syndrome in one and cerebellitis in the other. Postmortem pathologic findings in the first patient indicated cerebellar ischemic necrosis. Single-photon emission computed tomography in the second patient revealed diffuse cerebellar hypoperfusion with no supratentorial abnormalities, refuting a phenomenon of diaschisis between cerebellar and frontal connections. These findings confirm that this clinical syndrome may occur in a nonsurgical, nontraumatic context. They are consistent with recent integrative hypotheses explaining cerebellar anarthria.     

Cerebelitis aguda en Pediatría: nuestra experiencia

IntroductionAcute cerebellitis is a rare inflammatory disease with a highly variable clinical course that ranges from benign self-limiting symptoms to a fulminant presentation associated with a high risk of death due to compression of the posterior fossa, acute hydrocephalus, and intracranial hypertension.MethodsWe reviewed clinical, laboratory, and radiological findings from children diagnosed with acute cerebellitis between May 2007 and November 2016. We analysed treatments and clinical and radiological progression.ResultsNine children met the diagnostic criteria for cerebellitis. Headache, vomiting, and drowsiness were the most frequent initial symptoms; ataxia, dysarthria, and dysmetria were the most common cerebellar signs. Cerebellitis was diagnosed with magnetic resonance imaging, which revealed cerebellar involvement (unilateral or bilateral); computerised tomography images either were normal or showed indirect signs such as triventricular hydrocephalus due to extrinsic compression of the aqueduct of Sylvius. Corticosteroids were the most commonly used treatment (6 patients). One patient required surgery due to triventricular hydrocephalus. Eight patients recovered completely, whereas the ninth displayed neurological sequelae.ConclusionsCerebellitis is a medical and surgical emergency; diagnosis requires a high level of suspicion and an emergency brain magnetic resonance imaging study. It is a clinical-radiological syndrome characterised by acute or subacute encephalopathy with intracranial hypertension and cerebellar syndrome associated with T2-weighted and FLAIR hyperintensities in the cerebellar cortex (unilaterally or bilaterally) and possible triventricular dilatation. Treatment is based on high-dose corticosteroids and may require external ventricular drain placement and decompressive surgery.     

An adult case of cerebellar mutism after removal of cerebellar hematoma]

Cerebellar mutism is a well documented complication of posterior fossa tumor surgery in children. But only one case presenting with cerebellar mutism after removal of a cerebellar hematoma in adults was reported previously. We present 64-year-old male developing cerebellar mustism after surgery for hypertensive cerebellar hemorrhage. A cerebellar hematoma was located in the right side dentate nucleus and cerebellar peduncle dominantly not extending to the cerebellar vermis. During the period of cerebellar mutism, single photon emission computed tomography revealed a decrease in the contralateral temporo-parietal cerebral blood flow. Cerebellar mutism in this case may be associated with the low blood flow in the contralateral supplementary motor cortex owing to injury to the dentate-thalamo-cortical pathway at the dentate nucleus and cerebellar peduncle. The relevant literature is reviewed.     

Cerebellitis in an adult with abnormal magnetic resonance imaging findings prior to the onset of ataxia

BACKGROUND: Brain magnetic resonance imaging (MRI) findings during acute cerebellar ataxia in cases of postinfectious cerebellitis are frequently normal. This has resulted in the use of other imaging modalities, such as single-photon emission computed tomography, to aid diagnosis. OBJECTIVE: To illustrate the chronologic occurrence of cerebellar ataxia, abnormal findings on MRI, and cerebral spinal fluid pleocytosis in an adult case of postinfectious cerebellitis. METHODS: Case report. RESULTS: A patient with a 6-week history of occipital headaches and only mild tandem gait difficulty had abnormal MRI findings that were consistent with cerebellar inflammation. As cerebellar ataxia progressed in parallel with cerebral spinal fluid pleocytosis, MRI findings indicative of cerebellar inflammation resolved, while single-photon emission computed tomography showed cerebellar hyperperfusion. Recovery of neurologic function was accompanied by clearing of the pleocytosis and residual MRI-detected cerebellar atrophy. CONCLUSION: This case demonstrates that transient abnormalities can be detected by MRI before clinical manifestations of cerebellitis appear, while hyperperfusion detected by single-photon emission computed tomography is prolonged.     

儿童后颅凹术后缄默综合征

目的探讨儿童后颅凹肿瘤手术后并发缄默综合征的临床特点和发病机理。方法回顾96例儿童后颅凹肿瘤手术,其中8例出现术后缄默综合症,分析其临床特点并结合文献对其发病机理进行讨论。结果8例术后缄默综合征均为大型小脑蚓部肿瘤,其中6例侵及四脑室底部;所有患儿均不同程度的伴有其他神经功能障碍;缄默出现时间0-2d,持续15-78d。结论儿童大型小脑蚓部肿瘤,易发生缄默综合征,该病常伴有多种其他神经功能缺失,常有一定潜伏期,具有自愈性。     

Imaging of acute cerebellitis in children. Report of 4 cases

Acute cerebellitis is one of the main causes of acute cerebellar dysfunction in children. It is either infectious, usually viral, post-infectious or post vaccinal in etiology. Diagnosing acute cerebellitis may be difficult in patients with only subtle cerebellar signs and when cerebro-spinal fluid examination is normal. MRI is the most adequate imaging technique to demonstrate cerebellar involvement. The authors report the clinical and neuro-imaging findings in 4 paediatric cases. Patient's age varied from 2 to 7 years and predominant clinical symptoms were fever, headache and vomiting; ataxia was noted only in 2 cases. Viral serologic tests were negative in 3 cases and demonstrated Epstein-Barr virus in 1. Initial MRI examination (2 cases) demonstrated increased intensity on T2W and Flair sequences of the cerebellar gray matter with pial enhancement. Clinical outcome was good with complete resolution of symptoms in 3 cases and persistent mild right upper limb paresis in one. The resolution of the signal abnormality was well demonstrated on MRI in one case, suggesting an inflammatory etiology with moderate residual cerebellar atrophy.     

Single photon emission tomography following posterior fossa surgery in patients with and without mutism

MATERIALS AND METHODS: We designed a prospective study to investigate single photon emission tomography (SPECT) findings in patients with or without mutism who had undergone posterior fossa surgery. These patients had a (99m)Tc-hexamethylpropyleneamine oxime-SPECT scan postoperatively. SPECT was performed between the 1st and 2nd week after surgery. The patients with cerebellar mutism had a second SPECT scan after regaining their power of speech. The patients were evaluated in terms of age, gender, preoperative and postoperative neurological and radiological findings, tumor size and location, vermian incision and histopathology. RESULTS: Six girls and 5 boys, ranging in age from 3 to 11 years who had undergone posterior fossa tumor surgery were included in this study as controls. Their tumors were located in the vermis and fourth ventricle in 8 patients, in the cerebellar hemisphere in 1, fourth ventricle in 1, and 1 patient had a fourth ventricular tumor extending the cerebello-pontine angle. The histopathology of the tumors was as follows: astrocytoma in 5 patients, ependymoma in 3, medulloblastoma in 1, dermoid tumor in 1, and choroid plexus papilloma in 1 patient. Postoperative cerebellar mutism developed in 2 girls and 1 boy whose ages ranged from 2 to 5 years. Pathological examination revealed 2 medulloblastomas and 1 ependymoma. Mutism developed 2 to 4 days after surgery. All of the patients with cerebellar mutism showed either clinical or radiological signs and symptoms of brain stem involvement. SPECT findings were similar in the patients with cerebellar mutism and in those without. CONCLUSIONS: SPECT findings are not specific enough to explain how cerebellar mutism occurs. Clinical signs of brain stem involvement seem to be a significant risk factor in the development of cerebellar mutism.     

小脑性缄默4例报告及文献复习

目的探讨后颅窝手术后小脑性缄默的临床特点及其形成的病理生理机制。方法回顾性总结分析我科4例和英文文献报告的137例共141例后颅窝手术后出现的小脑性缄默病例。结果儿童病例127例,占90.1%。手术病变位于小脑蚓部者125例(88.7%)。小脑性缄默均为暂时性,发生的平均潜伏期和持续时间分别为1.7d和56.2d。结论小脑性缄默多见于儿童小脑蚓部肿瘤手术后其发生机制可能与小脑齿状核丘脑腹外侧核运动区和辅助运动区之间的纤维联系的直接或间接损害有关。     

Cerebellar mutism after spontaneous intratumoral bleeding involving the upper cerebellar vermis: a contribution to the physiopathogenic interpretation

Background  Transient mutism as a consequence of posterior fossa surgery is a well-known phenomenon. However, it has rarely been reported after focal nonsurgically induced cerebellar damage. Case report  We describe a 7-year-old child affected by a tumor arising from the quadrigeminal plate who developed transient cerebellar mutism after a spontaneous bleeding which extended to the upper cerebellar vermis. The recovery from mutism started about a week after the bleeding. At the time of the surgical treatment, 8 weeks after the spontaneous intratumoral bleeding, she was only dysarthric. Discussion  To our knowledge, this is the first pediatric case of presurgical cerebellar mutism due to a hemorrhage of a neoplastic lesion. Moreover, the focality of bleeding allows the confirmation of the role played by the upper vermis in speech control as well as exclusion of surgically induced lesions commonly suggested as possible cause of the cerebellar mutism.     

An adult case of peripheral facial nerve palsy following acute cerebellitis associated with high antibody titers against varicella-zoster virus]

Here we report a case of acute cerebellitis, in which the patient developed right peripheral facial palsy during the recovery phase of cerebellar ataxia. A 67-year-old man developed truncal and limb ataxia following a fever, general fatigue and anorexia. He was diagnosed to have acute cerebellitis. While the ataxia symptoms were improving without any treatment, right peripheral facial nerve palsy developed and an MRI revealed an enhancement of the right facial nerve proximal to the geniculate ganglion. After treatment with acyclovir and corticosteroids, his facial nerve palsy and ataxia both gradually improved. There has been no previous report of an adult case who developed peripheral facial nerve palsy during the recovery phase of acute cerebellitis. This case indicates that a wide spectrum of neurological complications may develop in association with a varicella-zoster virus infection.     

儿童后颅凹肿瘤术后缄默症

目的 探讨后颅凹肿瘤术后缄默症的发病机理,预后及预防。方法 回顾性分析我院小儿神经外科１９９６年至１９９９年收治的２００例后颅凹肿瘤,其中１２例出身术后缄默症。结果 １２例缄默症 ,全部为巨大型小脑蚓部凸入四脑室,并向双侧小脑半球浸润生长。术后当天不讲话６例,术后１天不讲话２例,术后２天不讲话２例,术后３天不讲话２例。持续时间１０～６１天,平均３２天。结论 儿童后颅凹小脑蚓部巨大型肿瘤全切术后易出     

Acute Cerebellitis with Cerebellar Swelling Successfully Treated with Standard Dexamethasone Treatment

Although cerebellitis is common in childhood but cerebellitis with cerebellar swelling is rarely reported. Pulsed high dose methylprednisolone treatment is the choice of treatment for cases who have non-progressive symptoms. An 8-year-old girl presented acutely with vertigo, headache, and vomiting. Brain magnetic resonance imaging showed marked bilateral cerebellar swelling with increased signal on T2-weighted imaging. Following treatment with standard dexamethasone dose, the clinical and radiological signs resolved in 1 week. We conclude that standard dexamethasone treatment should be used in mild cases of acute cerebellitis in order to avoid adverse reactions of pulsed high dose methylprednisolone treatment.