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Infantile tremor syndrome in Iraqi Kurdistan
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     1 Department of Psychiatry, College of Medicine, Sulaiamni Arbil Children's Hospital, Arbil, Iraq

    2 Department of Pediatrics, Arbil Children's Hospital, Arbil, Iraq

    Abstract

    Objective : To describe a rare condition that occurred for the first time in Iraqi Kurdistan during the UN Sanctions on Iraq in the nireties. Literature review was made to fine possible causes. Methods : Patients were collected from various sources including private clinics, hospital outpatient clinics and the Centre for Handicapped Children in the city of Arbil. Clinical features and proper history were the main diagnostic tools. Different treatment regimes were used on those patients. Results: 24 patients were collected; the majority was between 6-18 months. Males were more affected than females. Half of the patients were exclusively breast-fed and three quarter have received blood for anemia. Conclusion: Infantile tremor syndrome is a rare condition which was described both in India and outside India as well. It was observed among children in Iraqi Kurdistan during the difficult economic sitution under the UN Sanctions. The condition has the same features inside and outside India. The etiology is not very clear yet.

    Keywords: Kurdistan; Sanctions Arbil; Tremor; Breast-fed

    A clinical syndrome of infants and young children with neurological manifestations was reported in Indian children at the beginning of the second half of the twentieth century. Different clinical pictures were described. Nevertheless, the first clear and complete clinical description for the disease was by Pohawalla et al from India in the year 1960.[1]

    A few identical cases were reported from America, Italy, and South East Asia during the 1950s.[2] The Indian Pediatricians became interested in the condition and described it with different names such as Nutritional Dystrophy and Anemia by Dikshit in the year 1957,[3] Infantile Meningo-encephalitic Syndrome by Pohawalla et al in the year 1960,[1] Vitamin B12 deficiency Syndrome in Indian Infants by Jadhav et al in the year 1962,[4] Syndrome of Tremors, Mental Regression and Anemia by Sachdev et al in the 1965[5] and the Syndrome of Tremor in Indian infants by Kaul in the year 1972.[6]

    The syndrome was also reported from places outside India and the condition was seen among the Bangladeshis during the period 1971-1972.[7] Nine patients from Afghanistan were reported in the year 1988.[8] Many theories were proposed for the etiology including slow virus - infection of the brain or meninges,[1],[9] or viral infection before the appearance of the tremor.[8] Nutritional deficiencies were suggested such as cobalamine deficiency.[4],[10],[11],[12],[13] other anemias[12] and magnesium deficiency.[14] Infantile tremor syndrome was reported to appear for the first time after blood transfusion.[2] The condition was described in infants from families of lower socioeconomic status.[15],[16] and seasonal variation was also reported.[16],[17] In India, it was seen in children who were on exclusive breast-feeding with mothers who were predominantly vegetarian.[13],[18] The patients were five months to three years old.[16],[19] Males were twice more commonly affected than females.[16],[17] Tremor was the main presenting symptom.[20]

    Other neurological manifestations included myoclonic jerks, choreiform movements.[17] hypertonia, hypotonia[19], [20] with a characteristic posture of winging.[2] There were no sensory disturbances in any of the reports about Infantile Tremor Syndrome. Mental retardation was observed even earlier than the development of the clinical syndrome with a significant increase in the incidence and severity of the mental retardation at follow up.[21]

    Developmental regression was common in most of the reports.[19], [20], [21] Fever, cough and respiratory infection were associated with the disorder.[20] Infantile tremor syndrome is a self-limiting condition and recovery is common.[2], [4], [21] Although the condition was described as self limiting, many drugs were tried for treatment with inconclusive results including propranolol,[22] Vitamin B12, [12],[13],[14],[19],[23],[24],[25] carbamazepine,[26] phenobarbitone and chlorpromazine,[19],[23] iron, multivitamins, high protein diet and Vitamin C.[17], [19],[23] The condition was not known in Iraqi Kurdistan before the invasion of Kuwait in the year 1990. Iraqi Kurdistan involves the Northern, North Eastern and some of the Eastern parts of Iraq. The region was running a continuous process of economic sanctions and intentional deprivation from good health services compared to the rest of Iraq under Saddam's ruling. The international sanctions on Iraq and the sudden withdrawal of Iraqi administration created a crisis in the region because of the already poor health services. The deterioration of the economic state of the people, because of the lack of salaries and massive unemployment as well as the destruction of all the villages in the region by the regime, added to that situation. Cases of malnutrition were seen and cases of rickets disappeared because the bones were not growing as fast as they should be under ordinary circumstances. The authors were working in Arbil, which is the capital of Iraqi Kurdistan and was described as the summer capital of Iraq by the previous regime. They noticed appearance of a few cases of tremor in infants. This draw their attention and made them take up the present study.

    Materials and Methods

    The patients were examined by the authors in their private clinics, hospital outpatient clinics or in the Centre for Handicapped Children. Those working places were all inside the city of Arbil. The study lasted two years 1995-1997. The diagnosis was made on clinical features and history of transfusion was the triggering factor to suggest of the condition.

    The children were admitted with their mothers in most of the cases. They were admitted to the Children's Hospital in Arbil or treated as outpatients. They were allocated to different treatment regimes randomly. The patients remained until they improved or were discharged to be followed up in the outpatient clinic or in the private clinics.

    Results

    Both the authors collected 24 patients with age range of 6-36 months table1, and the majority was between 6-18 months. Males were more affected than females table2. Most of the cases were from Arbil city table3. 95.83% of the patients were suffering from malnutrition of various degrees table4. Half of the patients were exclusively breast-fed table5. Interestingly 79.17% of the cases had received blood for anemia table6. The tone of the muscles was of various types and developmental regression was noted in 75% of the cases table7. Comorbidity was demonstrated in table8. Some of the patients had more than one type comorbidity. EEG was done on seventeen patients and it was not significant table9. The patients were given anticonvulsants and tonics [Table - 10] and the overall result was that 91.67% of them improved [Table - 11].

    Discussion

    Infantile tremor syndrome was not known in the region before 1990. The deterioration of the economic state of the residents following the UN sanctions and the sanctions imposed by the Iraqi government laid the grounds for the appearance of cases of malnutrition in the infants and young children. Pregnant and nursing mothers were malnourished too. The high price of the usual sources of proteins in the region such as meat and peas as well as lack of vitamins due to the shortage of fruits support the nutrtitional etiology of the condition. The wars in the region especially the legacy of the pollution resulting from the use of chemical weapons, the use of radioactive weapons during the second gulf war and the distribution of poor quality food ingredients through the Oil for Food Program might support the hypothesis of toxic etiology. The high percentage of the cases in the city of Arbil and lower percentages in the rural areas are in favour of the toxic etiology due to overcrowding in the city, or it could be the result of poor access to the health services during those difficult days. The association with blood transfusion was mentioned in one reference only[2]; the present study showed that 79.17% of the cases had history of recent blood transfusion which indicates that those children were indeed anemic and that they were vulnerable to infection during blood transfusions that were carried out under the exceptional circumstances in Iraqi Kurdistan as mentioned in the introduction.

    However, this observation was not repeated in most of the literature that followed the first report.

    The main neurological manifestations including tremor, hypotonia and hypertonia, and posture were consistent with the findings of other researchers in India. Developmental regression with clouding of consciousness indicates an insult to the central nervous system and the recovery supports the viral etiology too. The comorbidities and the outcome of the cases are quite similar to the cases reported from India. The treatments used in the present study did not reveal remarkable results because they were already given on empirical bases.

    These findings indicate that infantile tremor syndrome does exist as an entity in Iraqi Kurdistan and the clinical picture is quite similar to the ones in the Indian medical literature. The lack of resources in the region during the study years was the reason that prevented the authors from investigating the cases in more depth. The 24 cases in two years with the odd cases in those days still indicate that the etiology is more related to the exceptional circumstances at that time.

    Conclusion

    This study demonstrated the existence of infantile tremor syndrome in Iraqi Kurdistan as a result of the exceptionally difficult circumstances and suggests that the etiology is more related to the nutritional state of the children or the mothers and to a lesser extent the possibility of infection mostly viral type.

    References

    1. Pohowalla JN, Kaul KK, Bhandri NR, Singh S.D. Infantile Meningo-encephalitic Syndrome. Indian J Pediatrics 1960; 27: 49.

    2. Gupta BD, Miglani M, Miglani N. Infantile Tremor Syndrome, Indian J Pediatrics 1978; 45: 221.

    3. Dikshit AK. Nutritional Dystrophy and Anemia, Indian Journal of Child Health 1957; 6 : 132.

    4. Jadhav M, Webb JKG, Vaishara S, Baker SJ. Vitamin B12 Deficiency in Indian Infants-a Clinical Syndrome. Lancet 1962; 2 : 903.

    5. Sachdev K, Manchanda SS, Lal H. The Syndrome of Tremor, Mental Regression and Anemia in Infants and Young Children. Indian Pediatrics 1965; 2 : 239.

    6. Kaul KK. Syndrome of Tremor in Indian Infants. J Indian Medical Association 1972; 59 : 473.

    7. Suraj Gupta. Infantile Tremor Syndrome. Indian Pediatrics 1987; 1987; 24 : 1159.

    8. Ayra LS, Singh M, Aram GN, Farahmand S. Infantile Tremor Syndrome. Indian J Pediatrics 1988; 55 : 913-917.

    9. Bajpai PC, Misra PK, Tandon KM, Newton G. Brain Biopsy in Infantile Tremor Syndrome. Indian J Medical Research 1971;59 : 413-417.

    10. Wadia RS, Bandishti S, Kharche M. Bi2 & Folate Deficiency: incidence and clinical features. Neurology India 2000; 48 : 302-304.

    11. Grattan-Smith PJ, Wilcken B, Procopis PG, Wise GA. The Neurological Syndrome of Infantile Cobalamine Deficiency, Developmental Regression and Involuntary Movements, Movement Disorders 1997; 12 (1) : 39-48.

    12. Mahajan CM, Walia BNS, Perkash A. Anemias in Infantile Tremor Syndrome. Indian J Pediatrics 1971; 38 : 362.

    13. Garewal G, Narang A, Das KC. Infantile Tremor Syndrome: A Vitamin B12 Deficiency Syndrome in Infants. J Tropical Pediatrics 1988; 34 : 174-178.

    14. Agarwal R, Sigh RN, Gupta BD, Agarwal D.K. and Arora A.K. Serum Magnesium, Calcium, Zinc in Infantile Tremor Syndrome. Indian Pediatrics 1993 ; 30 : 374-376.

    15. Bajpai PC, Tandon PN, Bajpai PC. Infantile Tremor Syndrome- Probable Etiology. Indian Pediatrics 1971; 8(2) 62-64.

    16. Bajpai PC, Misra PK, Tandon PN Further Observations on Infantile Tremor Syndrome. Indian Pediatrics 1968; 5(7) : 297-307.

    17. Garg BK, Srivatana SR. Infantile Tremor Syndrome. Indian J Pediatrics 1969; 36 : 213-218.

    18. Gupta S., Infantile Tremor Syndrome. In Short Text Book of Pediatrics, 8th edn. New Delhi; Jaypee Brothers, 1988; 558-561.

    19. Sharda B. and Bhandari B. Infantile Tremor Syndrome, Indian Pediatrics 1987; 24 : 415-421.

    20. Ramakumar L, Pandove SP. Infantile Tremor Syndrome. Indian J Pediatr 1975; 331(42) : 215-225.

    21. Bajpai PC, Misra PK. Follow up Studies in Infantile Tremor Syndrome. Indian J Medical Research 1972; 60 (11) : 1711-1717.

    22. Kalra V, Marwaha RK. Propranolo in Infantile Tremor Syndrome. Indian J Pediatrics 1981; (49) : 341-343.

    23. Mahajan CM, Walia BNS, Dutta BN. Infantile Tremor Syndrome- Some Aspects of Serum Biochemistry and Liver Histology. Indian Pediatrics 1971; 8(12) : 831-833.

    24. Vasmani LK, Surti V. Bavdekar SB, Kamat JR. Infantile Tremor Syndrome. J Indian Medical Association 1998; 96(4) : 126.

    25. Goraya J. Persistence of Neurological Damage Induced by Vitamin B12 Deficiency. Achieves of Diseases in Childhood 2004; 78 : 395.

    26. Murali MV, Sharma PP, Kaul PB, Gupta P. Carbamazepine Therapy for Infantile Tremor Syndrome. Indian Pediatrics 1993; 30 : 72-74.(Amin Nazar M. Mohammad, Z)