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CLINICA SANTA

CATALINA

HPCMNO

DEMENCIAS EN EL NIO
DR. JOS JESS COLN PREZ
NEUROLGO PEDIATRA
DIPLOMADO EN TDAH
HISTORY
The evolution of the science of cognition has
its beginning in Philosophy. Aristotle (BC 384-
322) in his Logic and Psychology and
Philosophy of Mind portrays cognitive
domains pertaining to memory, perception,
and mental imagery.
COGNITION
The term Cognition has its origin from the Latin
word cognosco(con 'with'; gnsc 'know), and
means 'to conceptualize' or 'to recognize'.
It refers to the mental processes (thinking,
learning, remembering, abstraction, judgment,
problem solving, language, imagination,
perception, planning, execution) involved in
acquiring knowledge and awareness about self
and environment.
EL CEREBRO EN DECLIVE
DEFINITION
Dementia in childhood
Involves the progressive loss of already
attained developmental skills.
This usually reduces the childs subsequent
developmental trajectory and generally, but
not always, leads to death.
The condition is not explicable in terms of
acute drug toxicity or the other causes of
delirium.
Delirium Aetiology
Hypoxia
Hypoglycaemia
Systemic infection
CNS infection
Polypharmacy, especially with anticholinergics,
anticonvulsants, and psychotropics
Substance abuse or withdrawal
Accidental and non-accidental overdose
Medicationssteroids, anticholinergics, lithium, all
neurotropics and psychotropics, and anticholinesterases
Inflammation
Radiation
Raised or fluctuating intracranial pressures
CNS trauma
Mitochondrial disorder
High
EPIDEMIOLOGY
The incidence is unknown but
likely to be between 5 and 15 per
100 000.
AETIOLOGY

There are over 600 possible


diseases, syndromes and
conditions that are known to
be able to give rise to
dementia in childhood.
CLINICAL PRESENTATION

The varieties of presentation


will depend upon the
developmental stage of the
child and the nature of the
neuropathology.
In infancy
Loss of fixing and following with the eyes.
Alteration in the quality of cry.
might be the only signs of regression.
Toddlers
May lose already accomplished skills of walking
and speech and develop an autistic picture.
The school age
Child may develop visual difficulties, learning and
behaviour problems, loss of writing skills and a
drop off in school function with social
withdrawal.
Seizures are a possible presenting feature at any
age.
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ASSESSMENT AND INVESTIGATION
Once the serious possibility of dementia has
been invoked it
Is critical to involve a paediatric neurologist
with whom investigation can be planned
strategically.
Most of the time, dementia as a differential
diagnosis will be in the less likely but needs
to be considered and excluded category.
ASSESSMENT AND INVESTIGATION
Six key questions need to be answered
1. Is there a definite history of sustained skill
loss or merely a short term?
2. Are there any neurological features to the
presentation such as seizures, ataxia,
deterioration in vision or weakness which might
give a clue as to the nature of the disorder?
3. Is there a family history that is suggestive of
one of the dementing conditions?
ASSESSMENT AND INVESTIGATION
4. How might the stage of development affect
the presentation of the disorder?
5. What is the current impact on the family of
the disorder and the threat associated with the
disorder?
6. Are any of the siblings similarly affected?
ALL CHILDREN WITH SUSPECTED
DEMENTIA
Should have full neurological
examination.
Neurological investigations.
Neuropsychometric
assessment.
DIFFERENTIAL DIAGNOSIS
Severe and profound depression
Pervasive refusal syndrome
Cerebral tumours
Schizophrenia
Side effects of medication
Somatoform disorder
Factitious disorder.
TREATMENT

Ascertaining the aetiology


Treating the psychiatric
symptoms
Supporting the family
Appropriate educational
placement.
REFERENCES
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2 Hepper PG (1988) Adaptive fetal learning: prenatal exposure to garlic affects
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3 Kim YS, Leventhal BL, Koh Y, Fombonne E, Laska E, et al. (2011) Prevalence of autism
spectrum disorders in a total population sample. Am J Psychiatry 168: 904-912.
4 Sanders SJ, Murtha MT, Gupta AR, Murdoch JD, Raubeson MJ, et al. (2012) De novo
mutations revealed by whole-exome sequencing are strongly associated with autism.
Nature 485: 237-241.
5 Shevell MI, Majnemer A, Rosenbaum P, Abrahamowicz M (2000b)
Etiologic yield of subspecialists evaluation of young children with global
developmental delay. J Pediatr 136: 593-8.
6 Flavell JH (1971) First discussant's comments: What is memory
development the development of? Human Dev 14: 272-8.
7 Hardan AY, Muddasani S, Vemulapalli M, Keshavan MS, Minshew NJ (2006) An MRI
study of increased cortical thickness in autism. Am J Psychiatry 163: 1290-2
8 Michaelson JJ, Shi Y, Gujral M, Zheng H, Malhotra D, et al. (2012)
Whole-genome sequencing in autism identifies hot spots for de novo
germline mutation. Cell 151: 1431-1442.
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