Brief Reports

TRENDS OF NEUROLOGICAL DISORDERS IN SAUDI CHILDREN

Mohamed El Hag El Awad, FRCPI, MRCP(UK),DCH; Michael Adedoyin, FAAP

Analysis of 302 pediatric medical cases with neurological disorders from the Southwestern Region of Saudi Arabia showed the following distribution: 38% were due to seizure disorders, 28.1% to mental retardation, 18.5% to cerebral palsy, 3.3% to paralytic disorders, 3.3% to speech disorders, 2.3% to muscle diseases, 2.0% to hydrocephalus, 1.7% to degenerative brain disease, 1.4% to CNS infections and 0.7% due to migraine. Three main disorders stand out as the most prevalent: seizure disorders, mental retardation, and cerebral palsy. This is the same trend in developed countries. Neurological disorders, either acute or chronic, constitute a big proportion of the pediatric population attending hospitals. Invariably they need sophisticated investigations, rather expensive treatment and longterm follow-up. Their impact is heavy on the child, the family and the community. A large number of these disorders carry the real potential of being or becoming lifelong disabilities or handicaps and henceforth jeopardize afflicted children of normal development and put a great deal of stress on their families.1,2 These factors make the need to study their impact (incidence, prevalence, trends, etc.) and types an important one so that proper recommendations can be drawn up. Neurological disorders do seem to be among the most common childhood ailments in the Middle East. This could in part be related to the high frequency of congenital CNS malformations, either structural or functional.3-7 It is possible that the high degree of consanguinity plays a major role in this. This paper studies the frequency of neurological disorders encountered in Prince Abdullah Hospital, Bisha (PAAH), Southwestern Region of Saudi Arabia over the period of 21 months from January 1991 to September 1992. The cases were looked after and followed up by senior general pediatricians. The hospital has good diagnostic and registry facilities. It serves only a rural population with people working mainly on small farms or

From the Department of Child Health (Dr. El Awad), College of Medicine, King Saud University, Abha Branch, Abha and Senior Consultant Pediatrician (Dr. Adedoyin), Prince Abdullah Ibn Abdulaziz Hospital, Bisha. Address reprint requests and correspondence to Dr. El Awad: Department of Child Health, College of Medicine, King Saud University, Abha Branch, P.O. Box 641, Abha, Saudi Arabia. Accepted for publication 25 May 1994. looking after herds of cattle. It is the only unit in the area that deals with childhood neurological disorders. The study was a retrospective one, looking at the distribution and frequency of the different disorders. This type of study (with its limitations) remains an important source of information in the absence of a proper registry system. One of those limitations is that some of the neurological disorders never present to hospitals, especially in rural areas. They are either kept at home or dealt with by the local healers.8,9 Material and Methods The case records of children with neurological disorders (age six weeks to 13 years) who were either admitted or seen as outpatients at PAAH (Bisha) between January 1991 and September 1992 were retrospectively reviewed. The distribution of cases included both acute and chronic conditions. Chi-square test was used in statistical analysis. Results

Three hundred and two cases were analyzed. This represented the major neurological diagnosis entered for the patients studied (222 patients). The discrepancy was due to the fact that some children have more than one neurological problem (e.g., a child with cerebral palsy could have mental retardation and/or epilepsy). The male to female ratio was 1:0.6. The frequency of the different conditions was as follows (Table 1): seizure disorders, 115 cases (38.0%); mental retardation, 85 cases (28.1%); cerebral palsy, 56 cases (18.5%); paralytic disorders 10 (3.3%); muscle disease, five (1.7%); CNS infections, four (1.4%); and migraine, two cases (0.7%). Table 1 also shows the age range and the male to female ratio of each disorder. Seizure Disorders This is the largest group of the series (115 cases). See Table 2. There were 60 cases (52.1%) of epilepsy; 51.7% of them idiopathic and 48.3% symptomatic. This last group was composed of 25 cases (86.2%) of cerebral palsy and four cases (13.8%) of degenerative brain diseases. Hypocalcemic seizures were encountered in three cases (2.6%) of the total). Eight other conditions (7% of the total) had episodic seizures. There was one case for each of the following: encephalitis, cerebrovascular accident, Down syndrome, hydrocephalus, hemiplegia, isolated mental retardation, congenital toxoplasmosis and DTP immunization encephalopathy. Mental Retardation This group was made up of children detected to have significant disabling mental retardation (moderate to severe). The distribution of the cases was as follows - (Table 3): 46 cases (54%) associated with cerebral palsy, 15 cases (17.6%) with Down syndrome, five cases (5.9%) postmeningitic, five cases (5.9%) undetermined, four cases (4.7%) degenerative brain disease, three cases (3.5%) congenital hypothyroidism, two cases (2.4%) hydrocephalus, two cases (2.4%) poikilodystosis, two cases (2.4%) congenital toxoplasmosis and one case (1.2%) hypoparathyroidism. Cerebral Palsy This group included 56 cases. Their ages ranged from three months to 13 years (mean three years and eight months) - Table 1. The following characteristics were noted - (Table 4): 51 cases (91.1%) of the spastic type, two (3.6%) dyskinetic and three (5.3%) mixed type. Birth asphyxia was detected in 26 cases (46.4%), prematurity six
TABLE 1. Type Seizure disorders Mental retardation Cerebral palsy Paralytic disorders Speech disorders Muscle disorders Hydrocephalus Degenerative brain disease Infections Migraine Neurological disorders - breakdown of cases No. (%) 115(38.0) 85 (28.1) 56 (18.5) 12 (4.0) 10 (3.3) 7 (2.3) 6 (2.0) 5 (1.7) 4 (1.4) 2 (0.7) Age Range (Mean) 2 wks - 13 yrs (3 yrs 9 mos) 1 mo - 13 yrs (4yrs 11 mos) 3 mos - 13 yrs (3 yrs 8 mos) 5 mos - 9 yrs (3 yrs 7 mos) 2 yrs - 9 yrs (5 yrs 3 mos) 1.5 mos - 11 yrs (5yrs 7 mos) 2 mos - 8 yrs (3 yrs 2 mos) 5 mos - 2.5 yrs (1 yr 8 mos) 2.5 mos - 8 yrs (3 yrs 10 mos) 9 yrs - 12 yrs Male: Female Ratio 1:0.6 1:0.7 1:0.7 1:1 1:0.8 1:0.8 1:0.5 1:1.5 1:0.3 -

(10.5 yrs) TABLE 2. Type Epilepsies Idiopathic Symptomatic Febrile convulsions Hypocalcemic seizures Others Total TABLE 3. Type Cerebral palsy Down syndrome Postmeningitic Unknown Degenerative brain disease Congenital hypothyroidism Hydrocephalus Poikilodystosis Congenital toxoplasmosis Hypoparathyroidism Total TABLE 4. Cerebral palsy. No. % Mental retardation analysis. No. 46 15 5 5 4 3 2 2 2 1 85 % 54 17.6 5.9 5.9 4.7 3.5 2.4 2.4 2.4 1.2 100 : : 51.7% 48.3% 44 3 8 115 (38.3) (2.6) (7) (100) The seizure disorders. No. 60 (%) (52.1)

Characteristics Type Spastic Choreoathetoid (dyskinetic) Mixed Etiology Unknown Birth asphyxia Prematurity Meningitis/encephalitis Hyperbilirubinemia Associate Major Neurological Problems Mental retardation Epilepsy

51 2 3

91.9 3.6 5.3

16 26 6 6 2

28.6 46.4 10.7 10.7 3.6

46 21

82.1 37.5

(10.7%), meningitis/encephalitis six (10.7%), hyperbilirubinemia two (3.6%) and the remaining 16 cases (28.6%) of unknown causes. Forty-six cases of the total (82.1%) had associated mental retardation while 25 cases (44.6%) had associated epilepsy. Miscellaneous Paralytic Group This group had the following disorders: two cases of Erb palsy, one case of poliomyelitic monoplegia, one case of acute hemiplegia, two cases secondary to toxoplasmosis (one hemiparesis and the other

quadriparesis), one case of quadriparesis following meningitis, two cases of progressive quadriparesis secondary to degenerative brain disease and one case of Bell palsy. The cases of muscle diseases included three cases of autosomal muscular dystrophy, two cases of congenital muscular dystrophy, one case of Duchenne muscular dystrophy and one case of undetermined myopathy with multiple deformities. Speech Disorders Ten cases were encountered. There were five deaf children with impaired speech, four with isolated delayed speech development and one case of dyslexia. Six cases of hydrocephalus were seen. Two of them were due to congenital toxoplasmosis, one case of Dandy Walker malformation, one with arachnoid cyst of the posterior fossa, one due to intracerebral hemorrhage and one congenital hydrocephalus of undetermined etiology. Five cases presented with regression of milestones. They were thought to have degenerative brain disease. They were referred to higher centers for further workup. The infectious group showed two cases of congenital toxoplasmosis (with other disabilities), one case of septic meningitis and another with encephalitis. Only two cases (males) with migraine as the primary complaint were seen. Discussion In this study, certain trends have emerged as leading causes of central nervous system disorders. The three main problems encountered are seizure disorders (38%), mental retardation (28.1%) and cerebral palsy (18.5%). It is interesting to note that cerebral palsy constituted the major portion of each of the other two disorders. Of the seizure disorders, 21.8% have associated cerebral palsy whereas 54% of the cases of mental retardation were due to cerebral palsy. Although the diagnosis of cerebral palsy was made mainly on clear clinical criteria, there remains the rare occasion of a neurometabolic disorder being hidden among these cases, especially in the absence of a detectable perinatal insult.10 This is the area where screening can be instituted early in life. Izoura's and Anis' study (1992) from the Al Majarda area showed 28.9% of their cases were due to seizure disorders, 18.9% due to cerebral palsy and 16.7% were due to mental retardation. 11 Their relative lower frequencies for both the seizure disorders and mental retardation are possibly due to the fact that they have not included the cases of cerebral palsy, with either epilepsy or mental retardation, to the total percentage of each. However, there is no statistical difference in the frequency of most of the disorders between our study and theirs (P>0.05). In our hospital experience, it is usually the case to see cerebral palsy cases with coexistent mental retardation and epilepsy. Awada et al. (1992), in a study from the Eastern Province, showed the seizure disorders constituted 35%, mental retardation 22% and cerebral palsy 15% of childhood neurological disorders. 12 Although it is of little value to compare absolute percentages, their results are comparable to ours, whereas an earlier study from the Gassim region by Awada (1990) showed a relatively small percentage of both cerebral palsy and mental retardation among children (6% and 4% respectively).13 Of the seizure disorders, febrile convulsions stand out as the most common. It is possible that their prevalence is much more common than the number admitted to hospitals. Some children might be treated locally by traditional healers.8,9 It is interesting to see that the largest portion of mental retardation cases is accounted for by cerebral palsy (over 50%), i.e., over 80% of the cases of cerebral palsy have significant mental retardation. Approximately half of the cases were due to complicated births with significant birth asphyxia. Home delivery is still common in this community. Prematurity accounted for 10.7% of cases while hyperbilirubinemia accounted for only 3.6% of the cases. Fifteen cases of Down syndrome were seen. This accounts for 5% of the total. It is notable that most of the children were born to multiparous women of relatively advanced age. This is the same trend among Arab and other populations.14,15 The rest of the disorders do not show an outstanding prevalence. It is noteworthy that we have encountered few infectious cases. There is virtual absence of recent paralytic poliomyelitis cases. We have not encountered conditions such as hyperactivity disorders, behavioral disorders and recurrent

headaches as the area served by the hospital is a rural one and such cases rarely present to the clinics. In conclusion, we acknowledge the limitations of this study for being retrospective and hospital-based. However, it casts light on one of the important childhood ailments (CNS disorders). The trend emerging in this rural area is of important conditions constituting the bulk of childhood patients suffering from CNS disorders. These conditions are seizure disorders, mental retardation and cerebral palsy. While regional and, still better, nationwide population studies could tell the true incidence of these disorders, we hope our data will be of help for future researchers tackling this problem. References 1. Khout SA. The abnormal child: his impact on the family. J Am Phys Ther Assoc 1986;65:60-3. 2. Goodman JF, Cech HS, Barker WF. Early intervention with retarded children: some encouraging results. Dev Med Child Neurol 1984;26:47-55. 3. Al Frayh A, Naguib NA. The pattern of central nervous disease in children in King Khalid University Hospital in Riyadh, Saudi Arabia. J Trop Paediatr 1987;33:124-30. 4. Thalji AA, Abu Osba YK, Han RW, et al. Incidence of neural tube defects in the Eastern Province of Saudi Arabia. J Kwt Med Assoc 1986;20:90-104. 5. El Awad ME. Infantile hydrocephalus in the Southwestern region of Saudi Arabia. Ann Trop Paed 1992;12:335-8. 6. El Awad ME, Sivasankarn S. Neural tube defects in Southwestern region of Saudi Arabia. Ann Saudi Med 1992;12:449-52. 7. Al Jawad ST, Shubbar AL, Khafaji NA, et al. A survey of serious congenital morphological abnormalities in Abu Dhabi. Ann Trop Paediatr 1988;8:76-9. 8. El Awad ME, Vijayakumar E. Native manual tonsillectomy: a dangerous practice in Asir. Ann Saudi Med 1992;12:188-90. 9. El Awad ME. Skin cauterization as means of remedy in Asir. Saudi Med J 1991;12:294-7. 10. Gascon GG. Neurological disorders in Saudi children. Ann Saudi Med 1993;13:91-3. 11. Izoura GI, Anis AS. Neurological disorders in Saudi children at the Al-Majardah General Hospital, Asir region. Ann Saudi Med 1992;12:191-5. 12. Awada A, Bademosi O, Ismail H, Al Rejeh S. Outpatient neurologic practice in Saudi Arabia and its implications for education in neurology. Ann Saudi Med 1992;12:503-5. 13. Awada A. Pattern of neurological disorders in Gassim region. Saudi Med J 1990;11:253-4. 14. Mir NA, Soni AL, Galak WK. Trisomic Down syndrome in Arab population. Ann Saudi Med 1992;12:228-9. 15. Trimble BK, Baird PA. Maternal age and Down syndrome: age specific incidence rates by single year maternal age intervals. Am J Med Genet 1978;1:1-5.