ABSTRACT
Subacute Sclerosing Panencephalitis (SSPE) is a rare, progressive degenerative disease of the brain caused by reactivation of aberrant measles virus. Classical symptoms of SSPE are described as behavioral changes, declining scholastic performance, myoclonic jerks etc. However, at times it presents with varied atypical manifestations, which makes diagnosis dif?cult. Hereby we report two patients with unusual manifestations of SSPE. In ?rst case, 14-year-old boy presented with recurrent falls of 9 months duration. Initially, it was 1-2 times per week, progressing over 9 months to frequency of 40-50 falls per day. In second case, 21-year-old gentleman presented with behavioral changes and memory disturbance of 1-year duration. However, his family members noticed worsening of symptoms 1 month before presentation as he started developing involuntary jerky movements involving the right upper and lower limb. In both of them electroencephalogram (EEG) was showing long interval periodic discharges and cerebrospinal ?uid was showing elevated titers of anti-measles antibody overall suggesting SSPE. Hence it needs much attention for atypical presentations and also considering SSPE in differential diagnosis of unsolved atypical neurological presentations.
ABSTRACT
@#Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal disease of the central nervous system caused by a persistent measles virus in the brain. It is clinically characterized by insidious onset of intellectual deterioration and behavioral changes followed by myoclonic jerks and eventually complete neurologic deterioration. In about 10% of patients, the clinical manifestations of SSPE are not typical and that may cause a delay in the diagnosis. We report here 3 cases of SSPE with atypical presentations. Bilateral vision loss, hemiparesis, ataxia and acute encephalopathy with focal seizures were respectively the presenting symptoms at the onset of disease. The typical periodic EEG complexes in two patients and positive CSF measles IgG antibody led to the diagnosis of SSPE.
ABSTRACT
An 11-month-old boy presented with focal seizures, myoclonic jerks and altered sensorium of one month duration, with a history of measles at eight months of age. A diagnosis of Subacute sclerosing panencephalitis (SSPE) was made on the basis of typical EEG changes and presence of anti-measles antibody in cerebrospinal fluid. A differential diagnosis of SSPE should be considered in all forms of acute encephalopathy in infants for early diagnosis and management.
ABSTRACT
A 12-yr-old boy with an atypical presentation of subacute sclerosing panencephalitis (SSPE) is described. Bilateral macular chorioretinitis preceded the neurological symptoms by 3 weeks. Both visual and neurological features had an acute onset. Clinicians need to be aware that macular chorioretinitis in a child may be the heralding feature of SSPE.
Subject(s)
Acute Disease , Child , Chorioretinitis/diagnosis , Chorioretinitis/etiology , Diagnosis, Differential , Disease Progression , Humans , Male , Subacute Sclerosing Panencephalitis/complications , Subacute Sclerosing Panencephalitis/diagnosisABSTRACT
This study was conducted to observe the impact of measles vaccination on the epidemiology of subacute sclerosing panencephalitis (SSPE) in the post measles vaccination era. This is a retrospective study from a tertiary care hospital, covering a ten year period starting a decade after the introduction of the national measles immunization programme in India. We analyzed 458 serologically confirmed SSPE cases. These patients had a high cerebrospinal fluid: serum anti-measles antibody ratio. The male to female ratio in the present study was 4.4:1. The mean age at onset of SSPE was 13.3 years, showing an increase in mean age at onset of SSPE. Clinical and other demographic details, available from 72 in-patients, are discussed in this report. Of these, a history of measles could be elicited in 34 cases. Mean latent period between measles infection and onset of SSPE was 7.8 years. Six patients gave a history of measles vaccination. A sizable percentage (15.5 %) of the patients was ≥ 18 years old and considered to have adult onset SSPE. The incidence of SSPE continues to be high and this report highlights the need for further strengthening routine measles immunization coverage.
ABSTRACT
The Bacillus cereus group includes B. anthracis, B. cereus, B. thuringiensis, B. mycoides, B. weihenstephanensis, B. pseudomycoides. The members of B. cereus group shares strong degree of DNA sequence similarity. Even though the biochemical test and bacteriological test have been used to identify the B. cereus group, an accurate identification system of the B. cereus group is required. We have developed a highly specific PCR-based assay for the B. cereus group chromosome using a sequence motif found within a spore structural gene (sspE). Using the assay, we were able to discriminate B. anthracis from the other members of B. cereus group. We also tried to find a new system for the B. cereus group identification. Five bacteriological tests (hemolysis, motility, penicillin susceptibility, rhizoid growth, toxic crystal formation), API system (API 50CHB & API 20E), MLST and sspE PCR were performed on 28 strains of the B. cereus group. The dendrogram generated from API system and bacteriological tests revealed that B. cereus and B. thuringiensis are grouped into the same cluster. In combination of sspE PCR and bacteriological tests, the dendrogram showed that 4 strains of B. cereus clustered within the same group. B. thuringiensis formed the subgroup in the same cluster. All strains of B. mycoides were encompassed together. Another cluster only included B. anthracis. The best system was determined to be sspE PCR and bacteriological tests. It is concluded that sspE PCR and bacteriological tests could be used for rapid discrimination and identification of B. anthracis and provided an effective means of differentiation between the B. cereus group.