Cognitive Decline in Childhood or Young Adulthood Flashcards
what characterizes cognitive decline in childhood/young adulthood?
cognitive deterioration (dementia)
what are the patterns of neuropsychological deterioration? (4)
- normally develop, development slows, plateaus, then declines
- normal development slows (but no loss), begins to lag behind peers
- acute and rapid decline followed by no further development (following TBI)
- acute and rapid decline followed by slow but ‘normal’ cognitive development (e.g., TBI)
what are the characteristics of lysosomal storage diseases? (2)
- deficiencies in lysosomal enzymes
- accumulation of unwanted proteins in lysosomes - cellular ballooning and then death
what are the lysosomal storage diseases? (4)
- Tay-Sachs disease: early infancy, mortality by 2-4 yrs
- Niemann-Pick disease: infancy (ranges), mortality by midage
- metachromatic leukodystrophy: demyelination of CNS; motor and cognitive deficits; adolescent onset
- Hurler syndrome: late infancy; macrocephaly, etc; mortality by 10 yrs
what causes neuronal ceroid lipofuscinosis disorders?
lipopigment accumulation
what are the neuronal ceroid lipofuscinosis disorders? (3)
- Jansky-Bielschowsky disease: late infancy onset; mortality by 11
- Batten disease: onset 4-9 yrs; vision loss; mortality by late teens or 20s
- Kuf’s disease: onset 15-25 yrs; myoclonic epilepsy or personality changes and dementia
what causes aminoacidopathies?
aminoacidic disruptions
what are the characteristics of phenylketonuria? (5)
- autosomal recessive
- variability in severity and intellectual decline
- can be treated with diet
- if caught early, patient appears fairly cognitively intact with deficits restricted to attention/executive functioning, word retrieval, memory problems
- if untreated leads to intellectual decline and seizures
what are the characteristics of acute disseminated encephalomyelitis? (4)
- occurs following an infection (viral or bacterial)
- recovery can be complete, but not for all. Adult onset more likely to recover
- most often occurs before 10 yrs old
- acute symptoms: flu like, seizures, stiff neck
what are the characteristics of Rassmussen’s Encephalopathy? (5)
- autoimmune disease
- unilateral symptoms, move bilaterally
- hemiparesis
- progressive seizures
- can have unilateral neuropsychological impairments
what are the characteristics of vitamin B12 deficiency? (2)
- most commonly due to poor absorption rather than lack of exposure
- intellectual disability can occur; general cognitive decline
what are the symptoms associated with vitamin B12 deficiency in infants?
failure to thrive (not gaining weight or meeting developmental milestones)
what are the symptoms associated with vitamin B12 deficiency in childhood? (3)
- weakness
- sore tongue
- parethesias
what are the symptoms associated with vitamin B12 deficiency in adolescence? (4)
- weakness/fatigue
- nausea
- constipation
- paresthesias of toes and fingers
what are the characteristics of Galactosemia? (4)
- cannot metabolize lactose
- occurs from birth
- jaundice and anorexia are common; failure to thrive
- lactose free diet can help but deficits still occur
what are the characteristics of Rett syndrome? (2)
- symptoms become apparent by 18 months
- normal development followed by a reversal in cognitive and motor function
what are the 2 main categories of hydrocephalus?
congenital: problems with brain development
acquired: something causes the problem
what can cause hydrocephalus? (3)
- obstruction of CSF
- insufficient absorption of CSF
- excessive secretion of CSF
what are the 2 main types of hydrocephalus?
communicating (absorption) and noncommunicating (obstruction)
what is a common treatment option for hydrocephalus?
ventricular-peritoneal shunt
what are the characteristics of pseudotumor cerebri (idiopathic intracranial hypertension? (3)
- increased intercranial pressure
- headache, fatigue, blurred vision, nausea/vomiting or diplopia, visual field loss
- optic nerve damage might occur even if controlled
what are the treatment options for idiopathic intracranial hypertension? (3)
- diuretics
- weight loss
- shunting
what are the characteristics of sickle cell disease? (4)
- autosomal recessive
- red blood cells clump together
- multisystemic involvement
- waxing and waning course
what are the characteristics of HIV associated progressive encephalopathy (HPE)? (2)
- exposure to HIV at birth or in breastmilk
- learning disorders, neuropsychological impairments if left untreated
what are the symptoms associated with infant exposure to HPE?
developmental delays
what are the symptoms associated with childhood exposure to HPE?
slowed developmental milestone attainment
what are the characteristics of cerebral autosomal dominant arteriopathy with subcortical infarct and leukoenceohalopathy (CADASIL)? (3)
- early adulthood onset
- migraines (leads to strokes and dementia)
- subcortical dementia profile
what are the characteristics of Wilson’s disease (hepatolenticular degeneration)? (5)
- autosomal recessive disorder
- copper accumulation
- onset ages 10-14 for hepatic symptoms; 20s for neurological symptoms
- jaundice, green-brown ring around cornea
- motor symptoms: tremors, complex attention/EF. memory, visuoconstructional neurpsychological deficits
