1/18. The value of serial auditory brainstem response in patients with subacute sclerosing panencephalitis.A total of 98 serial auditory brainstem responses from 17 patients (11 boys and 6 girls) with subacute sclerosing panencephalitis were compared with their clinical course and stages. These patients were exposed to measles early in life (at 1.8 /- 1.4 years old) with the average onset of the disease at 8.8 /- 0.7 years of age. The main abnormalities of their auditory brainstem responses were a prolongation of waves I, III, and V, and of the I to V interpeak interval. Wave V thresholds were also elevated. These effects on the auditory brainstem responses started 1 to 2 years after onset of neurologic signs. I to V interpeak latency became prolonged with the progress of clinical stages, especially in Jabbour's clinically advanced stage IV. In two patients with an acute progressive type of subacute sclerosing panencephalitis, very rapid deterioration with a distorted wave pattern of later components was observed. These findings suggest rostrocaudal progression in the central nervous system of both long-term and fulminant cases.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/18. Acute multifocal placoid pigment epitheliopathy-like lesion as an early presentation of subacute sclerosing panencephalitis.PURPOSE: To report a case of acute multifocal placoid pigment epitheliopathy (AMPPE)-like lesion as an early presentation of subacute sclerosing panencephalitis (SSPE). DESIGN: Observational case report. methods: A 21-year-old woman presented with a profound loss of vision in the left eye. The initial clinical picture was characterized by a whitish infiltration of the macular area and papillary edema in the left eye. Six days later multiple chorioretinal infiltrations were present in the peripheral fundus of the left eye, which led to the diagnosis of AMPPE. RESULTS: During the following year, a gradual change in her personality developed. The diagnosis of SSPE was made by cerebrospinal fluid examination. Her clinical condition deteriorated rapidly, and she died. CONCLUSIONS: AMPPE can be accompanied by central nervous system involvement. We wish to emphasize the importance of considering the diagnosis of SSPE in cases of AMPPE.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/18. subacute sclerosing panencephalitis presenting with hemiparesis in childhood: case report.subacute sclerosing panencephalitis is a chronic and fatal disease of the central nervous system. Most patients present with progressive psychointellectual disturbances. A 14-month-old girl was admitted to our hospital because of left-sided hemiparesis. During hospitalization, focal and generalized seizures occurred. The electroencephalogram (EEG) revealed that periodic lateralized discharges consisted of polyspike and high-voltage slow waves in the left hemisphere. The clinical and EEG findings and positive serology of measles in the cerebrospinal fluid were consistent with subacute sclerosing panencephalitis. In conclusion, we suggest that subacute sclerosing panencephalitis be considered in the differential diagnosis of focal neurologic signs in infants.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/18. Combination therapy with intraventricular interferon-alpha and ribavirin for subacute sclerosing panencephalitis and monitoring measles virus rna by quantitative PCR assay.subacute sclerosing panencephalitis (SSPE) is a degenerative disease of the central nervous system that leads to death within a few years. Recently, it has been reported that combination therapy with intraparenchymal interferon-alpha (INF-alpha) and intraventricular ribavirin is effective. An 11-year-old SSPE patient whose clinical symptoms progressed rapidly, was treated first with intraventricular INF-alpha and then with combined intraventricular INF-alpha and ribavirin therapy. To monitor viral load over the course of the therapy, measles virus rna was quantified using a real-time polymerase chain reaction assay. measles virus rna decreased rapidly after the INF-alpha therapy was started, paralleling the decrease in the measles antibody titer in the cerebrospinal fluid and the improvement in the neurological disability. After intraventricular ribavirin was combined with INF-alpha therapy, no further improvement was observed. The neurological disability gradually progressed, although the amount of virus rna remained low.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/18. Fulminating adult-onset subacute sclerosing panencephalitis in a 49-year-old man.CONTEXT: subacute sclerosing panencephalitis (SSPE) is a rare, slow viral infection caused by a defective measles virus. It is characterized by progressive mental deterioration associated with motor impairment and prominent myoclonus. In about 10% of all cases, the disease can progress rapidly and lead to death within a few months. The oldest previously reported fulminating case was in a 25-year-old man. OBJECTIVE: To emphasize the relationship between retinal involvement and acute SSPE by reporting the case of a 49-year-old man with clinical, laboratory, and pathological evidence of acute SSPE. SETTING: Hopital de l'Enfant-Jesus, quebec, quebec. REPORT OF A CASE: This man was referred to the Department of Neurological Sciences on March 21, 2001, because of recent behavioral changes and progressive cognitive impairment over the past few months. Medical history was unremarkable except for an episode of measles in his childhood. Neurological examination showed bilateral myoclonic jerks. Ophthalmic examination revealed bilateral macular swelling and papilledema. electroencephalography showed periodic sharp and slow-wave discharges. magnetic resonance imaging showed bilateral diffuse T2-signal hyperintensities in both periventricular and subcortical white matter. cerebrospinal fluid antimeasles antibody titers were highly positive. An Omaya reservoir was inserted and therapy using a combination of high-dose intrathecal interferon alfa and oral isoprinosine were administered for 6 weeks. Despite transient subjective improvement in the patient's condition, it continued to deteriorate, he became bedridden, and he died on June 26, 2001. CONCLUSION: To our knowledge, this patient is the oldest case of SSPE reported in the literature. This patient and other patients with acute SSPE associated with bilateral macular swelling described in the literature raised the possibility of measles virus-acquired virulent neurotropism in the retina before invading the central nervous system.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/18. Progressive rubella panencephalitis. Follow-up EEG study of a case.Progressive rubella panencephalitis is a very rare slow virus disease of the nervous system. The authors present a case, concerning a young man, aged 20 years, died 11 months after the onset of the disease. The following peculiarities of the case are emphasized: 1) the clinical symptomatology and the evolution (myoclonus, lack of cerebellar impairment) could suggest the diagnosis of SSPE; 2) the EEG recordings showed epileptiform abnormalities, long latency diffuse periodic complexes and--during interferon therapy and simultaneously with a temporary clinical improvement--the appearance of short latency anterior periodic complexes.- - - - - - - - - - ranking = 0.26567254872451keywords = nervous system (Clic here for more details about this article) |
7/18. subacute sclerosing panencephalitis in two brothers.We report the occurrence of subacute sclerosing panencephalitis (SSPE) in two brothers two years after measles infection. The diagnosis was confirmed by compatible data from medical history, occurrence of autochthonic measles virus (MV) IgG production in the central nervous system (CNS), and pathognomonic EEG changes. Pathogenetically, SSPE is caused by a genome mutation of intracellularly persisting MV, causing viral nucleocapsides to accumulate in the brain cells. A specific predisposing immune defect is not known. The occurrence of two cases in one family is suggestive of a genetic predisposing factor.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/18. subacute sclerosing panencephalitis: a cause of acute vision loss.A 4-year-old male presented with only acute vision loss. His neurologic examination, funduscopic examination, and pupils were normal. Cranial magnetic resonance imaging revealed abnormal hyperintense, bilaterally symmetric lesions (on T(2)-weighted and fluid-attenuated inversion recovery, images) in bilateral optic radiations, pulvinar region in the thalami, crus posterior of internal capsules, periventricular white matter, and unilaterally left anterior pons. Elevated measles antibody titers in the cerebrospinal fluid confirmed the diagnosis of subacute sclerosing panencephalitis. Vision loss improved and cranial magnetic resonance imaging findings regressed,but myoclonic jerks and deterioration began 7 months later. The diagnosis of subacute sclerosing panencephalitis should be considered in cases with acute vision loss resulting from cortical blindness even when classical findings of the central nervous system do not exist.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/18. role of CSF serology in follow-up of subacute sclerosing panencephalitis patients on treatment.subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disease of the central nervous system with poor prognosis and high mortality. No effective treatment has a proven role; oral isoprinosine and intrathecal administration of alpha-interferon may prolong survival. We report an unusual case of adult onset SSPE patient on treatment with significant clinical improvement, even in the absence of conversion to seronegativity in either CSF or serum, on follow-up serological examination.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/18. Coincidental fluctuations of humoral immunity and clinical progression in a patient with subacute sclerosing panencephalitis.serum and cerebrospinal fluid were obtained from a 6-year-old male with subacute sclerosing panencephalitis (SSPE). Specimens were collected over a 9-month period beginning in the unusually acute phase and ending in a more quiescent phase of the disease. Immune complexes, auto-antibodies and viral antibodies were measured by radio-immunoassays. Fluctuations in these humoral immune parameters coincided with cessation of the acute phase of this disease. The results show that neurological changes in SSPE patients can be reflected in immune responses within both the peripheral circulation and the central nervous system.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
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