A proposed classification for subtypes of arterial ischaemic stroke in children.

The aim of this study was to propose a classification system for childhood arterial ischaemic stroke (AIS). Subtypes from the Trial of Org 10172 in Acute Stroke Therapy (TOAST) classification, previously shown to be applicable to children, were retained in the proposed Paediatric Stroke Classification (PSC). Additional important paediatric AIS aetiologies were identified from a literature review. Preliminary validation was performed by three raters who categorized clinical vignettes from 135 patients (66 male; median age 6.3 y, range 0.1 to 16 y). Eight aetiological subtypes were identified and defined, as follows: (1) sickle cell disease; (2) cardioembolic; (3) moyamoya syndrome; (4) cervical arterial dissection; (5) steno-occlusive cerebral arteriopathy; (6) other determined aetiology; (7) multiple probable/possible aetiologies; and (8) undetermined aetiology. There was very good agreement between the raters about categorization of the vignettes. Causes of disagreement were identified and final categories and definitions were modified accordingly. We conclude that the PSC enables the categorization of children with AIS into aetiological subtypes relevant to this age group. This will be useful in multicentre studies of natural history and treatment but will require further independent validation.

Arterial dissection complicating tonsillectomy.

Tonsillectomy is a frequently performed surgical procedure. Surgical complications can be serious. We report the case of a 7-year-old female who experienced an arterial ischaemic cerebral infarction following elective adenotonsillectomy.

Ischaemic stroke subtypes in children and adults.

Ischaemic stroke subtypes in children and adults were compared to determine the similarity in aetiologies. Thirty-six children (22 females, 14 males; median age 5 years 7 months, range 6 weeks to 15 years 10 months) and 50 adults (35 males, 15 females; median age 44 years, range 17 years 2 months to 49 years 11 months) who had presented with ischaemic stroke between 1995 and 2000, were categorized using a modified version of the Trial of Org 10172 in Acute Stroke Therapy (TOAST) classification. Proportions of patients in the subtypes of the TOAST classification system were significantly different in the two groups (chi2 test, p<0.01). The first three subtypes (large artery atherosclerosis, cardioembolic, and small vessel disease) accounted for the majority of adult strokes (27 of 50). In contrast, only three of 36 children were accounted for within these three subtypes. The majority of children (29 of 36) were classified within the 'other determined aetiology' subtype. Aetiology was undetermined in 12 of 50 adults compared with three of 36 children. Causes of ischaemic stroke in children and adults are distinct. A classification system for ischaemic stroke in children would be useful for collaborative studies.

Can mild head injury cause ischaemic stroke?

Five cases of ischaemic stroke preceded by minor head trauma in children are described. All patients had striatocapsular infarction. Three had no cerebrovascular abnormality; two had turbulent flow in the proximal middle cerebral artery. None of the patients had evidence of arterial dissection or any other risk factors for stroke. All made an excellent neurological recovery. Possible mechanisms include mechanical disruption to the flow in the perforating branches of the middle cerebral artery, intimal trauma and subsequent thrombosis, or arterial spasm induced by trauma. The specific susceptibility in affected children remains unexplained; both genetic and environmental factors (for example, previous chickenpox) may be implicated.

Idiopathic intracranial hypertension: is papilloedema inevitable?

Headaches and papilloedema are key features of idiopathic (benign) intracranial hypertension (IIH). We describe three children in whom IIH was diagnosed in the absence of papilloedema. Recognition of atypical cases of IIH is important because pressure lowering treatment may be effective.

Lower limb and back pain in Guillain-Barré syndrome and associated contrast enhancement in MRI of the cauda equina.

UNLABELLED: This study assesses the frequency of lower limb and back pain in children with Guillain-Barré syndrome and reviews the magnetic resonance imaging results of those undergoing spinal imaging. Over an 8-y period, nine children presented with various combinations of severe back pain, leg pains, impairment of gait and bladder dysfunction. Guillain-Barré syndrome was confirmed on clinical examination and peripheral electrophysiology (n = 8). Magnetic resonance imaging in four patients, following contrast injection, showed enhancement of the cauda equine and, additionally, of the cervical nerve roots in one of the patients. A further patient, who was not scanned with contrast, had abnormal thickening of the lumbar roots. Carbamazepine and steroids were effectively used for analgesia in three cases. All the patients recovered. CONCLUSION: Guillain-Barré syndrome should be considered in the differential diagnosis of children presenting with back and/or leg pain. Early diagnosis ensures prompt monitoring for autonomic dysfunction and respiratory compromise.

Clinical expression of Menkes disease in a girl with X;13 translocation.

Menkes disease is a rare X-linked recessive disorder of copper metabolism, characterised by progressive neurological degeneration, abnormal hair and connective tissue manifestations. We report on a girl with classic Menkes disease, carrying a de novo balanced translocation 46,X,t(X;13)(q13.3; q14.3). The translocation breakpoints at Xq13.3 and 13q14.3 coincide with the Menkes disease and Wilson disease loci, respectively.

Familial Guillain-Barre syndrome.

There are few reports of Guillain-Barre Syndrome (GBS) occurring in families. We have encountered a mother, who developed acute inflammatory demyelinating polyradiculoneuropathy at age 35 years, whose son developed the bulbar form of GBS 7 years later. Both shared HLA DR2.

Rapid onset transverse myelitis in adolescence: implications for pathogenesis and prognosis.

Five adolescents with transverse myelitis were reviewed. All presented with a rapid onset paralysis of the lower limbs and impairment of bladder control. The maximum disability developed between 10 minutes and six hours. There was no history of trauma, asthma, or prodromal illness. Investigations failed to demonstrate a vascular cause. Extensive spinal cord abnormalities were observed on magnetic resonance imaging. Electrophysiological investigations, performed in four cases, were all consistent with anterior horn cell damage. In all five adolescents there was poor recovery. The underlying pathogenesis of this rapid onset condition remains a subject of debate. Similarities with both transverse myelitis and fibrocartilaginous emboli are evident, widening the spectrum of conditions within the transverse myelitis umbrella. These observations suggest that in rapid onset "transverse myelitis" the combination of extensive hyperintensity on spinal cord neuroimaging with electrophysiological evidence of anterior horn cell involvement might have adverse prognostic significance.

Summary of measured radiofrequency electric and magnetic fields (10 kHz to 30 GHz) in the general and work environment.

We have plotted data from a number of studies on the range of radiofrequency (RF) field levels associated with a variety of environmental and occupational sources. Field intensity is shown in units of volts/meter (V/m) for electric field strength and amps/meter (A/m) for magnetic field strength. Duty factors, modulation frequencies, and modulation indices are also reported for some sources. This paper is organized into seven sections, each cataloging sources into appropriate RF frequency bands from very-low frequency (VLF) to super-high frequency (SHF), and covers frequencies from 10 kHz to 30 GHz. Sources included in this summary are the following: Coast Guard navigational transmitters, a Navy VLF transmitter, computer visual display terminals (VDTs), induction stoves or range tops, industrial induction and dielectric heaters, radio and television broadcast transmitters, amateur and citizens band (CB) transmitters, medical diathermy and electrosurgical units, mobile and handheld transmitters, cordless and cellular telephones, microwave ovens, microwave terrestrial relay and satellite uplinks, and police, air traffic, and aircraft onboard radars. For the sources included in this summary, the strongest fields are found near industrial induction and dielectric heaters, and close to the radiating elements or transmitter leads of high power antenna systems. Handheld transmitters can produce near fields of about 500 V/m at the antenna. Fields in the general urban environment are principally associated with radio and TV broadcast services and measure about 0.1 V/m root-mean-square (rms). Peak fields from air traffic radars sampled in one urban environment were about 10 V/m, 300 times greater than the rms value of 0.03 V/m when the duty factor associated with antenna rotation and pulsing are factored in.

Neurological sequelae of the dancing eye syndrome.

UNLABELLED: Fifty-four patients with dancing eye syndrome (DES), presenting over a 25-year period, were reviewed retrospectively. One third of them were on active follow up at the time of the study. Malignancy was uncommon, diagnosed in only 4 patients, neuroblastoma in 3 cases and acute lymphoblastic leukaemia (ALL) in 1. An intercurrent illness preceded onset of DES in 51% of the children and was of equivocal significance. There was no clear temporal relationship to immunization. The acute phase of the illness was severe and caused total immobilization in 88% of patients. A favourable initial response to treatment with prednisolone or corticotrophin gel was observed in all patients. Although corticotrophin seemed to produce a more rapid response, overall improvement was similar with both treatments. Long-term neurological morbidity was a major problem with 91% of children suffering either persisting symptoms or repeated relapses. A persisting disability was found in 88% and was considered severe in 61% of patients. No features prognostic of neurological severity or outcome were identified. CONCLUSION: Demonstrable malignancy is uncommon in the dancing eye syndrome. The neurological legacy of DES is often evident well into adult life.

The septum pellucidum and spatial ability of children with optic nerve hypoplasia.

Animal studies suggest that spatial skills are dependent on an intact septum pellucidum. This theory was tested by comparing patients who were visually impaired due to bilateral optic nerve hypoplasia: 13 with a septum pellucidum were compared with six children without a septum pellucidum. There was no difference in spatial ability. The finding of an absent septum pellucidum may only indicate the timing of a congenital brain insult, and it cannot be used to predict specific clinical, neuroendocrinological, cognitive or spatial abnormalities.

Ataxia-telangiectasia in a child with vaccine-associated paralytic poliomyelitis.

Vaccine-acquired poliomyelitis developed in a nonimmunized 10-month-old boy. At age 4 years, ataxia-telangiectasia was recognized. We conclude that the occurrence of vaccine-related poliomyelitis warrants a detailed assessment of immunity, and that, in patients with ataxia-telangiectasia, the use of live vaccines may be hazardous, even in those with apparently normal immunity.

Magnetic resonance imaging of inflammatory and demyelinating white-matter diseases of childhood.

Magnetic resonance imaging (MRI) was performed on 36 children and two adults (with clinical presentation during childhood) with white-matter disease of the central nervous system. Abnormalities were readily demonstrated in patients with multiple sclerosis, acute disseminated encephalomyelitis, leucodystrophies and subacute sclerosing panencephalitis: MRI demonstrated the extent and distribution of abnormalities more clearly than computed tomography for all these disorders. The abnormalities tended to be asymmetrical and multifocal in multiple sclerosis and acute disseminated encephalomyelitis, and more confluent and symmetrical in the leucodystrophies. Children with clinically isolated optic neuritis had a significantly lower frequency of MRI brain-lesions than adults with the same disorder. MRI should be regarded as the radiological investigation of choice when white-matter disease is suspected in children.

The 'happy puppet' syndrome of Angelman: review of the clinical features.

Thirty six children with typical features of Angelman's syndrome, including global developmental delay, ataxia, episodes of paroxysmal laughter, seizures, and microcephaly were studied. The series included three sibships of three affected sisters, two affected brothers, and two affected sisters, respectively. The facial appearance is characterised by a prominent jaw, a wide mouth, and a pointed chin. Tongue thrusting is common. The movement disorder consists of a wide based, ataxic gait with frequent jerky limb movements and flapping of the hands. Tone is variable in the limbs with normal reflexes, and the plantar responses are usually flexor. The syndrome is being diagnosed more often, and attention is drawn to its diagnostic aspects.

Choreic syndrome after cardiac surgery.

Five children had an acute choreic syndrome that started three to seven days after hypothermic cardiopulmonary bypass surgery. Improvement after an interval was seen in all, with complete resolution in two. In addition to the chorea, all children suffered complete supranuclear ophthalmoplegia, which has also shown subsequent improvement. Bulbar damage caused considerable speech problems. There was no cognitive impairment. Several drugs were tried without effect. Investigations of the cause, including histological examination, have been unrevealing.

Studies on the inhibition of hepatic microsomal glucuronidation by uridine nucleotides or adenosine triphosphate.

The ability of five nucleotides in the presence of excess divalent cations to inhibit UDPglucuronosyltransferase in sealed or leaky liver microsomal vesicles was studied. Two nucleotides inhibited potently while three others were weak inhibitors. At low concentration, both of the potent inhibitors, uridine tri- and diphosphates tended to inhibit more in sealed microsomal vesicles than in leaky microsomes, while the weak inhibitors, uridine diphosphate glucose and adenosine triphosphate behaved in the opposite manner and inhibited less in sealed than in leaky microsomes. At physiological concentrations of UDPglucuronic acid (0.4 mM) quite extensive inhibition of oestradiol glucuronidation could be achieved with physiological concentrations of uridine tri- or diphosphates (0.2 or 0.4 mM). In sealed or leaky microsomes, beta, gamma-methylene-interrupted uridine triphosphate, which is resistant to hydrolysis by nucleoside triphosphatase, inhibited much less than did uridine triphosphate.