A case of neuro-Beh?et's encephalitis with pleds as distinct from herpes simplex encephalitis: a differential diagnosis

We report a male patient with neuro-Beh?et's disease who had somnolence, mild motor aphasia and periodic lateralized epileptiform discharge. SPECT study showed decreased cerebral blood flow in the left frontal and temporal lobes. An MRI scan of the head showed only mild brain stem atrophy. His clinical signs were markedly improved after administration of prednisolone. We propose that some cases of neuro-Beh,cet's disease may have very similar clinical features to herpes simplex encephalitis and that SPECT and MRI examinations can be very useful in distinguishing the ischemic lesion of neuro-Beh?et's from the inflammatory lesion of herpes simplex encephalitis.     

Herpetic simplex encephalitis followed by myelopathy

A 48-year-old male was admitted to our hospital because of fever, headache and vomiting. At admission, the level of consciousness was depressed (drowsy) with slight confusion. Extremely miotic pupils, nuchal stiffness, ataxia and myoclonic movements of both upper limbs were observed. The eye movements were almost normal and there was no definite limb weakness or sensory impairment. A few days after admission, his level of consciousness further decreased, and opsoclonus, ataxic breathing and intestinal paralysis appeared. The body temperature fluctuated remarkably ranging from 33.0 degrees C to 39.0 degrees C. The cerebrospinal fluid (CSF) examination revealed lymphocytic dominant pleocytosis, increase of protein and decrease of glucose. Enzyme-linked immunosorbent assay (ELISA) showed increased antibody (IgG) to herpes simplex virus (HSV) in both serum and CSF. The antibody in CSF further elevated at the later examination. Magnetic resonance imaging (MRI) demonstrated high signal intensity areas mainly in the cerebellum and sporadically in the supratentorial subcortical white matter on T2-weighted images. Administration of Gadolinium-DTPA also revealed an additional lesion in the pons. From these findings, he was diagnosed as herpetic encephalitis involving the brainstem and the cerebellum, and acyclovir was administered. Although his initial symptoms and signs started to recover three weeks after admission, he newly developed complete flaccid paraplegia, dysuria and sensory disturbance with the spinal cord level of the 4th thoracic segment. The oligoclonal IgG bands were detected in the cerebrospinal fluid of the convalescent stage.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of cortical reflex positive-negative myoclonus--electrophysiological study

An 11-year-old girl who had the positive-negative myoclonus and the history of the generalized tonic clonic seizure was electrophysiologically studied. She had no siblings with either myoclonus or epilepsy, and her intellectual level was normal. She had no other neurological deficits including ataxia, pyramidal and extrapyramidal signs. Surface EMG showed a brief increase in the EMG activity followed by the silent period associated with positive and negative myoclonus during sustained wrist extension. Giant SEP and C reflex (38.6 ms) following electric stimulation of the median nerve at the wrist were obtained in the resting condition and the silent period (about 180 ms) following C reflex was obtained during voluntary contraction. Jerk-locked back averaging of the EEG time-locked to the onset of the myoclonic discharge recorded from the right biceps muscle showed a cortical spike at the left central region preceding the myoclonus onset by 12.6 ms. The latency of C reflex in this case was very short compared with that of previously reported cortical reflex myoclonus. The estimated cortical delay between the arrival of the somatosensory volley and the motor cortex discharge responsible for the C reflex was -1.0 ms and this value was shorter than that in patients with typical cortical reflex myoclonus (mean 3.7 +/- 1.1 ms). Conditioning stimuli (C) of the right median nerve at the wrist started to facilitate the amplitude of the motor evoked potential recorded from the right abductor pollicis brevis muscle after magnetic test stimuli (T) of the left motor cortex at 20 ms of the C-T interval. This C-T interval was shorter than that (24.6 +/- 1.6 ms) in patients with the typical cortical myoclonus. These electrophysiological findings suggested the shorter reflex pathway of the cortical reflex myoclonus in this case than in typical cortical reflex myoclonus. We speculated that the myoclonus was based upon the direct sensory projection from the thalamus to the motor cortex in this case.     

Immunochemical detection of the proteoglycans decorin and biglycan in human gingival crevicular fluid from sites of advanced periodontitis

PURPOSE: To evaluate the utility of an assay based on a polymerase chain reaction (PCR) of cerebrospinal fluid in the management of patients with suspected herpes simplex encephalitis. METHODS: A decision model was constructed and used to compare a PCR-based approach with empiric therapy. Inputs required by the model included the sensitivity (96%) and specificity (99%) of PCR (derived from review of the literature), the prevalence of herpes simplex encephalitis (5%, based on the actual prevalence at Barnes Hospital among patients treated empirically with acyclovir), the outcomes for patients with and without herpes simplex encephalitis (derived from clinical studies of the Collaborative Antiviral Study Group and the actual experience at Barnes Hospital), and the average duration of empiric acyclovir therapy for patients with possible herpes simplex encephalitis (5.3 days based on actual experience at Barnes Hospital). RESULTS: Using these input values, the decision model predicted better outcomes with empiric therapy. However, low rates of inappropriate discontinuation of empiric therapy in patients with herpes simplex encephalitis or improved diagnosis and outcome resulting from a negative PCR assay result in patients without herpes simplex encephalitis led to better outcomes with the PCR-based approach. The PCR-based approach was associated with 9.2 fewer doses of acyclovir per patient. CONCLUSION: Based on the decision model using conservative assumptions, a PCR-based approach can yield better outcomes and reduced acyclovir use compared with empiric therapy.     

Production of a monoclonal antibody against the 128 kDa (CagA) protein of Helicobacter pylori

We report a case of herpes simplex encephalitis in which the patient was repeatedly examined by magnetic resonance imaging (MRI) and single photon emission computed tomography (SPECT). The patient was a 36 year-old woman who had been transferred to our institution 6 days after the onset of symptoms with mild consciousness disturbance, nuchal rigidity, and high fever. Cerebrospinal fluid examination revealed an elevated mononuclear cell count with normal sugar concentration. Intravenous aciclovir was started 7 days after the onset of symptoms. The initial plain computed tomography (CT) scans did not reveal any abnormal findings, but contrast enhanced CT the next day showed a slight enhancement effect around the affected middle cerebral artery. Serial MRI showed the initial high intensity lesion starting on the medial cortex of the temporal lobe, then spreading to throughout the entire temporal lobe. During this period SPECT showed a marked, broad hot spot in the temporal lobe. The medial temporal lobe was high density on the CT 15 days after the onset. As the encephalitic lesion spread more laterally, the hot spot on SPECT moved laterally and then decreased in activity. Eleven weeks after the onset, the MRI showed intracerebral vacuolization of the lesion and it appeared as a wide cold spot on SPECT. The cause of the hot spot seen in the acute period was thought to be vasoparalysis of the affected area rather than breakdown of the blood-brain barrier, or impaired washout of the isotope, because the SPECT images after acetazolamide administration showed the cold spot even in the subacute phase.     

Pathophysiologies of dystonia and myoclonus--consideration from the standpoint of treatment

Pathophysiologies of disorders with dystonia or myoclonus were studied by evaluating the effects of treatment. Naturally, the main lesion of the dystonia responding to levodopa is in the nigrostriatal dopamine neuron. The target of stereotaxic operations is ventrolateral palladium for postural dystonia and the nucleus ventralis oralis posterior (Vop) thalamus for action dystonia. Torsion dystonia with lesion in the striatum and/or the pallidum causes axial torsion, it may be postural through the descending pathway and action through Vop. Stereotaxic operations on these pathways have shown to be effective. Focal dystonia is a reflection of abnormal co-activation of cortical motor neurons, occurring in a particular voluntary movement. Botulinus toxin injected into the affected muscle should be effective. Of myoclonus with epilepsy, cortical reflex myoclonus or cortical induced reticular myoclonus responds to valproic acid. However, no antiepileptic drugs are effective on those with primary brainstem lesion. Reticular reflex myoclonus due to asphyxia responds to ventralis intermedius thalamotomy. Idiopathic myoclonus associated with dystonia is particular because it responds to ventrolateral thalamotomy. Myoclonus except for idiopathic myoclonus with dystonia is associated with atonic NREM suggesting dysfunction of the dorsal raphe serotonergic neuron or the brainstem nucleus reticularis gigantocellularis, the causative neuron for experimental uremic myoclonus. Treatment for these neurons is necessary.     

Overexpression of Glut1 and Glut3 in stage I nonsmall cell lung carcinoma is associated with poor survival

We report the electrophysiologic findings of myoclonus in a patient with Huntington's disease (HD). This patient was studied postoperatively after a bilateral fetal cell transplant in his striatum. Incomplete transient improvement was seen in the myoclonus, followed by gradual deterioration. The myoclonus itself had a cortical correlate and was associated with an enlarged somatosensory evoked potential (SEP), consistent with the presence of cortical reflex myoclonus. An enlarged SEP has not been previously reported in myoclonus associated with HD. The postulated mechanisms for myoclonus, when it occurs in HD, have differed in the literature. The reason for the transient improvement of the myoclonus following transplantation is unclear, but this case raises the possibility that basal ganglia circuits may modulate cortical myoclonic activity.     

Herpes simplex virus type 2 infections presenting as brainstem encephalitis and recurrent myelitis

We describe here 3 patients with central nervous system infection caused by herpes simplex virus type 2 (HSV-2); one patient with brainstem encephalitis and two with recurrent transverse thoracic myelitis. All three patients showed increased IgG antibodies to HSV in the cerebrospinal fluid (CSF). HSV-2 DNA was demonstrated in the CSF by polymerase chain reaction (PCR) amplification. Upon treatment with acyclovir, one patient with myelitis partially recovered and the others completely recovered. It is important to recognize the wide spectrum of clinical manifestations of HSV-2 infection in the central nervous system (CNS).     

Herpes simplex encephalitis: treatment with surgical decompression and cytosine arabinoside

Herpes simplex encephalitis in a 21-year-old man presented as a flu-like illness, followed by inappropriate behaviour, drowsiness and focal neurologic signs. Investigations indicated a lesion in the right temporal lobe. The diagnosis was confirmed by isolation of the virus from a cerebral biopsy. Pronounced clinical improvement was noted when cytosine arabinoside therapy was started in the postoperative period. This report supports the observation by some authors that cytosine arabinoside may be of value in the treatment of herpes simplex encephalitis.     

Psychosocial risk factors in near-fatal asthma and in asthma deaths

We report a patient with familial myoclonus showing an extremely benign clinical course. The patient was a 70-year-old woman, who first noticed shaking of hands at age of 25. The symptom did not worsen for more than 40 years. She visited our hospital at the age of 70 because of disturbance in chores because of worsening of her hand shaking in the past one year. A family history showed that 4 members had similar symptoms and that the two were afflicted with fits of loss of consciousness. On neurologic examination, rhythmic myoclonic jerks were noted in all the extremities, more in the upper limbs, both at rest and during action. Tandem gait was mildly disturbed. The remainings of neurologic examination were normal. SEP and jerk-locked back averaging provided evidence of cortical myoclonus. EEG showed multifocal polyspike discharges. Gene analysis for DRPLA, pyruvate and lactate levels in serum and the cerebrospinal fluid, serum amino acid levels, and CSF HVA and 5-HIAA levels were all normal. No brain atrophy was noted in cranial MRI. Myoclonus was markedly reduced after administration of clonazepam. The clinical features and electrophysiological data of our patient are consistent with the clinical diagnosis of familial essential myoclonus and epilepsy/benign adult familial myoclonic epilepsy.     

Adult-onset temporal lobe epilepsy associated with smoldering herpes simplex 2 infection

A 40-year-old man with chronic genital herpes simplex infection developed partial complex temporal lobe seizures of insidious onset, with EEG and MRI evidence of a unilateral temporal lobe destructive, atrophic process. Extensive workup did not reveal an infectious etiology. Three years of escalating number and severity of daily seizures with memory loss led to temporal lobectomy. Histologic study revealed active, low-level viral infection in the resected hippocampus and temporal lobe cortex, with immunohistochemical evidence for infection by herpes simplex 2, principally in neurons. In situ hybridization confirmed the presence of herpes simplex virus in neurons. Anticonvulsant-resistant seizure episodes began to recur several times daily soon after surgery, but the addition of acyclovir to the treatment regimen resulted in a substantial reduction in seizure occurrence, maintained for the subsequent 2.5 years.     

Early diagnosis of herpes simplex virus encephalitis by single photon emission computed tomography (SPECT) in patients with normal MRI

Since treatment of herpes simplex virus encephalitis (HSVE) is most effective when started early, a sensitive and specific method for early diagnosis would be of great benefit. MRI and CT are commonly used for this purpose. In this study, we presented two patients who had serologically confirmed HSVE and had normal CT and MRI, but were diagnosed as having HSVE by means of SPECT in the early stage. Case 1 was a 56-year-old man who suddenly developed alexia. On admission, physical and neurological examination were unremarkable except for alexia, agraphia, acalculia, and left-right disorientation. Brain CT, MRI, and cerebral angiography were all normal. However, SPECT showed hyperaccumulation of 99m Tc-HM-PAO in the right temporal-occipital area. On the 5th hospital day, he became comatose. CSF study revealed marked pleocytosis. Even then, MRI including Gd-enhanced study was normal while SPECT continued to show hyperaccumulation. Detection of herpes simplex virus DNA in CSF by polymerase chain reaction was negative. Anti-HSV antibody titer in CSF and serum confirmed intrathecal production of the antibody on the 14th hospital day. Abnormal accumulation of tracer in SPECT returned to normal on the 31st day when he was alert but still had a mild Gerstman syndrome. Case 2 was a 61-year-old man with disturbance of consciousness, mental dysfunction, and generalized convulsion. He was diagnosed as having HSVE by means of CSF pleocytosis, detection of HSV DNA in CSF by polymerase chain reaction, and presence of anti-HSV antibody in the CSF. CT and MRI again revealed no abnormality while SPECT clearly showed hyperaccumulation in the left temporal lobe in an early stage. Hyperaccumulation of lipophilic tracer on SPECT study, especially in the temporal lobes, has been reported in the early stage of HSVE by previous investigators. Unlike MRI or enhanced CT, the increased tracer accumulation in SPECT does not reflect disruption of the blood-brain-barrier or inflammatory edema, but reflects hyperperfusion or some other HSVE related abnormality which is currently unknown. From these observations, we suggest that local hyperperfusion occurs before local inflammation, and that SPECT is the most useful scanning method for early diagnosis of HSVE when this disease is clinically suspected.     

A case of herpes simplex encephalitis presenting complete destruction of the medial portions in the bilateral temporal lobes

A 57-year old man, who had suffered from herpes simplex encephalitis and had been cured without antiviral agents, was admitted to our hospital three years after the encephalitis. He had a complete loss of memory for events subsequent to the encephalitis and a retrograde amnesia for the several years leading up the encephalitis. MRI revealed the abnormalities located in the limbic system, especially the medial portions in the bilateral temporal lobes of the cerebrum were completely destroyed. We consider that this case suggests the specific affinity of herpes simplex virus especially for the medial portions in the temporal lobes of the cerebrum.     

Radiological diagnosis of viral encephalitis

Most of viral encephalitis may demonstrate no specific change on CT and MR images. Brain swelling, edema, abnormal density (CT) and abnormal intensity (MR) can be detected in herpes simplex encephalitis and enterovirus encephalitis (coxsackie, echo, polio). The common finding on CT and MRI in patients with HIV encephalopathy are atrophy, leukomalacia. Progressive multifocal leukoencephalopathy (PML) shows multifocal oval or round white matter T2-hyperintensities on MR images. Subacute sclerosing panencephalitis (SSPE) may present slight changes in the subcortical and periventricular white matter, as well as basal ganglia. Progressive disorder makes widespread T1-low, T2-high intensity area and atrophy. MRI of acute disseminated encephalomyelitis (ADEM) shows multifocal subcortical hyper intense foci on T2-weighted studies. The deep white matter, brainstem, thalamus and cerebellum can be affected. Most of ADEM lesions resolve. Imaging findings of acute lymphocytic meningitis by echovirus and coxsackievirus are usually normal.     

Herpes viruses--herpes simplex virus, varicella-zoster virus, EB virus, cytomegalovirus

Herpes simplex encephalitis is the commonest viral encephalitis among individuals, and the mortality has been markedly decreased by the use of vidarabine and acyclovir. Early diagnoses and immediate treatment are essential for favorable prognoses. Neuro-imagings, such as MRI and SPECT, and PCR technique for detection of HSV-DNA in CSF, are useful for early diagnoses, without requiring brain biopsy. Varicella and herpes zoster viruses are complicated, only rarely, with neurological manifestations, such as meningoencephalitis, myelitis, or peripheral neuropathy. Acyclovir is mostly effective in these cases. Neurological complications of Epstein-Barr virus infections are variable, including meningitis, cerebellar ataxia, cranial neuropathy, and Guillain-Barré syndrome. Their prognoses are generally good. Cytomegalovirus encephalitis is one of the common complications in AIDS patients. Its clinical diagnosis is difficult and the prognosis is considered to be poor.     

Familial cortical tremor, epilepsy, and mental retardation: a distinct clinical entity?

OBJECTIVE: To describe a European family with cortical tremor, epilepsy, and mental retardation, the pedigree of which indicates an autosomal dominant inheritance of the disease. DESIGN: Clinical, laboratory, neurophysiological, and neuroimaging data were studied. SETTING: Institute for research on mental retardation. PATIENTS: Two siblings (aged 25 and 28 years) and their 49-year-old mother had postural and action tremor, seizures, and mental retardation. Only tremor was present in the maternal grandmother (aged 68 years). The electroencephalogram showed diffuse spike-and-wave complexes and/or posterior spikes, and a photoparoxysmal response in the 4 subjects. The typical electrophysiologic features of cortical reflex myoclonus, such as giant somatosensory evoked potentials, enhancement of the C-reflex, and jerk-locked premyoclonus spikes, were found in all patients. CONCLUSION: This syndrome may represent a specific form of familial cortical tremor with a benign form of epilepsy and a new genetic model of cortical hyperexcitability inherited with an autosomal dominant mechanism.     

Prevalence of rheumatic fever in children from a public high school in Belo Horizonte

Genetic studies have suggested homogeneity between the Baltic-type and Mediterranean-type progressive myoclonic epilepsy. Magnetoencephalography was applied to elucidate the mechanism underlying the giant evoked responses in cortical reflex myoclonus. A new concept of negative myoclonus mediated by cortical reflex mechanism was proposed. Cortical myoclonus was demonstrated in various neurodegenerative or metabolic disorders, such as presenile or senile dementia, olivopontocerebellar atrophy, and myoclonus epilepsy associated with ragged-red fibres. Myoclonus in corticobasal degeneration is especially noteworthy because it has clinical and electrophysiological features of cortical reflex myoclonus, but its latency is shorter compared with the conventional cortical reflex myoclonus. Clinical features of 'palatal myoclonus' were reported by the name of 'palatal tremor'.     

Acyclovir given as prophylaxis against oral ulcers in acute myeloid leukaemia: randomised, double blind, placebo controlled trial

OBJECTIVES: To evaluate (a) the prophylactic effect of the antiherpetic drug acyclovir on oral ulcers in patients with acute myeloid leukaemia receiving remission induction chemotherapy and thus (b), indirectly, the role of herpes simplex virus in the aetiology of these ulcers. DESIGN: Randomised, double blind, placebo controlled trial. SUBJECTS: 74 herpes simplex virus seropositive patients aged 18-84. Thirty seven patients received acyclovir (800 mg by mouth daily) and 37 placebo. The patients were examined daily for 28 days. MAIN OUTCOME MEASURES: Occurrence of herpes labialis, intraoral ulcers, and acute necrotising ulcerative gingivitis. RESULTS: The two populations were comparable in age, sex, type of antineoplastic treatment, and history of herpes labialis. Acute oral infections occurred in 25 of the acyclovir treated patients and 36 of the placebo treated patients (relative risk 0.69 (95% confidence interval 0.55 to 0.87)). This difference was due to a reduction in the incidence of herpes labialis (one case versus eight cases; relative risk 0.13 (0.02 to 0.95)), intraoral ulcers excluding the soft palate (one case versus 13 cases; relative risk 0.08 (0.01 to 0.56)), and acute necrotising ulcerative gingivitis (one case versus eight cases; relative risk 0.13 (0.02 to 0.95)). However, ulcers on the soft palate were diagnosed with similar frequency in the two groups. Isolation of herpes simplex virus type 1 in saliva was reduced from 15 cases in the placebo group to one case in the acyclovir group (relative risk 0.07 (0.01 to 0.48)). CONCLUSION: Intraoral ulcers excluding the soft palate are most often due to infection with herpes simplex virus, whereas ulcers on the soft palate have a non-herpetic aetiology. The findings suggest that acute necrotising ulcerative gingivitis may also be due to herpes simplex virus. Prophylaxis with acyclovir should be considered for patients with acute myeloid leukaemia during remission induction therapy.     

Acyclovir-resistant chronic cutaneous herpes simplex in Wiskott-Aldrich syndrome

A 28-year-old man with Wiskott-Aldrich syndrome presented with ulcerative-proliferative lesions on his face from which herpes simplex type 1 (HSV-1) was isolated. He was initially treated with 10 mg/kg of acyclovir (Zovirax) intravenously every 8 h, but his skin lesions worsened. Clinical resistance to acyclovir was suspected, and therapy with this drug was intensified. The dosage of acyclovir was increased to 45 mg/kg, administered by continuous infusion, and the lesions subsequently resolved. The strain of HSV recovered from the patient showed acyclovir-resistance in vitro, using the colorimetric method with neutral red. Herpes simplex virus resistance to acyclovir is rare. It is more common in immunocompromised patients if subtherapeutic doses are administered in the treatment of chronic persistent forms of infection. Whenever clinical resistance to acyclovir is suspected, the dosage should be increased to 2 mg/kg per h administered via an infusion pump. If no improvement is observed in the patient's condition with this regimen, a phosphorylated medication whose mechanism of action is not dependent on viral thymidine kinase, such as foscarnet (phosphonoformic acid), should be substituted.     

Magnetic resonance imaging of herpes simplex virus encephalitis: reversible asymmetric basal ganglia lesions

We report a patient with herpes simplex virus type 1 encephalitis (HSE) who showed abnormal magnetic resonance imaging (MRI) signals in the basal ganglia. The lesions were asymmetric and became apparent with relapse of the neurological symptoms, but they completely disappeared, concomitantly with improvement of the illness.