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Abstract. Cerebellar disorders due to herpes simplex virus (HSV) infection are rare and always associated with herpes simplex encephalitis. We report 2 cases . This article includes discussion of acute cerebellar ataxia (in children), acute cerebellitis, and acute postinfectious cerebellitis. The foregoing. Acute cerebellar ataxia is a syndrome that occurs in previously well children, often presenting Acute cerebellar ataxia, acute cerebellitis, and.

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Cerebellar disorders due to herpes simplex virus HSV infection are rare and always associated with herpes simplex encephalitis. We report 2 cases of severe primary acute cerebellitis caused by HSV type 1 that were identified by nested polymerase chain reaction performed on cerebrospinal fluid samples.

Herpes simplex virus type 1 HSV-1 is one of the most common causes of sporadic viral cwrebellitis in adults. Herpes simplex encephalitis HSE may be a consequence of both primary and recurrent HSV infections, and it is characterized by focal necrosis of the medial temporal and inferior frontal lobes and, occasionally, of the insular cortex and cingulate gyrus [ 12 ].

Acute cerebellitis in children: an eleven year retrospective multicentric study in Italy

Such lesions are unusual in other brain areas, and, to our knowledge, isolated cerebellar involvement has never been reported. Patient 1, a accute year-old woman who had experienced headache, dysarthria, ataxia, vomiting, and moderate fever for 7 days, was referred to our hospital because of a gradual worsening of her symptoms. At admission, neurologic examination revealed normal cognition, ataxia, dysarthria with exacerbation of her dysphemia, dysmetria on the entire left side of the body, and nystagmus.

Reflexes were brisk, and there was acuhe evidence of meningeal signs. Results of liver and renal function tests were within acuge ranges. Neurological evaluation included a head CT scan and electroencephalography, both of which revealed normal findings.

An MRI of the brain revealed a bilateral cerebellar enhancement on T1-weighted images after gadolinium administration figure 1A and diffuse, increased signal intensity in the cerebellar cortex on fluid-attenuated inversion recovery images, suggesting an acure process confined to the cerebellum figure 1B. Furthermore, the cerebellar cortex appeared swollen, a finding consistent with diffuse cerebellitis.

A and BMRIs obtained at admission to the hospital. AT1-Weighted image revealing marked cerebellar enhancement after gadolinium administration. BFluid-attenuated inversion recovery FLAIR image showing increased signal intensity of bihemispheral cerebellar cortex with swelling. C and DMRIs obtained 1 month later. CT1-weighted image revealing complete disappearance of the cerebellar enhancement after gadolinium.

Reaction conditions for first- and second-round PCR analyses were as described elsewhere [ 4 cerebelliitis.

Acute cerebellitis in adults: a case report and review of the literature

To avoid false-positive results, all reactions were performed under stringent conditions, DNA-free control was included, and all samples acte tested at least twice in separate PCR analyses [ 56 ]. A few days after the acut of treatment, neurological symptoms gradually improved, and they disappeared entirely within 2 weeks.

PCR analysis of a second CSF sample obtained 14 days cerebellitid the initiation of treatment was negative for HSV-1 DNA; an MRI performed 1 month after the onset of acute cerebellitis revealed complete disappearance of the bihemispheral enhancement of the cerebellar cortex figure 1C and 1D.

Patient 2, a year-old girl, received a diagnosis of HIV-1 infection in HAART was started in and the virological and immunological response was excellent. In Februarythe patient presented with a moderate fever, dysarthria, severe headache associated with photophobia, and nausea.


At admission to the hospital, cognition was intact, but there was mild dysarthria. Reflexes were brisk, findings of cranial nerve examination were normal, and no evidence of meningeal signs was found.

Electroencephalography revealed nothing abnormal, and a brain MRI revealed a partial bihemispheral cerebellar enhancement after gadolinium administration cerevellitis T1-weighted images and an increased signal intensity with a mild cerebellar swelling on fluid-attenuated inversion recovery images figure 2A and 2B. Although total length of treatment lasted 21 cwrebellitis, the patient experienced a clinically complete recovery 2 weeks after the initiation of treatment.

AT1-Weighted image revealing partial cerebellar enhancement after gadolinium administration.

BFluid-attenuated inversion recovery FLAIR image showing increased signal intensity of bihemispheral cerebellar cortex with mild swelling. CT1-Weighted image revealing complete disappearance of the cerebellar enhancement after cerebelltiis.

Post viral cerebellar ataxia

Acute cerebellitis is a neurological condition characterized by mild or high-grade fever, nystagmus, tremor, truncal ataxia, dysarthria, headache, and altered mental state [ 7 ]; on the other hand, acute cerebellar ataxia is defined as the acute onset of gait ataxia without fever, prominent meningismus, seizures, or a significant alteration of mental state [ 89 ].

Acute cerebellar inflammation may occur during several illnesses, including measles, mumps, pertussis, scarlet fever, diphtheria, and typhoid fever, as well as during infections due to coxsackievirus, poliovirus, echovirus, rotavirus, varicella-zoster virus, and Epstein-Barr virus [ 12—15 ]. Coxiella burnetii and Borrelia burgdorferi have also been reported to cause acute cerebellitis [ 16—18 ], and a syndrome of unexplained cerebellar degeneration associated with HIV infection has also been described [ 19 ].

Acute cerebellitis was diagnosed in our patients on the basis of clinical symptoms and laboratory findings, including high-grade fever, severe headache, and mild CSF pleocytosis. MRI of the brains of the patients revealed increased signal intensity without focal lesions of the cerebellar cortex, which appeared to be swollen.

The clinical courses and the MRI findings were similar for our patients but quite different from those described in cases of acute cerebellitis due to other causes, in which foci of edema or demyelination in cerebellar white matter were observed [ 72425 ]. Our patients experienced rapid clinical improvement and complete recovery after 2 weeks of treatment; HSV-1 DNA was undetectable in CSF samples obtained after 14 days of treatment, which is not surprising considering that HSV-1 DNA can disappear from CSF as early as 1 week after the beginning of specific antiviral therapy [ 36 ].

One month after the initiation of treatment, MRI findings demonstrated a marked decrease of the diffuse hyperintense signal in both patients. Although involvement of the cerebellum has been reported to occur during the course of HSE [ 20—23 ], we are not aware of any other patient who has manifested HSV-1 acute cerebellitis. Therefore, this is the first report of a definite diagnosis of acute cerebellitis due to HSV. Our findings suggest that primary cerebellar involvement may occur during the course of HSV-1 infection.

MRI findings associated with positive PCR results, obtained as early as 1 day after admission to the hospital, allowed us to establish a definite diagnosis and immediately to administer specific treatment.

In contrast to virus isolation, which is less sensitive and more time-consuming, PCR assay proved to be a reliable and sensitive tool for the early diagnosis of HSV-caused neurological disease [ 1312 ]. Although the clinical diagnosis of most cases of HSV-caused neurological disease is eased by the presence of clinical symptoms and brain lesions such as focal necrosisPCR for HSV DNA detection in CSF specimens is especially valuable in those cases in which atypical clinical and neuroradiological findings are observed.


Moreover, the feasibility of an early definite diagnosis by PCR analysis could help investigators determine whether there is a trend toward the reduced severity of HSV-related neurological alterations, with less severe forms of the diseases and better outcomes [ 26 ]. It has been argued that isolated cerebellar abnormalities that occur in patients with HIV infection may be caused by other diseases for which the patients were not tested, rather than by HIV infection [ 27 ].

Alternative diagnoses must be excluded early for these patients, including etiologic association with other viral infections, such as HSV infection. In conclusion, we believe that HSV infection may be partly responsible for some of the undiagnosed cases of acute cerebellitis and therefore should be considered as a possible etiological agent of this condition. Oxford University Press is a department of the University of Oxford.

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Abstract Cerebellar disorders due to herpes simplex virus HSV infection are rare and always associated with herpes simplex encephalitis.

View large Download slide. Rapid diagnosis of herpes simplex encephalitis by nested polymerase chain reaction assay of cerebrospinal fluid. Detection of herpesvirus DNA in the large intestine of patients with ulcerative colitis and Crohn’s disease using the nested polymerase chain reaction.

Evaluation of the polymerase chain reaction for diagnosis of herpes simplex virus encephalitis. Detection of varicella-zoster virus DNA by polymerase chain reaction in cerebrospinal fluid of patient suffering neurological complications associated with chicken pox or herpes zoster. Cerebellar degeneration associated with human immunodeficiency virus infection. Early cerebellar involvement on diffusion-weighted magnetic resonance images in herpes simplex encephalitis.

A lesion simulating a cerebellar infarct on CT in a child with herpes simplex encephalitis. Evaluation of the range of clinical presentations of herpes simplex encephalitis by using polymerase chain reaction assay of cerebrospinal fluid samples.

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