Abstract
Background-Historical-notes-purpose: PDDs are-gradable-constellations of neurolobiologicaldisorders with abbreviated-life -expectancies, including CDD, its anecdotal-extreme-rarity made it initially unscientifically-acknowledged-Rettssyndrome(RS)-affects only females. Previously most girls diagnosed asCDD, retrospectively had RS. In addition to Autism-spectrum-disorders(ASD),- Aspergers syndrome(AS). An unequivocal initial diagnosis of a specific PDD was unusual, because of their striking overlapping features. Although features were spectral-overlapping, they were aetiologicallyprognostically distinct. Accurate-diagnosis is crucial because interventions-differ. The compositeroles of prematurities-birth- traumas-genetic factors-amyloid-interleukins-1-beta-endorphinsenvironmental- toxigenic- autoimmunityrelated disruption of neuronal-transmissionsneuro- pathological associations with TSC, -intrauterineTORCHES-viral-exanthematousrelated- SSPE-associated-symptomatic-seizures which suggests the-diagnosis of otherwise-similarbut- very -therapeutically-responsive-Landau- Kleffner-syndrome(LKS) especially in boys-the influence of other-infective-processes suggests the benefits of an apposite index of suspicion for a timely diagnosis/-specific-interventions. Ongoing research although undermined by the relative-rarity of these disorders-suggests that overall, PDDswere underdiagnosed-misdiagnosed. However the putative-roles of infective-antecedents suggests that impacts could be epidemio-geographically distinct.
A delineation of categorical-figures from a definedsetting- could be scientifically -illuminating with implications for research-directions.
Methods-case-definitions-interventions: Descriptors- pre-morbid-post morbid- historical notes were retrieved from teachers-care giversparents of pervasive-developmental-educationalsocial- emotional- physical aspects of cases-relevant to the theme. Putatively-universally-acceptablesymptomatic- syndrome-driven-classifiers with batteries of developmental-tests were applied for
Case-definitions. Interventions were structuredbehavioural- educational-speech-language therapies-social skills-development-sensoryintegration- occupational therapies-hypnoticsedatives- antiepileptics-tranqulizers. Rewards to reinforce desirable behaviours-discouraginguntoward- behaviours-Cares in respite-homes were proffered.
Results: Of the(n=13)cases-(n=8) were males.
Mean-age at diagnosis =89.08months.
On the basis of epidemiological-historicald emo graphics-temporality-chronology-interventional-responsiveness of symptomatologies, the diagnosis of compatibleearly infantile-autism-spectrum-disorders was suggested in(n=1), compatible high functioning autism.(n=1)CDD(n=1), Retts-syndrome(n=1). PPD-NOS (n=9)-no compatible- AS. Unprovoked- Remote-symptomatic-seizures were frequent(n=11) Other associations were primary enuresis-socialdeviance- defective-self-regulation-derangedsleep- wake cycles-acute-psychosis-inordinatetemper- tantrums-non-specific-abdominal-painshallucinations- recurrent- febrile-responses-irritablebowel- syndromes-multiphasic-hallucinations-poor co-operation-attention span-concentrations/- syncope.
Conclusions: In PPD, because outcomes were improved with earlier interventions, an opportunistic screening for developmental-defects should be undertaken in every well baby-child visits. Although symptomatically similar,- patterns of onset-courseoutcomes were categorically distinct.A consistentpositive approach results in improved outcomes. These figures could direct-diagnostic-therapeuticapproaches/- relevant interventions.
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Onyekwelu, E. 570 An Annotated Compilation of Childhood Pervasive Disintegrative Spectrum Disorders(PDD) Check Lists with Emphasis on Differential Algorithmic, Diagnostic Therapeutic- Interventional Approach. Pediatr Res 68 (Suppl 1), 292 (2010). https://doi.org/10.1203/00006450-201011001-00570
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DOI: https://doi.org/10.1203/00006450-201011001-00570