LYME NEUROBORRELIOSIS AND ALICE IN WONDERLAND SYNDROME

Pediatric neurologists at the Floating Hospital, Tufts University, Boston, MA report a 7-year-old boy with Alice in Wonderland syndrome associated with Lyme disease.

expanded like a telescope after eating the cake. Distortions of form, size, movement, color, or sense of time are characteristic of metamorphopsia that may occur as a sensory aura during migraine or frontal lobe epilepsy. A PubMed literature search lists reports of several infectious agents associated with Alice in Wonderland syndrome, including Epstein-Barr virus, varicella, coxsackievirus B1, H1N1 influenza virus, and infectious mononucleosis. Lyme disease in the Tufts' case presents with visual and other illusions without systemic manifestations of neuroborreliosis. Alice in Wonderland syndrome is a benign disorder of childhood caused most frequently by migraine or Epstein-Barr virus infection. (Losada-Del Pozo R et al. Rev Neurol 2011;53(11):641-648).

HEMORRHAGE RISK OF CAVERNOUS MALFORMATION
Researchers at the Mayo Clinic, Rochester, MN reviewed the records and radiograph data of 292 patients (47.3% male) seen between 1989 and 1999 with a diagnosis of intracerebral cavernous malformation (ICM). The mean age at diagnosis was 45.8 years (range 3.5-88.9 years). Seventy-four patients presented with hemorrhage, 108 with seizure or focal deficit (symptoms not related to hemorrhage), and 110 were asymptomatic. The overall annual rate of hemorrhage at follow-up in the patients grouped according to presenting symptoms was 6.19%, 2.18%, and 0.33%, respectively. The median length of follow-up was 7.3 years (range 0-25 years); 68 patients underwent surgical excision. Patients who presented initially with symptomatic hemorrhage were at higher risk for future hemorrhage (p<0.001), and hemorrhage risk decreased with time; 81% were free of prospective hemorrhage at 10.6 years. Of 32 patients with prospective symptomatic hemorrhage, 19 (59%) initially presented with hemorrhage. The annual statistically significant risk factors for prospective hemorrhage included younger age (p=0.02) (without adjustment for gender etc.), male gender (p=0.02), infratentorial location (p=0.015), initial presentation as hemorrhage (p<0.001), and multiplicity of ICMs (p=0.01). Pregnancy was not a risk factor. The median time from first to second hemorrhage was 8 months. . Mean age at presentation was 9.7 years, and mean age at operation was 11.3 years. One-quarter of all CCMs affect children.
In a study of outcome of 20 children with CCM treated in Berne, Switzerland, average age at presentation was 8.5 years (range 7 months to 16 years). Presentation was acute hemorrhage in 17 (85%), seizures in 9 (45%), focal neurologic symptoms in 5 (25%), and headache only in 3 (15%). Location was supratentorial in 15 (75%). Treatment was conservative in 10 and surgical in 10. Neurological sequelae at follow-up (0.5-10 years) occurred in 6 (30%) patients. A study of the natural history of CCM in 92 children and young adults at the University of Michigan, Ann Arbor, found the imaging prevalence of CCM increased with advancing age (p=0.002). Multiple CMs occurred in 28 (30%) patients. Thirty patients presented with hemorrhage, and the hemorrhage rate was 8% per patient-year in the symptomatic group. Symptomatic hemorrhage after long-term follow-up was associated with initial acute hemorrhage (p=0.02). A prospective, Scotland, population-based cohort study of 139 adults with CCM, radiologically validated, found the risk of recurrent intracranial hemorrhage or focal neurological deficit from a CCM is greater than the risk of a first event, greater for women than for men (p=0.01), and declines over 5 years from 9.8% in year 1 to 5.0% in year 5. (Salman R A-S et al. Lancet Neurol March 2012;11:217-224). The increased risk in women in this study of adults only is different from that in the Mayo Clinic study showing a preponderant risk in males.

SECKEL SYNDROME WITH HOLOPROSENCEPHALY
A case of Seckel syndrome (SS) accompanied by semilobar holoprosencephaly and arthrogryposis is reported from Erciyes University, Kayseri, Turkey. Seckel syndrome is a rare autosomal recessive disorder characterized by prenatal and postnatal growth retardation, microcephaly, and "bird-like" face with prominent, beak-like nose and micrognathia. A 1-day-old female newborn was admitted with dysmorphic features and feeding difficulties. The parents were consanguineous. In addition to typical features of SS, the baby had arthrogryposis, and cranial MRI showed semilobar holoprosencephaly, lissencephaly/pachygyria, dilated occipital horn of the lateral ventricle, hypoplasia of the frontal horn, non-cleavage of the basal ganglia and frontal lobe, and dysgenesis of the corpus callosum. Neuronal migration disorders should be

TRACTOGRAPHY NEUROANATOMICAL STUDY OF DYSLEXIA
Researchers at Catholic University of Leuven, Belgium used diffusion tensor imaging tractography, a structural MRI technique, to assess the integrity of white matter tracts involved in reading. Group comparisons of 20 adults with dyslexia and 20 typical reading adults showed a significantly reduced fractional anisotropy (i.e. an index of the amount of anisotropy) in the left arcuate fasciculus of dyslexics, reflecting reduced myelination. Performance on phoneme awareness and speech perception was specifically related to the integrity of the left arcuate fasciculus (dorsal phonological route underlying grapheme-phoneme decoding), whereas orthographic (direct word) processing was related to fractional anisotropy values in the left inferior fronto-occipital fasciculus (ventral orthographic route). Structural anomalies found in the left arcuate fasciculus of dyslexics corroborate the hypothesis of dyslexia as a disorder of network connections. (Vandermosten M et al. Brain 2012;135:935-948