|Year : 2014 | Volume
| Issue : 1 | Page : 57-59
Catatonia as presenting clinical feature of subacute sclerosing panencephalitis
Prabhoo Dayal, Yatan Pal Singh Balhara
Department of Psychiatry, National Drug Dependence Treatment Centre, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||25-Apr-2014|
Yatan Pal Singh Balhara
Department of Psychiatry, National Drug Dependence Treatment Centre, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Catatonia is not a usual clinical presentation of subacute sclerosing panencephalitis (SSPE), especially in the initial stages of illness. However, there is only one reported case of SSPE presenting as catatonia among children. In this report, however, there were SSPE-specific changes on EEG and the catatonia failed to respond to lorazepam. We describe a case of SSPE in a child presenting as catatonia that presented with clinical features of catatonia and did not have typical EEG findings when assessed at first contact. He responded to lorazepam and EEG changes emerged during the course of follow-up.
Keywords: Catatonia, India, subacute sclerosing panencephalitis
|How to cite this article:|
Dayal P, Balhara YS. Catatonia as presenting clinical feature of subacute sclerosing panencephalitis. J Pediatr Neurosci 2014;9:57-9
| Introduction|| |
Subacute sclerosing panencephalitis (SSPE) is a progressive disorder of central nervous system (CNS).  It usually follows infection with measles virus early in life.  There is a latent period of around 6 to 8 years before SSPE manifests clinically. SSPE typically progresses in stages, which can vary from person to person. Cognitive decline (such as memory loss) and myoclonus coupled with personality, behavioral and intellectual changes are common initial clinical features of SSPE seen in around 80-97% of cases.  Other neurological features like seizure, blindness and hemiplegia tend to occur as illness progresses. In advanced stages of the disease, individuals may lose the ability to walk, as their muscles stiffen or spasm. There is progressive deterioration to a comatose state, and then to a persistent vegetative state. Psychiatric symptoms have also been reported as presenting features of SSPE. 
The diagnosis of SSPE is based on signs, symptoms and typical changes observed in electroencephalograph (EEG).  Additionally, there is elevated anti-measles antibody (IgG) in the serum and cerebrospinal fluid. However, the presence of additional signs and symptoms during initial stages make the case challenging clinically and can delay the diagnosis.
Catatonia is not a usual clinical presentation of SSPE, especially in the initial stages of illness. Catatonic features among cases of viral encephalitis have been reported in the literature. However, there is only one reported cases of SSPE presenting as catatonia among children.  In this report, however, there were SSPE-specific changes found on EEG and the catatonia failed to respond to lorazepam. We describe a case of SSPE in a child presenting as catatonia that presented with clinical features of catatonia and did not have typical EEG findings when assessed at first contact. He responded to lorazepam and EEG changes emerged during the course of follow-up.
| Case Report|| |
A 14-year-old schoolboy was brought to Department of Psychiatry at a tertiary care general hospital in India. The parents reported an insidious onset illness with a progressive course of 6 months duration with no apparent precipitating factor. The presenting complaints included reduced interaction, minimal responsiveness to questions and occasional mimicking of utterances of others. He would keep standing at one spot for long periods of time without any apparent reason, make poor eye contact and would resist attempts at moving him. He would not follow instructions and had to be force fed. At times he would clench his teeth while being fed making it difficult to feed him. He was admitted to Psychiatry ward for detailed assessment. The parents reported that these features followed a brief initial phase characterized by poor school performance, withdrawn behavior and decreased interaction with others. His birth history and developmental milestones were found to be normal. Past medical and family history was also non-contributory. There was no history of any prolonged high-grade fever or history of abnormal body jerky movements or history of fits. There was no history to suggest motor, sensory, and bladder or bowel involvement. There was no history of headache, vomiting, loss of consciousness, dimness of vision or jaundice. There was no history suggestive of cranial nerve dysfunction. There was no history suggestive of measles in past. Immunization status could not be confirmed. There was no history of use of psychoactive substances. On examination, he was conscious and afebrile. General physical examination was unremarkable and his vitals were stable. Application of Bush Francis Catatonia Rating Scale (BFCRS)  revealed a score of 8. He was found to have signs of mutism, posturing, echolalia, grimacing, rigidity and negativism. No hallucinatory behavior was observed during the course of examination.
Bio-chemical variables including complete hemogram, liver functions and renal functions were with-in normal range. Electroencephalogram (EEG) showed generalized slowing and CT brain also did not reveal any abnormality. The case was provisionally diagnosed as catatonic syndrome. Intravenous lorazepam was initiated in a dose of 1mg three times a day. Catatonic signs and symptoms partially responded to lorazepam with a reduction in BFCRS score to 4.
Then patient was referred to department of neurology for further evaluation and management. However, he dropped out of follow-up. He again visited Department of Psychiatry after 4 months with myoclonus, episodes of generalized tonic-clonic movements and neurological deficits. Neurological consultation was sought and the case was diagnosed as SSPE. EEG was repeated and blood sample was sent for anti-measles serology. EEG was characterized by periodic complexes consisting of bilaterally symmetrical, synchronous, high-voltage bursts of polyphasic, stereotyped delta waves [Figure 1]a-c. He, however, again dropped out of treatment during the first week.
| Discussion|| |
Catatonia is a distinct motor syndrome first described in 1874 by the German Psychiatrist Karl Ludwig Kahlbaum. Various studies have reported psychiatric causes of catatonia ranging from 70% to 80% and organic causes of catatonia ranging from 20% to 30%. , Also, responses to Lorazepam treatment among catatonic patients have been reported in some studies, mainly among psychiatric cases. , Catatonia has not been reported as a usual clinical manifestation of SSPE during early stages of the illness. States of minimal conscious progressing to akinetic mutism are usually seen only in the advanced stages of SSPE. 
There are only two earlier published reports of SSPE presenting as catatonia in the initial stages. , One of these two cases was a 13-year-old boy.  The case presented with features of catatonia and the diagnosis of SSPE could be confirmed based on typical changes on EEG, MRI and serological findings at time of first presentation. In the current case, however, EEG changes were not suggestive of SSPE at the time of first presentation. Only a non-specific generalized slowing was observed on EEG. Consequently, the case could not be identified as that of SSPE and was managed symptomatically for catatonia. Unlike the earlier reported case, the current case responded to lorazepam during the initial few days and a reduction in catatonic features was observed. The previously reported case deteriorated following a trial of lorazepam. 
The diagnosis of SSPE was later confirmed based on usual neurological manifestation, EEG recordings and serological findings. Interestingly, a history of measles infection earlier during childhood could not be established as well in this case. Failure to elicit past history of measles infection has been reported in earlier reported cases of SSPE among children as well. 
The case highlights the importance of considering possibility of viral encephalitis as a possible cause of catatonic presentation during childhood. Such a consideration is warranted even if the history of viral illness earlier during childhood is not evident. The patient needs to be kept under regular follow-up. Relevant investigations need to be repeated during the course of follow-up even if the earlier findings were non-contributory. Also, a trial of lorazepam can be considered for catatonic features during earlier stages.
The case is of particular relevance as annual prevalence of SSPE has been reported to be high at 21 cases per million people in some settings.  While the prevalence has been reported to be lower in developed countries, SSPE is of relevance to these countries as well in context of the migrated population. Cases of SSPE have been reported among immigrants even years after resettlement. 
Also, EEG is a cost-effective tool in establishing diagnosis of SSPE in such settings. It can be repeated at periodic intervals if the clinical features fail to resolve. Neuroimaging techniques such as MRI and serological investigations are more expensive and not widely available in many settings.
In conclusion, SSPE can present initially with catatonic features. Therefore, pediatricians, neurologists as well as psychiatrists should be aware of this possibility. A high index of suspicion is needed to detect SSPE in its atypical forms.
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