We evaluated ictal 99mtechnetium hexamethyl propylene-amine-oxime single-photon emission computed tomography (SPECT) in 22 children with electroclinical features of frontal lobe epilepsy (FLE). Ictal SPECT demonstrated unilateral frontal hyperperfusion in 20 of 22 children (91%) (one lobar, two frontocentral, six dorsolateral, six frontopolar, three orbitofrontal, one medial frontal, and one insula), concordant with electroclinical lateralization in 19 of 20 (95%). Hyperperfusion was evident in the ipsilateral basal ganglia in 16 of 22 (73%) and the contralateral cerebellum in 14 of 22 children (64%). Interictal SPECT showed unilateral, localized frontal hypoperfusion concordant with electroclinical lateralization in only two of 22 children (9%). Ictal SPECT localization to the frontocentral, media frontal, or dorsolateral regions was associated with asymmetric tonic posturing, contralateral head/eye deviation, and unilateral clonic jerking (p < 0.01). Ictal SPECT localization to the frontopolar or orbitofrontal regions was associated with vocalization, hyperventilation, truncal flexion, and complex gestural automatisms (p > or = 0.05). Ictal SPECT has the potential to (1) localize seizures in patients with intractable FLE, and (2) advance understanding of the in vivo anatomico-clinical relationships of frontal lobe seizures.

Pitt, D. and Hopkins, I. (1978). Aust. Paediatr. J., 14, 182–184. A syndrome of mental retardation: wide mouth and intermittent overbreathing. Two retarded children are described, with similar facies, consisting of an unusually wide mouth and palate, thick fleshy lips, and a broad‐beaked nose. They also had an unusual respiratory rhythm, clubbing of the fingers, and abnormal electroencephalograms. This may represent a new syndrome.

Sixty-three children with new-onset temporal lobe epilepsy (TLE) underwent extensive clinical, EEG, and neuroimaging investigation as part of a prospective, community-based cohort study of the natural history of TLE in childhood. Complex partial seizures occurred in 94% of the children, and tonic-clonic seizures occurred in 14%. Developmental, behavioral, or learning problems were present in 38%. Eighteen children (29%) had a significant illness/event prior to the onset of TLE, including febrile status epilepticus in seven, meningitis in four, respiratory arrest in two, and head injury in one. Magnetic resonance imaging or computed tomography revealed structural abnormalities of the temporal lobe in 24 children (38%), including hippocampal sclerosis (HS) in 13 and tumor in eight. There was a strong association between HS and a history of significant illness/event prior to the onset of TLE (p < 0.001). Analysis of past history and neuroimaging findings led us to propose three etiologically defined subgroups of TLE; developmental TLE (10 children with long-standing, nonprogressive temporal lobe tumors and malformations), TLE with HS/significant antecedents (18 children with HS or a history of a significant illness/event), and cryptogenic TLE (34 children with normal neuroimaging findings and no significant past history). Etiologic differences between children with new-onset TLE may confer prognostic information that will be useful for counselling families and planning treatment.

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We reviewed the outcome of corpus callosal section in 64 adult and pediatric patients to identify factors associated with a good outcome: 48% of patients had a favorable outcome for overall seizure frequency. Improvement was noted in several seizure types and was most likely for drop attacks, particularly in the setting of a unilateral focal cerebral lesion or a true generalized epilepsy of Lennox-Gastaut type. Poor outcomes for drop attacks were more likely if there was associated severe intellectual handicap or bilateral independent spikes on interictal EEG. Complex partial seizures (CPS), most commonly of frontal lobe origin, also responded favorably. The complications of callosal section were usually mild and transient. New focal seizures occurred in only 2 patients and were not as frequent or disabling as preoperative seizures types. A worthwhile improvement in seizure outcome was achieved by completion of the callosotomy in 6 of 10 patients with unsatisfactory results from anterior callosotomy.

Paroxysmal tonic upgaze (PTU) of childhood is a distinctive neuro-ophthalmological syndrome of unknown etiology and pathogenesis that is characterized by episodes of sustained upward deviation of the eyes, often with incomplete downward saccades on attempted downgaze. It is generally regarded as having a benign outcome. We observed 16 children with PTU, from 10 months to 11 years from onset (mean, 5.4 years), to study the natural history and possible etiology. Five cases were from two unrelated families. Onset of PTU occurred either during or after an intercurrent infection or vaccination in 5 children. No antecedent was identifiable in the rest. PTU had completely resolved in 10 children (62%) (mean age at offset, 2.5 years), whereas 2 children intermittently manifest a modified form of the disorder. At follow-up, 11 children (69%) had developmental delay, intellectual disability, or language delay and 9 (56%) had ocular motility problems other than PTU. Only 3 children (19%) had normal development and neurological findings. PTU is a heterogeneous syndrome with respect to associations and outcome and may simply be an age-dependent manifestation of a variety of disorders affecting corticomesencephalic control of vertical eye movement. This disorder may be an early sign of more widespread neurological dysfunction.

SUMMARY The clinical features and outcome of idiopathic acute transverse myelopathy were reviewed for 21 children aged between seven months and 14 years. Pain, most commonly in the back, was the initial symptom for 12 patients; for another six it was weakness and for two urinary retention. All patients had weakness of the legs, and 11 had arm weakness as well. Five patients had very acute onset of severe weakness and were unable to walk within three hours of onset of symptoms. Two children made no significant recovery; for the remainder onset of recovery was evident within two to 17 days. 12 patients later were normal or had only minimal neurological deficit, but nine had a poor outcome with major disturbance of motor or sphincter function. Only one of the five with very acute onset had a good outcome. Over–all, the prognosis after acute transverse myelopathy in childhood is a little better than that reported for adults. RÉSUMÉ Myélopathie transverse aigue de l'enfance Les caractéristiques cliniques et le devenir de la myélopathie transverse aigue myélopathique ont été précisées chez 21 enfants âgés de sept mois á 14 ans. La douleur, le plus fréquemment au niveau du dos, a été le symptome initial chez 12 sujets. Ce fut une faiblesse chez six sujets et une rétention urinaire chez deux sujets. Tous les sujets présentaient des faiblesses des jambes, associées chez 11 d'entre eux á des faiblesses des bras. Un début par faiblesse trés sévere de caractére trés aigu est survenu chez cinq sujets qui ont été incapables de marcher dans les trois heures du début des Symptômes. II n'y a pas eu de ré cupération significative chez deux enfants; pour les autres, le début de la récupé ration a étéévident aprés deux á 17 jours. Chez 12 sujets, le devenir ultérieur a été normal ou marqué seulement par un déficit neurologique minime, mais l'évolution a été mauvaise dans neuf cas avec troubles graves des fonctions motrices ou sphinctériennes. Un seul parmi les cinq enfants avec début brutal a eu un bon devenir. Globalement, le pronostic de la myélopathie transverse de I'enfance est un peu meilleur que ce qui a été rapporté chez les adultes. ZUSAMMENFASSUNG Akute Querschnittsmyelopathie im Kindesalter Bei 21 Kindern im Alter zwischen sieben Monaten und 14 Jahren wurden die klinischen Merkmale und Endbefunde der akuten idiopathischen Querschnittsmyelopathie zusammengestellt. Bei 12 Patienten war der Schmerz, meistens im Rücken, das erste Symptom: weitere sechs gaben Schwache und zwei eine Harnverhaltung an. Alle Patienten gaben Schwache in den Beine an und 11 klagten zusätzlich über Schwäche in den Armen. Fünf Patienten hatten einen sehr akuten Beginn mit hochgradiger Schwäche und konnten innerhalb von drei Stunden nicht mehr laufen. Zwei Kinder hatten sich nicht wesentlich gebessert; bei den restlichen stelite sich nach zwei bis 17 Tagen eine Besserung ein. 12 Patienten waren später gesund und hatten nur geringfügige neurologische Befunde, aber neun hatten ein schlechtes Endergebnis mit einer deutlichen Störung der motorischen‐oder...