Remarkable recovery in an infant presenting with extensive perinatal cervical cord injury.

Cervical-cord damage is a complication of a difficult delivery, and results in spinal shock with flaccidity progressing to spastic paralysis. Conventionally, outlook for such patients is extremely poor and most will recover only slightly from quadriplegia and autonomic dysfunction. Here, we report a case in which the extent of damage considerably contrasted with the outcome and recovery. A full-term baby girl born by difficult vaginal delivery displayed bilateral flaccid paralysis of the lower limbs with absent spontaneous movements, weakness of both upper limbs, hyporeflexia in all limbs and axial hypotonia. MRI of cervicothoracic spine exhibited raised signal intensity in the dorsal aspects of C7 to T1 signifying myelopathy. MRI at 4 months revealed a near-total transection of the cervical cord. However, at 6 months, the child could move all lower limbs independently with a marked increase in power. There was no spasticity, wasting or incontinence. Reflexes had also returned.

Risperidone treatment in 12 children with developmental disorders and attention-deficit/hyperactivity disorder.

BACKGROUND: Risperidone is a novel antipsychotic drug that has been tried in the treatment of several child psychiatric disorders. In an open clinical study, we evaluated the safety and efficacy of risperidone in children with developmental disorder and behavioral problems including attention-deficit/hyperactivity disorder (ADHD). METHOD: Twelve patients aged 4 to 14 years who had a DSM-IV-diagnosed developmental disorder and ADHD in addition to other behavioral problems, in particular aggression, were treated with risperidone for a period of up to 2 years with daily doses ranging from 1 to 3 mg. Data were gathered from December 2002 to December 2004. RESULTS: A positive clinical response was noted in 9 of the 12 patients within 3 months of study recruitment according to the Clinical Global Impressions-Improvement scale. Risperidone was well tolerated by all 12 patients. The most commonly reported side effect was sedation, which necessitated dosage reduction in 2 patients, but not discontinuation. CONCLUSIONS: Our findings suggest that risperidone may be an effective and safe treatment for children and adolescents with developmental disorder and disruptive behaviors.

Epilepsy in children with cerebral palsy.

OBJECTIVES: To study the occurrence, associated factors, nature and prognosis of seizures in children with cerebral palsy (CP). DESIGN: A prospective, descriptive, hospital-based, case-control study. SETTING: Tertiary level University Teaching Hospitals in the Al Ain Medical District, United Arab Emirates. PATIENTS: Fifty-six children with CP and seizures seen in the neurodevelopmental clinics at Al Ain and Tawam University Hospitals during the period of 1997-1999 were studied (group 1). Two control groups of 35 children with CP without seizures (group 2) and 50 children with seizures but no CP (group 3) were also studied. RESULTS: Spastic tetraplegia was the commonest type of CP associated with seizures whereas spastic diplegia was the commonest variety of CP in group 2. Most children with CP had an early onset of seizures within the first year of life as against those without CP. The children in group 1 had a higher incidence of neonatal seizures (42.9% vs. 29.4% in group 2 and 0% in group 3), presence of significant developmental delay (98.2% vs. 20.0% in group 3), occurrence of significant abnormalities on brain imaging (94.6% vs. 19.6% in group 3) and a need for use of more than 1 antiepileptic drug (66.1% vs. 30.0% in group 3). Over half of children in the study group presented with generalized tonic clonic seizures; the electroencephalogram (EEG) showed focal epileptic discharges with or without secondary generalization in 39.3%. The overall outcome of seizures in children with CP was poor needing prolonged course of anticonvulsant medications, polytherapy and higher incidence of refractory seizures and admissions for status epilepticus compared to the control group. CONCLUSIONS: Cerebral palsy is associated with a higher incidence of seizure disorders, which, in a majority, has its onset in the neonatal period; brain imaging showed abnormal pathology in most affected children, which possibly accounts for the tendency to more refractory seizures in these children.

Predictors of febrile seizure: a matched case-control study.

In a prospective matched case-control study carried out to determine risk factors of febrile seizures among children in the United Arab Emirates, 84 patients with febrile seizure were identified and were matched with 84 control febrile patients without seizure in the same age range, who attended the same hospital during the same period of time. Logistic regression analysis showed that the age at first seizure, family history of febrile seizure, duration of fever, and height of temperature were the only significant predictors for febrile seizures.

CT features of olivopontocerebellar atrophy in children.

Between 1990 and 1992, 14 children were seen in whom a clinical diagnosis of olivopontocerebellar atrophy (OPCA) had been made. The majority of patients presented with cerebellar ataxia and hypotonia. Five children had a family history of a similar illness in first-degree relatives. All cases had undergone clinical and neurologic examinations, routine laboratory tests and cranial CT. CT features were graded to quantitate the degree of atrophy in each cerebellar hemisphere, vermis and brain stem. All patients had varying degrees of atrophic changes of cerebellum, brain stem and cerebrum. These CT features appear to be distinctive enough to enable the diagnosis of OPCA to be made.

A syndrome of olivopontocerebellar atrophy and deafness with onset in infancy.

Olivopontocerebellar atrophy (OPCA) is rare in childhood and onset in infancy is uncommon. We encountered 11 consecutive children with clinical and radiological features of OPCA which started in infancy. In addition to cerebellar ataxia, these children also had sensorineural deafness and speech impairment. Of the present cases, 8 were sporadic and the pedigree patterns in 3 (with a sibling also involved) point to an AR inheritance. The CT scan showed varying degrees of cerebellar and pontine atrophy. The underlying genetic and neurochemical substrates of this syndrome await further study.

Changing trends in the epidemiology and management of gonococcal ophthalmia neonatorum.

Between June 1985 and December 1988, 58 cases of gonococcal ophthalmia neonatorum were admitted to the neonatal unit at the University Hospital (USM). Of these, 15 (25.9%) cases were due to penicillin-resistant strains of Neisseria gonorrhoea. Of the 58 cases, 56 cases were treated effectively with a single dose of antibiotic given systemically. The mean period of recovery was shorter with spectinomycin in doses of 40 mg/kg than with cefotaxime (100 mg/kg). There was no permanent sequelae in the treated cases. An increasing incidence of infection with penicillin-resistant strains of N. gonorrhoea has been observed in the area of study.

A clinical, laboratory and echocardiographic profile of children with acute rheumatic fever.

A retrospective study of 42 children with acute rheumatic fever admitted to Hospital Universiti Sains Malaysia from April 1985 to March 1989 was undertaken to assess the clinical, laboratory, echocardiographic aspects and outcome. The ages of the children ranged from 5 years 9 months to 11 years 11 months. There was no significant sex difference. 69.4% were admitted between November and April with a seasonal low between May and August. Sixteen children (38.1%) were hospitalised for recurrence of rheumatic fever. Carditis was the commonest manifestation and was seen in 28 (66.6%) children, followed by arthritis in 24 (57.1%), and chorea in 3 (7.1%). Echocardiography detected abnormalities in 24 out of 35 cases and the most common echocardiographic findings were poor coaptation of mitral valve (ten) left ventricular dilatation (ten), thickened mitral valve cusps (seven) and pericardial effusion (seven). In those children followed up, there were 2 recurrences while on secondary prophylaxis and complete recovery was seen only in 11 (26.9%).

Pyogenic meningitis in hospitalized children in Kelantan, Malaysia.

A 2.5-year retrospective study of pyogenic meningitis in hospitalized children in Kelantan was carried out with regard to aetiology, clinical features, investigation, treatment and outcome. There were 58 children with 43 cases (74.1%) occurring below the age of 1 year. Frequent presenting symptoms included fever (98.3%), fits (77.6%), anorexia (39.7%), vomiting (34.5%) and drowsiness (12.1%). On admission, 37 (63.7%) had neck stiffness, 10 (17.2%) had Kernig's sign and 32 (55.2%) had coma. CSF cultures were positive for Haemophilus influenzae in 29 (50%), Streptococcus pneumonia in 13 (22.4%) and Neisseria meningitidis in 3 (5.2%). The antibiotic sensitivity profiles showed that the three main organisms were 100% sensitive to Chloramphenicol, Streptococcus pneumoniae was 100% sensitive to penicillin, Neisseria meningitidis was 100% sensitive to penicillin and ampicillin, and Haemophilus influenzae was 90% sensitive to penicillin and ampicillin. The total hospital mortality was 18.9%. All but two of the eleven deaths occurred in children younger than 1 year. Nineteen of the 35 (54.3%) survivors attended for at least one follow-up after discharge from hospital. Of these 19 children, 47.4% had neurological sequelae.

Effective management of long Q-T syndrome with amiodarone.

It is well known that episodic loss of consciousness and convulsions may have a cardiac rather than a cerebral origin (1). We report a case where these episodes were caused by recurrent ventricular tachycardia/ventricular fibrillation secondary to idiopathic long QT syndrome which was effectively treated with amiodarone.

Evoked potentials and Doppler vascular studies in hydranencephaly.

A study of brainstem auditory evoked potentials, electro-encephalography and doppler vascular scans were conducted in an infant with hydranencephaly. The abnormalities detected were consistent with the gross cerebral cortical anomaly in hydranencephaly.

Clinical and electroencephalographic features of complex partial seizures in infants.

The clinical and electroencephalographic (EEG) features were evaluated in a consecutive series of 50 infants with complex partial seizures. The age of onset of seizures showed a peak at age of 2 months. Significant development delay was seen in 60% of the infants. In 92% an underlying aetiological factor could be identified. Birth asphyxia was the commonest aetiological factor (30%). The seizure patterns were most frequently described as behavioural arrest, upward deviation of eyes, tonic posturing of the limbs, apnoea and cyanosis. Interictal EEG showed bilateral temporal lobe foci in 22%, unilateral foci in 78% and multiple foci in 46% of the cases. The response of the seizures to anticonvulsant drugs is discussed.

Hydrops of the gall bladder associated with Kawasaki syndrome.

Acute hydrops of the gall bladder has been reported in Kawasaki syndrome (KS) (Mucocutaneous lymph node syndrome/MCLS) as a major component of abdominal crises. We report 2 cases of KS where hydrops of the gall bladder was not associated with abdominal pain and was diagnosed by ultrasonography. In additional, one of these cases had ischemia involving the penoscrotal region secondary to vascular involvement.

Cerebral infarction in juvenile rheumatoid arthritis.

Cerebral involvement associated with juvenile rheumatoid arthritis is rare. It is not influenced by treatment and the presentation can be varied. We describe a case of cerebral infarction secondary to vasculitis in a child with juvenile rheumatoid arthritis.