Monique Gingold

Age-Related Changes in the Relative Growth of the Posterior Fossa:

We have established a normative data set for the relative size of the structures of the midline posterior fossa from birth to 90 years old. Data were obtained from morphometric analysis of midsagittal magnetic resonance imaging scans of the brain utilizing a simple image analysis system. There are several significant changes in the size of these structures with an increase in chronologic age. The relative size of the cisterna magna decreases with age. Anterior cerebellar vermal lobules (I through V) appear to grow more rapidly than the rest of the cerebellum. Other, less significant, trends include a decrease in the overall size of the cerebellum, superior posterior vermal lobules (VI and VII) and inferior posterior lobule (VIII) with an increase in age. It is, therefore, necessary to use age-standardized normative data when making morphometric correlations with clinical disorders. (J Child Neurol 1991;6:15-19).

ALEXANDER'S DISEASE: UNIQUE PRESENTATION

Subacute necrotizing encephalomyelopathy (Leigh syndrome) refers to a nebulous disease entity characterized by lactic acidosis, a wide variety a clinical manifestations, and a consistent conglomeration of pathlogic findings. Several abnormalities in metabolism have been delineated in association with Leigh syndrome, but many cases have no identified metabolic abnormality. We report a case that clinically, metabolically, and neuroradiologically appeared to be Leigh syndrome. In addition, our patient exhibited other unusual clinical findings, including ocular motility abnormalities. Neuropathologically, however, the diagnosis of Alexanders disease was confirmed. A review of the literature failed to find other cases of Alexanders disease reported with the metabolic abnormalities and clinical manifestations with which our patient presented (J Child Neurol 1999;14:325-336).

The rise and fall of the plantar response in infancy

To determine when the plantar response becomes reliably flexor in infants, 169 infants underwent serial evaluation from 2 weeks to 12 months of age during routine well-child visits. The plantar response, elicited in a standardized fashion, was extensor in 95.5%, 64.8%, 10.9%, 0.7%, and 0% of the infants at 2, 4, 6, 9, and 12 months, respectively. The plantar response becomes predominately flexor by 6 months of age in normal infants. This maturation of response correlates closely with that of other infantile reflexes.

Stroke Due to a Traumatic Vertebral Artery Dissection in a Girl

pendently with eyes open and was able to walk only with support. A complete blood count, serum lactic acid, routine chemistries, and echocardiography were normal. Her coagulation profile was normal, including platelet counts, prothrombin time, antithrombin III, protein C, and antilupus anticoagulant. A cranial CT scan showed mild hydrocephalus and a large, lucent inferior vermian cerebellar lesion that extended

Childhood Presentation of Idiopathic Epidural Lipomatosis: A Case Report With Magnetic Resonance Imaging and Pathologic Confirmation:

The causes underlying the cerebral pathology in homocystinuria are controversial.3,4 In fact, Chou and Waisman5 described extensive spongy degeneration and demyelination of the white matter of cerebrum, cerebellum, brain stem, and basal ganglia, and even in cervical spinal cord. Gaull et all also reported neuronal loss in the cerebral cortex and hippocampus in their patient. Interestingly, no vascular changes were noted in these studies. Whether they are of vascular or metabolic origin, the hyperintense small areas in the basal ganglia of our patient, which increased in number and intensity with time, were seemingly consistent with his recently developed extrapyramidal findings. We also believe that the appearance of extrapyramidal findings-an awkward posture and bradykinesia, as well as gesture loss-were manifestations of a dystonia, which was mentioned earlier in association homocystinuria,7-1° but also of secondary parkinsonism that developed in association with homocystinuria. His response to levodopa and the late appearance of other extrapyramidal findings were believed to support our proposal. Sabiha Keskin, MD Fatma Yurdakul, MD Pediatrics Department Istanbul University Cerrahpasa Faculty of Medicine Istanbul, Turkiye

The Plantar Response in Normal Newborn Infants

The neonatal plantar response has been reported as extensor in 90% of newborns and flexor in 93% of newborns, leading to uncertainty about its reliability and significance. To determine the normal neonatal plantar response we examined 349 healthy newborn infants, >32 weeks gestation within 24 hours of birth. A supramaximal noxious stimulus was applied in a standardized manner to the lateral plantar surface of each foot. The plantar response was extensor in 90%, equivocal in 7%, and flexor in 3%. With proper physiologic technique, the normal neonatal plantar response is extensor.

Melatonin to Treat Fragmented Sleep Needs Study

nerve compound muscle action potential was 0.4 mV, the mean median nerve F response in four of the six patients (absent in two) was 33 msec, while two patients had evidence of conduction block prior to intravenous immunoglobulin treatment. All patients made a rapid recovery clinically and neurophysiologically (mean time to improve by at least one grade on the functional GuillainBarr6 syndrome scale, 2.9 days; follow-up period, 10.5 months). The only intravenous immunoglobulin-related side effect noted was a headache in a 14-year-old male patient, which subsided promptly after the use of paracetamol. We agree with Wong and Glauserl that serious complications from the use of intravenous immunoglobulin are rare in children with Guillain-Barr6 syndrome and are related to increased serum viscosity. The safety and efficacy of this treatment remains to be determined by randomized control studies.

Hypoxia-induced CHAP syndrome.

have been used to enhance GABAergic inhibitory activity.9 Presynaptic GABAB receptors, capable of inhibiting release of a variety of neurotransmitters, have been found in many areas of the central nervous system.9 Ohisalo et all’ and Airaksinen and Leinoll have shown a decrease in cerebrospinal fluid GABA in patients with Unverricht-Lundborg disease. It is possible that baclofen in this patient is working as a GABA mimetic substitute in a patient who is GABA deficient. It is not clear why some patients such as A.B. do not respond well to valproic acid and clonazepam, which are GABAergic drugs. It is interesting to note that baclofen often causes sedation in an adult at about 40

Altered consciousness and shock in a malnourished child

A 3-year 9-month-old child presented unresponsive, dehydrated, and in shock, a consequence of child neglect, abuse, and starvation. This scenario provides the vehicle for a discussion of three problems which can be precipitated by child neglect, specifically kwashiorkor, central pontine myelinolysis, and intellectual repercussions of malnutrition.

Book Review: Pediatric Neuroimaging : A Casebook Approach, by Charles L. Truwit and Todd E. Lempert. Published in 1991 by DPS Press, Piedmont, California; 290 pages,

can Association of Mental Retardation in 1876 to the present. The volume is hardbound, clearly printed with useful and functional tables, and is well done-I found only one typographical error in my reading. Although I did not find the style of writing particularly charming, the organization of the book is such that the important points are repeated several times so that by the time you finish you are unable to forget the message. There are many illustrative cases presented throughout the text to clearly demonstrate appropriate application of the various aspects of the definition or classification. In fact, throughout the book, the reader is told what is to be learned, given the information and examples, and then told what has just been learned. This technique, despite its proven effectiveness as an educational method, is somewhat slow for a reader who is already partially familiar with the material. Although the volume will not add much to the medical knowledge base of most pediatric neurologists who deal with patients with mental retardation, it is nevertheless an important document for all health-related professionals who are involved in the delivery of services to these patients. It will be particularly useful to the inexperienced physician who may not understand or be familiar with the functional aspects of the definition of mental retardation. As the emphasis on IQ shifts to an emphasis on functional adaptation and the delineation of necessary support services, the physician needs to know the basics of the terminology and the methods of testing and evaluation of the patient if he/she is to operate efficiently as a member of the patient management team. To this end it would be a good idea for all physicians, as well as other members of this &dquo;manage-