วารสารสมาคมจิตแพทย์แห่งประเทศไทย
Journal of the Psychiatrist Association of Thailand
ISSN: 0125-6985
บรรณาธิการ มาโนช หล่อตระกูล
Editor: Manote Lotrakul, M.D.

Nongpanga Limsuwan, M.D. *
Spain UneAnong, M.D. *
Tana Nilchaikovit, M.D. *

 * Department of Psychiatry, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.

Abstract A pair of previously well eight-year old identical twin girls developed seizures and mental deterioration. They developed psychiatric symptoms that shared some characteristics of autistic syndrome and other childhood psychoses. Based on the evidences of encephalopathy from the EEG and evidences of inflammation in the CNS from the CSF examination, the most likely diagnosis was encephalitis. They might support the organic bases of autistic syndrome and other childhood psychoses.

J Psychiatr Assoc Thailand 1997 ; 42(3) : 133-141.

 Key words: Autism, childhood psychosis, encephalitis, identical twins 

นงพงา ลิ้มสุวรรณ พ.บ. *
สเปญ อุ่นอนงค์ พ.บ. *
ธนา นิลชัยโกวิทย์ พ.บ. *

  บทคัดย่อ เป็นการรายงานผู้ป่วยเด็กหญิงซึ่งเป็นฝาแฝดไข่ใบเดียวกันและมีอาการทางจิตในเวลาไล่เลี่ยกัน อาการที่เกิดขึ้นมีลักษณะเข้ากับโรคออทิสติก ผลการตรวจทางห้องปฏิบัติการบางอย่างพบลักษณะผิดปกติที่เข้ากันได้กับโรคสมองอักเสบ อาการป่วยของผู้ป่วยทั้งสองรายนี้ช่วยสนับสนุนว่าโรคออทิสติกอาจเกิดจากความผิดปกติของการทำงานของสมอง

วารสารสมาคมจิตแพทย์แห่งประเทศไทย 2540; 42(3) :133-41.

 คำสำคัญ โรคออทิสติก โรคจิตในเด็ก โรคสมองอักเสบ ฝาแฝดเหมือน

 * ภาควิชาจิตเวชศาสตร์ คณะแพทยศาสตร์โรงพยาบาลรามาธิบดี มหาวิทยาลัยมหิดล กรุงเทพฯ 10400

  INTRODUCTION

Viral encephalitis sometimes cause problems in making diagnosis, especially when the cerebrospinal fluid findings do not show obvious abnormalities. Electroencephalography is useful to demonstrate evidences of cases in this condition even when there are only minor clinical abnormalities.^1,2 The attempts to find the specific etiologic agent are more problematic since in the majority of cases we cannot find the specific responsible viruses because tests for many viruses should be done to establish these pathogens and the rising titers of antibodies are inevitably delayed.

We report cases of encephalitis in a pair of monozygotic twin girls who developed similar psychiatric symptoms following the similar acute phase of illness and eventually full recovery were followed in both girls.

 CASE REPORT

At the age of eight years and six months, one of the twins was noticed to be different. She appeared to be confuse, looked out aimlessly and did not respond when being talked to. She could not do her homework, could not remember numbers and was unable to add or subtract. Gradually, she could not recognize her relatives, lost bladder and bowel control, and acted strangely, for example, shouting and screaming inappropriately. Sometimes she exhibited dancing like movements. On the forth or fifth day of her illness, there were jerking movements of her right upper and lower extremities which were relieved by intravenous injection of diazepam at the emergency room. There was one generalized seizure at home.

Six months after the first girl was sick, another girl had fever and sorethroat. After the fever subsided, she developed abnormal movements of left upper and lower extremities. While being examined by the doctor, she had tonic posturing of the left upper extremity, left lateral gazing, teeth clenching, and lost of consciousness for about one minute. She was given anticonvulsive medications and had no convulsion since then, but she continued to have abnormal movements. After two weeks, she decreased talking and started to have jerking movements on her left side. Gradually she stopped talking but still was able to understand when being talked to. After eight months, she became psychotic, she laughed and cried inappropriately, hit and bit others as well as herself. Other symptoms of both twins were listed in Table 1 and Table 2.

Gestation and developmental history

Both twins come out of a full-term first pregnancy. The pregnancy was uneventful. Cesarean section was done for delivery due to twin pregnancy. The birth weights were 2,700 and 2,600 grams. Both twins had normal motor and psychosocial developments. Premorbid personalities were friendly and cheerful.

Social and family history

The patients’ parents have a small business. There is no history of family conflict, financial problem, nor serious child rearing problems. The grandparents live in the same house, and are helpful in taking care of children and household work. The twins have one younger brother and one younger sister who are normal and healthy. The family has neither history of psychiatric illness nor mental retardation.

Physical examination and investigations

The first twin’s physical examination was mostly normal except for positive grasping reflex. Her investigations were also mostly negative, the only abnormality was the electroencephalography (EEG).

The second twin had choreiform movement of the left upper and lower extremities which lasted seven months. She had a limping gait, showing motor weakness on the left side. There was hypertonia and pain in the left side. Her speech was unclear. All investigations were shown in Table 3 and Table 4, most of which were normal. The positive investigations include abnormal electroencephalography, increased pressure and cell count in the cerebrospinal fluid once from three times of lumbar puncture. The Bender Gestalt Test was abnormal, showing signs of organicity, and she got only fifty four scores from the IQ Test.

Psychiatric examination

The first girl was referred to the psychiatrist after one month of neurological care. In the first psychiatric examination, she showed marked disturbances in speech and language functions, and later, she developed more and more psychiatric symptoms as listed in Table 1.

The second girl was seen by the psychiatrist eight months after the onset of her illness. Her symptoms were very similar to the first girl as shown in the same Table.

After two years of the illness, both girls gradually recovered and eventually all symptoms disappeared. Then all medications were discontinued. They were quite normal, could study with average performance, and could have relationship with peers and other family members normally. Both of them remain symptom-free up to the present time.

 DISCUSSION

We had reported two cases of encephalitis in a pair of identical twin girls. Both girls were presented with behavior changes and developed convulsions early in the course of illness followed by involuntary movement and intellectual decline which continued for a long period of time before full recovery gradually ensued.

The clinical features and the course of the illness of both girls were quite similar and since they were close siblings and were living in the same environment we could assume that both had got the same illness. Convulsion, disorientation, and disturbance in higher cortical functions were evidences of encephalopathies. The abnormalities in electroencephalography also confirmed this. The computerized tomography brain scans in both girls were normal, enabling us to exclude space-taking lesion within the crania. The negative cerebrospinal fluid cultures helped us to exclude bacterial infection in the central nervous system. The history of fever and sorethroat preceding the illness in the second girl suggested that the process of the illness might be related to viral infection. In order to find out what virus was responsible for this, a numerous serological tests for a wide range of viruses should be done, although this might not be very beneficial clinically, since the management and prognosis of encephalitis from various viruses were not different from each other, except for that of herpes simplex encephalitis to which specific treatments were available.³ Furthermore, it might take a long period of time to demonstrate the rising of the titer of the antibody against specific viruses.² In our cases there were prominent myoclonic movement which was a feature of subacute sclerosing panencephalitis or SSPE.⁴ The measles antibody titers had been tested, but neither case showed rising nor high titers.

Both girls had had cerebrospinal fluid examination done for several occasions but only once in the second girl was abnormal, showing mononuclear pleocytosis as an evidence of inflammation within the central nervous system. At this point we could conclude that both girls had suffered from an inflammatory process within the brain related to viral infection which could be either an encephalitis (direct invasion of the virus to the brain) or a post-infectious encephalitis (brain inflammation following viral infection elsewhere in the body).

Psychiatric symptoms in patients with encephalitis were not uncommon and could occur in any phase of the illness which might cause problems in making diagnosis.⁵ Patients with encephalitis followed by residual sequele resembling autistic syndrome had been reported.⁶ In our patients, there were disturbances in social interactions, irrelevant speech, agitation, irritability, and inappropriate posturing which might be difficult for physicians to differentiate these from autistic syndrome, childhood schizophrenia, or other childhood psychosis, especially if they saw the patients in later stages of the illness while signs of organic brain syndrome were less obvious.

Kennedy et al⁷ and Rautonen et al³ found that patients who had been unconscious in the course of the illness were likely to have a poor prognosis, hence death or severe sequele were likely, particularly those with young age at onset. Our two cases corresponded with their finding, nevertheless it took two years for both girls to achieve full recovery.

The clinical features and course of viral encephalitis were quite varied⁵ but those presented in our patients were quite similar. The fact that these two girls were identical twins might be responsible for this. They might have some inherited vulnerabilities to the some agents or they might share some tendencies to develop the same psychiatric disorders.

Psychiatric syndromes following organic brain diseases had been used as models for the organic basis of psychiatric disorders.⁶ Our cases which developed autistic-like syndrome and other psychiatric symptoms after encephalitis support the idea that autistic syndrome and other childhood psychoses may have organic basis. The fact that the twins developed similar psychiatric symptoms also suggests that the manifested symptoms might have been determined in part by some genetically related characteristics of the twins.

 REFERENCES

1. Kennard C, Swash M. Acute viral encephalitis: its diagnosis and outcome. Brain 1981; 104: 129-48.

2. Cameron PD, Wallace SJ, Munro J. Herpes simplex virus encephalitis: problems in diagnosis. Dev Med Child Neurol 1992; 34: 134-40.

3. Rautonen J, Koskiniemi M, Vaheri A. Prognostic factors in childhood acute encephalitis. Pediatr Infect Dis J 1991; 10: 441-6.

4. Dunn RA. Subacute sclerosing panencephalitis. Pediatr Infect Dis J 1991; 10: 68-72.

5. Lishman WA. Organic psychiatry. 2nd ed. Blackwell: London, 1987: 290.

6. Gillberg C. Autistic syndrome with onset at age 30 years: Herpes encephalitis as a possible model for childhood autism. Dev Med Child Neurol 1991; 33: 912-29.

7. Kennedy CR, Duffy SW, Smith R, Robinson RO. Clinical predictors of outcome in encephalitis. Arch Dis Child 1987; 62: 1156-62.

 Table 1 Psychiatric symptoms of the twins

 Table 2 Medical data

 Table 3 Neurologic differential diagnoses

 Table 4 Investigations