Recurrent acute cerebellar ataxia of childhood following nonspecific respiratory tract infection

Acute cerebellar ataxia in childhood following viral infection is a self-limited disease. The disease with recurrent course has rarely been reported. At the Department of Pediatrics, Ramathibodi Hospital, three children with recurrent episodes of acute cerebellar ataxia following nonspecific viral infection were encountered. The age at onset of each patient was 2 years, 18 months and 2 years old. The clinical symptoms were similar and improved rapidly after gluco-corticoid was given. All patients recovered without residual deficit. Six, 5 and 3 recurrent attacks of similar illness were noted in each patient respectively after the first episode. However, no further attack occurred after the age of 5 years and the age of last follow-up was 17, 16 and 14 years old respectively. The pathogenesis of the recurrent episodes is uncertain. The abnormal immunological response is postulated.     

Systemic candidiasis with acute Epstein-Barr virus infection

The binding of a classical cannabinoid agonist, [3H]R-(+)-(2,3-dihydro-5-methyl-3-[(4-morpholinyl)methyl]pyrol[1,2 ,3-de]-1,4-benzoxazin-6-yl)(1-napthalenyl)methanone monomethanesulfonate ([3H] WIN55212-2), and a selective cannabinoid receptor (CB1) antagonist, N-(piperidin-1-yl)-5-(4-chlorophenyl)1-(2,4-dichlorophenyl)-4-meth yl-1H-pyrazole-3-carboxamide hydrochloride ([3H]SR141716A), to rat cannabinoid receptors was evaluated using rat cerebellar membranes. Guanine nucleotides inhibited [3H]WIN55212-2 binding by approximately 50% at 10 microM and enhanced [3H]SR141716A binding very slightly. In the same tissue, the binding of guanosine 5'-O-[gamma-[35S]thio]triphosphate ([35S]GTP-gamma-S) was characterized and the influence of cannabinomimetics evaluated on this binding. Cannabinoid receptor agonists enhanced [35S]GTP-gamma-S binding, whereas SR141716A was devoid of action by itself but antagonized the action of cannabinoid receptor agonists. The good correlation obtained between the half maximum efficient concentration (EC50) values in [35S]GTP-gamma-S binding and the IC50 values [3H]WIN55212-2 binding shows that [35S]GTP-gamma-S binding could be a good functional assay for brain cannabinoid receptors.     

Anti-interleukin-10 antibodies in patients with chronic active Epstein-Barr virus infection

The Epstein-Barr virus (EBV) genome encodes a protein in its BamHI C restriction fragment rightward open-reading frame-1 (designated BCRF1 or viral interleukin-10 [vIL-10]) that shares protein homology and biologic properties with human IL-10. Several EBV disorders are characterized by prolonged active EBV infection. Because continued EBV replication could allow for increased vIL-10, ELISA and immunoprecipitation were used to determine whether vIL-10 expression during chronic active EBV infection resulted in vIL-10 and IL-10 antibodies. IL-10 antibodies were assayed in patients diagnosed with chronic and acute infectious mononucleosis (CIM, AIM), nasopharyngeal carcinoma (NPC), and EBV-associated lymphoproliferative disease (LPD), as well as from healthy organ transplant patients and EBV-negative or EBV-positive persons. Whether anti-IL-10 antibodies could inhibit IL-10 biologic activity was determined. vIL-10 antibodies were found in CIM, NPC, and LPD patients and antibodies reactive to IL-10 were found in CIM patients. One CIM patient had IL-10 antibodies that neutralized IL-10 bioactivity in vitro.     

Acute cerebellar ataxia after subclinical hepatitis A infection

Infection is a well-recognized triggering factor for both guttate and chronic plaque psoriasis. We investigated prospectively 13 patients with recalcitrant psoriasis exacerbated by recurrent tonsillitis, who underwent tonsillectomy between 1990 and 1993. There were 12 female patients and one male, with a mean age of 17 yr (range 6-28). Six patients had guttate psoriasis resistant to standard treatments and seven patients had chronic plaque psoriasis exacerbated by tonsillitis that was severe enough to warrant at least one admission to hospital. Patients were followed by chart review and postal questionnaire. Psoriasis was cleared completely after tonsillectomy in five out of the six patients (83%) with guttate psoriasis and was improved in one patient. Two out of seven patients with plaque psoriasis (29%) were cleared, two (29%) were improved and three (42%) were unchanged. We conclude that tonsillectomy may be a successful treatment modality in selected patients with recalcitrant guttate or chronic plaque psoriasis.     

Fulminant cerebello-brainstem encephalitis with polyradiculitis following probable Epstein-Barr virus infection]

Epstein-Barr virus (EBV) infection is occasionally accompanied by acute neurological impairment. The pathogenesis of neurological manifestations with EBV infection consists of primary inflammations of EBV infection, and secondary immunologic reactions. However, their clinical course and prognosis are usually favorable. Here we report a patient with fulminant neurological involvement in association with EBV infection. The patient was a 44-year-old man. One morning he developed ataxic gait and speech following flu-like symptoms. He noticed double vision in the afternoon. He had disturbance of consciousness, bilateral ptosis with mydriasis, opthalmoplegia, facial diplegia, bulbar palsy, and weakness of muscles in extremities and respiratory system on the next day. He required mechanical ventilatory support for a month. His symptoms began to improve gradually two weeks after the onset. Two month later, neurological examinations disclosed severe cerebellar ataxia of the four extremities and ocular movement, cerebellar speech, and moderate weakness in his limbs. Moderate cerebellar ataxia and diminished deep tendon reflexes remained for 8-months. Although he had no physical manifestations of infectious mononucleosis, DNA of EBV was identified in the cerebrospinal fluid (CSF) by the polymerase chain reaction method. From these results, we diagnosed his condition as a cerebello brainstem encephalitis with polyradiculitis associated with EBV infection. The cell counts and protein content of CSF gradually normalized in the early stage of his illness, but CSF protein increased again, and had the peak of 275 mg/dl in about one month. In spite of normalized CSF cell counts, his neurological symptoms persisted. CT scan and MRI studies of the brain and the spinal cord were repeated, but demonstrated no significant abnormalities. Clinical course and CSF findings revealed that his fulminant neurological symptoms were most likely produced by the secondary immunologic reactions following the primary inflammations by EBV infection.     

Epstein-Barr virus infection in Desert Storm reservists

Approximately 150 U.S. Army reservists from Indiana reported symptoms consistent with chronic fatigue syndrome after returning stateside from the tour of duty in Saudi Arabia. A psychiatric team confirmed the diagnosis, evaluated possible etiology, and treated the service members when appropriate. Those available service members who met the study's diagnostic criteria for chronic fatigue syndrome (n = 37) received an Epstein-Barr virus panel. Seventy-three percent of these selected service members were positive either for an acute or reactivated Epstein-Barr viral infection. These data suggest that service members who suffer from chronic fatigue syndrome may have their symptoms increased and prolonged by secondary viral infections.     

Method of diagnosis of Epstein-Barr virus infection

The CO2 laser can be used for the treatment of various lesions of the eyelids and peri-orbital region with the advantage over other modalities of precise, controllable, hemostatic, non contact atraumatic ablation with the reduction of post-operative pain and improved cosmetic results. Its application in palpabral and peri-orbital surgery is recommended.     

The diagnosis and follow-up evaluation of acute cerebellar ataxia supported by a cerebellar stimulation study]

A 70-year-old woman who has been suffering from diabetes mellitus since 67 years of age rapidly developed severe truncal ataxia. Neurological examination showed severe truncal ataxia, incoordination and decreased deep sensations in the bilateral lower extremities. A CSF study revealed a moderately elevated total protein (125 mg/dl) without any elevation of the cell count. A nerve conduction study supported the diagnosis of polyneuropathy. Lumbar MRI revealed spinal canal stenosis at the L3/L4-L5/S1 intervertebral levels due to disk herniations and ossification of the yellow ligaments. We examined cerebellar stimulation in order to determine whether the ataxia was due to dysfunction of the cerebellum or peripheral nervous system. Conditioning electrical stimulation over the cerebellum did not change the size of motor potentials evoked by magnetic cortical stimulation in the right first dorsal interosseous muscle. Her clinical course was good, and the limb and truncal ataxia became very mild about 4 months after the onset, although there was little change in the decreased deep sensations. The cerebellar stimulation in the second study was normal. We diagnosed her as having acute cerebellar ataxia and thought that the decreased deep sensations were due to diabetic polyneuropathy and lumbosacral radiculopathies. A cerebellar stimulation study was useful for the diagnosis and follow-up evaluation of acute cerebellar ataxia in this patient.     

Spontaneous retropharyngeal haematoma attributable to Epstein-Barr virus infection

Life-threatening sequelae of Epstein-Barr virus infection are uncommon but may present as: local pharyngeal manifestations, splenic rupture, neurological and haematological disorders and altered hepatic function. We present a case of retropharyngeal haematoma with posterior hypopharyngeal wall necrosis, thrombocytopenia and altered clotting function as a result of Epstein-Barr virus infection. A review of the literature on retropharyngeal haematoma reveals this to be the only recorded case which can be directly attributed to Epstein-Barr virus infection.     

Associative learning in patients with cerebellar ataxia

It has been claimed that patients with cerebellar pathology are impaired at associative learning. Patients with cerebellar ataxia (n = 7) were taught a visual-motor associative task. The task was chosen so as to allow comparisons with data currently being collected on the effects of cerebellar lesions on associative learning in monkeys. As a group the patients were as impaired at learning the task as a group of 8 patients with Huntington's disease. When each patient was individually matched with a control of the same age and IQ, some patients with cerebellar ataxia were found to be clearly impaired, but 2 were not. Of the 4 patients who were most clearly impaired, 2 had brainstem pathology and 2 did not. The relevance of these findings is discussed in relation to views concerning the functions of the cerebellum.     

Immunoreactivation of Epstein-Barr virus due to cytomegalovirus primary infection

Interleukin (IL)-6 plays a major role in the control of the survival and proliferation of myeloma cell lines and primary myeloma cells. The genes of the receptors of IL-6 have been cloned, and the major signaling pathways involved in gp130 IL-6 transducer activation have been identified. In addition, another five cytokines that activate the gp130 IL-6 transducer have been identified. We review the recent data on gp130 cytokines and gp130-mediated signal transduction, their involvement in myeloma cell biology, and we discuss the possible therapeutic applications of this knowledge.     

Graft-induced restoration of function in hereditary cerebellar ataxia

Fetal cerebellar cell suspensions, prepared from wild-type (+/+) mice, were implanted bilaterally into the cerebellum of Purkinje cell degeneration' (pcd) mutant mice, a model of adult-onset recessively inherited cerebellar ataxia, to study the functional effects of the grafts on motor coordination and fatigue resistance in a rotating rod treadmill paradigm. The viability of transplanted Purkinje cells was verified with immunocytochemistry for calbindin-D28k and for glutamate receptor 2/3 subunits and with in situ hybridisation histochemistry for insulin-like growth factor I mRNA, biochemical markers normally expressed by Purkinje cells in the cerebellum. Sham injections of vehicle did not appreciably modify the performance of pcd mutants in the rota-rod tests. On the other hand, bilateral cerebellar grafts led to a 3.5-fold increase in the time period that recipient pcd mice were able to stay on the rotating drum based on the comparison of mean scores (of three trials) or a 5.5-fold increase based on the comparison of maximum scores (of the three trials). These findings provide evidence for a motor enhancement in the pcd mouse model of hereditary cerebellar ataxia following intracerebellar transplantation of primordial Purkinje cells.     

Calcification in the basal ganglia with chronic active Epstein-Barr virus infection

The objective of this study was to determine whether measurements of human CRH in the inferior petrosal sinuses could distinguish patients with Cushing's syndrome from those with pseudo-Cushing states or normal physiology. Twenty-five patients with Cushing's disease, 17 patients with the syndrome of ectopic ACTH, 7 patients with Cushing's syndrome of adrenal origin, 6 patients with pseudo-Cushing states, and 11 volunteers believed to have normal hypothalamic-pituitary-adrenal axes were studied. Basal plasma human CRH and ACTH were measured at two time points in the petrosal sinuses and in a peripheral vein. Most subjects were studied after the administration of intravenous diazepam or midazolam and fentanyl, but because of the known inhibitory effects of such sedation on CRH secretion, 2 normal volunteers and 3 patients with pseudo-Cushing states were studied without sedation. Human CRH levels were near or below the detection limit of the assay in all subjects. Although the normal volunteers and patients with pseudo-Cushing states who were studied without sedation had significantly greater inferior petrosal sinus ACTH levels than those who received sedation, there were no differences in measured human CRH levels for any of the groups. We conclude that inferior petrosal sinus human CRH levels are not easily measured in the inferior petrosal sinuses and cannot be used to determine whether individual patients may have hypersecretion of CRH causing their ACTH secretion.