Pediatrics (week 5): Neurology
May. 6th, 2011 11:57 am![[personal profile]](https://www.dreamwidth.org/img/silk/identity/user.png){kind=small}
liveonearthNeurology
Most imp to distinguish btw epilepsy and siezures
kids won't have a hx to help with dx
anyone can have a few seizures without having epilepsy
SEIZURES
4-6% of kids have at least 1 seizure by age 16
recent fever mc reason in small children
ETIO: thermogenic, metabolic, head trauma hx, forgotten anti-seiz meds, other drug or toxin
newborns may seize dt hypoxia, birth trauma-->intracranial bleeds, siezures usu self limiting
hx of previous acute illness, stroke, birth asphyxia, cerebral palsy, meningitis or other cns infx
metabolic encephalopathy, hypoglycemia, new diabetes
fainting, hyperventilation, night terrors, breath holding, all causes of anoxia, migraine, drugs
mc in newborns dt metabolic
hypoxia, ischemia more in premies
hypoglycemia more if mom is undxd DM insulin dependent(50 is not abn BS for baby
infx can cause hypoglycemia, HSV is very bad in neonates and causes siezures by itself
(4 drops colostrum will raise babies blood sug to normal)
or focal nervous sys problem
shaking can cause hemorrhage ddx shaken baby syndrome
RISKS for recurrent nonepileptic seizures::: hx of prev siez febrile or otherwise, recent withdrawal of anticonvulsant meds, major growth spurt without meds adjustment, brain tumor, neurodegenerative dz, hx of neuro insult, post vaccination, infx mc or neuro disorder
Tx: if febrile and known hx of febrile seizures: tylenol and sleep, but consider ER always esp if doc can't put eyes and hands on kid, or if first siezure and cause not sure, or if parents are freaked even if you're not
EPILEPSY
altered LOC, motor activity, change in sensation, autonomic fx
accelerated rate of synchronicity of nervous impulses??
*need good pediatric neurologist for referral list!
partial/focal seizure, single hemisphere*
simple partial has no impairment of consciousness
complex partial has altered LOC, may start as simple
both can end with grand mal convulsions
generalized siezures starts in both hemispheres symmetrically and simultaneously (???)
consciousness disturbed immediately
petit mal incl absence seizure is nonconvulsive, generalized, consciousness disturbed, no convulse
Hx: aura? sequence of events, general appearance, triggers?, duration is difficult to peg
Hx: post ictal state?
TX: refer to neurologist, need EEG while seizing for conclusive dx
Tx: picking meds is a progressive process, try one if not work try another
Tx: NDs may help manage SEs, don't try to manage grand mal s neurologist, lawsuit waiting to happen
BENIGN SLEEP MYOCLONUS
rhythmic jerking while sleeping or v drowsy
mc in kids under 3
sim to adult twitch when falling asleep and just as b9
may last longer than typical siezure
strike difference btw this and siezure: goes away with waking, doesn't happen while awake
EEG always normal
HEADACHES
common
ddx: trauma, tension, migraine, acute illness, trigeminal neuralgia
migraines more in kids than you'd think, often BL, dull, throbbing, aura, n/v, etc, relief by sleep
common migraine is mc headache in children and adolescents, stress exacerbates
regular Monday h/a: headache is not the issue, school is
complicated migraine has neuro changes same as in adults
most start at puberty onset, early would be age 8-10
some want off drugs, others circle around, remind pts that there are options
hx: concussions? football? bike? falls?
q: what age OK to do headers in soccer? not so dangerous dt no mo change in head, no direct answer
ddx: dental infx? sinusitis, tumor, hydrocephalus, undxd visual prob
vision: "kids don't know not everybody sees blurry", elementary school screenings are crap,
need specialist screening if any suspicion, fhx
dx: based on hx, acute, chronic, repetitive, location, onset, duration, associated, response to tx
pe: eent, brudzinski's, jaw, mouth, face, ears, neuro exam usu n with migraine or tension
consider referral: if can't find prob and nothing helps
LEARNING DISORDERS, disabilities
common
terms used when performance is substantially below expectation for age and intelligence
many forms that don't fit categories
dyslexia
parents come with concerns of their own or from school
they may not want a dx or label
is there a problem? behind in school?
loss of previously acquired skills is bad sign
misbehavior mb willful
outcast kid may become quirky but high performing adult
many kids have certain areas with deficits and other areas where they are strong
if you don't tx the deficit area, deficits may become general
ddx: hearing loss dt ear infx? vision problem? bipolar is overdxd, substance abuse?
ddx: adhd, odd, borderline mental retardation
dx: refer to developmental pediatrician who coordinates care with specialists
ddx: lead poisoning? anemia? meds? antiepileptics, bronchodilators-->wired
ddx: hypothyroid, absence and partial complex siezures can be missed by teachers
ddx: malnutrit, anorexia nervosa, chronic illness, poor growth
ddx: psychosoc, child abuse, bullying account for much dread of school
tx: through 4.5-5 can refer to early intervention program (in OR I think)
tx: school age you can use school system
but parents don't like school to own the dx, would rather pay to have the information and power
tx: REFER if siezure or neurodegen, depression or another psych dx, physical dysmorphology, possible genetic disorder, hearing or visual impairment, neuro ped doc
SLEEP DISORDERS
APNEA
restless sleep
onset age 2-6
"children should not snore"
consider: allergies, enlarged "ginormous" tonsils, gerd, obesity, poor growth
sx: fatigue
consider: refer to HEENT doc
tx: she has recommended removal of tonsils, worked wonders in 4-5 kids in 15 years in her practice
SLEEPWALKING
abrupt onset, benign
common in school age, 15-30% do it at least once, fewer do it often
bumbling, rumbling, walkers usu return to bed
resolves by adolescence
NIGHT TERRORS
very disturbing to parents too
child screams, sits up, sweats, breathes hard, glassy eyes, inconsolable
will sleep again after a while and won't remember
resolves with age
mb at a regular interval after bedtime
tx: find trigger, most common is being overtired
homeop: calc carb, calms forte
CEREBRAL PALSY
nonprogressive motor impairment dt dmg to developing brain
50% in premies
etio usu unknown, usu dxd at birth or early in infancy
sx: toe walking, stuttering/dysfluency, motor probs, airway, sensory and cognitive probs mb present
STUTTERING
if child doesn't blink or have physical sx with stutter, don't worry, let child finish thought, don't tell child what to do, don't ask a lot of questions or add stress, REFER if it lasts over 3-4 months, occurs in every sentence, if child is disturbed by stutter, if FHx, if sx of physical struggle while trying to speak or child starts avoiding speech
MUSCULAR DYSTROPHIES
Duchennes 1/3500 live male births, x linked
many other types
waddling gait, lordosis, poor prog, die in 20's
change in existing skills-->refer
ATAXIA AND CLUMSINESS
lack of coordination usudt cerebellar dysfx
etio: metab, tumor, infx, intox, ear infx, thyroid, head trauma
chronic: fhx or tumor
kids are clumsy, if it comes and goes don't worry so much, more with growth spurts
******************************
I. seizures
A. epilepsy vs. seizures
1. anyone can have a seizure without having epilepsy
a. 4-6% of kids will have at least one seizure by age 16
1. careful hx will often indicate cause
b. DDX of non-epileptic seizures
i. idiopathic
ii. febrile
iii. manifestation of previous illness or injury
iv. sxs of an acute condition
v. some oddball causes of non-epileptic seizures
c. risks for recurrent non-epileptic seizures
2. in newborns, they can be due to metabolic problems or focal CNS lesions
a. intracranial causes
b. metabolic causes
c. infectious causes
B. Epilepsy
1. definition: epileptic seizures are marked by altered consciousness often accompanied by motor activity and or sensory alterations. “a transient involuntary alteration of consciousness, behavior, motor activity, sensation, or autonomic function due to an excessive rate and hypersynchronicity of neuronal discharges.” (from 5 minute ped consult)
2. seizure types
a. accurate classification of seizure type often critical to getting appropriate care
i. partial aka focal
a. simple
b. complex
ii. generalized
a. consciousness disturbed immediately
b. bilateral motor manifestations
c. grand mal is an obvious example
d. petit mal is a non-convulsive generalized seizure
3. diagnosis
a. history may give indication, but EEG is definitive
b. physical descriptions are not definitive
c. diagnosis makes a difference
C. Management and Treatment
1. epilepsy should be managed by a neurologist
2. other seizures can be managed depending on the causes
D. Benign sleep myoclonus
1. rhythmic myoclonic jerking when child is sleeping or drowsy.
2. similar to jerking when falling asleep
3. may look like a seizure
4. will not respond to anti-convulsants
II. Headaches
A. Differential diagnosis
1. head injury/ trauma
2. tension headache
4. migraine
a. onset usually gradual
b. often bilateral
c. generally dull, throbbing
d. migraine types
i. classic
ii. common
iii. complicated
5. acute illness
6. trigeminal neuralgia
7. brain tumor or other structural lesion, hydrocephalus, abscess
B. good history can be the key
1. acute vs chronic or repetitive
2. duration, location, onset, associated symptoms
3. response to therapy
4. FH, PMH
5. stress
6. preceding events
C. PE
1. vitals; funduscopic exam
2. signs of meningeal irritation
3. jaw, mouth, face, ears
4. neuro exam
D. consider referral with any chronic, recurrent, persistent HA
III. Learning Disorders
A. in the Primary care setting
1. parents will often come to you with their concerns
2. determining if there is a problem
a. falling behind expected levels of performance in school
b. loss of previously acquired skills/ deterioration of performance
3. determining possible sources
a. recent changes
b. long-term problems
c. things to watch for in the primary care setting
4. pediatrician often responsible for coordination of care among other providers
a. through preschool age, early intervention
b. school age, often through the school system
c. psych referral often needed
d. always refer if
i. hx indicates seizures or neurodegenerative disorder
ii. significant depression or other psych dx
iii. physical dysmorphology or FH might indicate genetic disorder
iv. hearing or visual impairment seems apparent
v. consider referral to neurodevelopmental specialist if no cause isolated
IV. Sleep Disorders
A. apnea
1. children should not snore as a rule
B. somnambulism
1. usually abrupt onset
2. typically see bumbling, purposeless movements, often some speaking
3. typically sleepwalkers return to bed
C. night terrors
1. common in preschool children
2. often very disturbing for parents
a. child suddenly sits up and screams
b. agitated, sweating, rapid breathing, aroused but glassy-eyed
c. child is inconsolable, unlike nightmare
d. child generally returns to sleep after a while and does not recall the event in the morning
e. resolve spontaneously as children age
V. Cerebral Palsy
A. non-progressive motor impairments that result from damage to or dysfunction of developing brain
1. various classifications, including spastic, dyskinetic, ataxic, mixed
B. Associated concerns
1. sensory impairment
2. cognitive
3. neurological
4. Musculoskeletal
5. Respiratory
6. GI
7. GU
8. dental
VI. Stuttering
A. developmental dysfluency very common in preschool kids
1. most will be transient
B. refer to speech pathologist if
1. it goes on more than a few months
2. it occurs in every sentence
3. child clearly reacts to the stutter
4. FH of persistent stuttering
5. child shows signs of physical struggle when speaking
6. stutter makes the child reluctant to speak or frustrated
VII. Motor pathologies
A. muscular dystrophies
1. hereditary disorders causing progressive weakness and muscle degeneration
a. typically slowly progressive disorders involving all muscle groups, though some more than others
b. generally have loss of muscle cells, necrosis of muscle fibers, accumulation of connective tissue between muscle fibers. Can affect smooth and cardiac muscle as well
2. multiple types, classified by clinical criteria including age of onset, muscles involved, severity of disorder, associated conditions
3. Duchenne type most commonly presents in boys aged 3-7
a. proximal muscle weakness results in waddling gait
b. toe-walking common
c. lordosis exaggerated
d. frequent falls
e. difficulty standing or climbing stairs
4. prognosis poor for Duchenne type
B. ataxia and clumsiness
1. true ataxia
a. generally associated with cerebellar dysfunction
b. cerebellum is very delicate and sensitive to disruption
c. acute ataxia
i. history of insult
intoxication
trauma
recent infection
current infection
migraine
labyrinthitis
stroke
seizure
family history
d. chronic ataxia
i. associated with
brain tumor
FH
metabolic diseases
2. clumsiness
a. often presents with concerned parents
b. common in young children
c. generally transient
d. ddx includes
visual problems
intoxication
muscular dystrophy
hypothyroidism
infection
trauma
C. Spinal Musclar Atrophy (SMA)
1. progressive weakness caused by disorder of motor neurons
a. severe type starts before 6 months
b. less severe starts by age 2
c. prognosis poor, most die of respiratory failure.
2. generally see hypotonia, poor head control, generalized weakness with early onset cases.
3. late motor developmental milestones and hypotonia cause for concern
4. genetic, FH may contribute
Most imp to distinguish btw epilepsy and siezures
kids won't have a hx to help with dx
anyone can have a few seizures without having epilepsy
SEIZURES
4-6% of kids have at least 1 seizure by age 16
recent fever mc reason in small children
ETIO: thermogenic, metabolic, head trauma hx, forgotten anti-seiz meds, other drug or toxin
newborns may seize dt hypoxia, birth trauma-->intracranial bleeds, siezures usu self limiting
hx of previous acute illness, stroke, birth asphyxia, cerebral palsy, meningitis or other cns infx
metabolic encephalopathy, hypoglycemia, new diabetes
fainting, hyperventilation, night terrors, breath holding, all causes of anoxia, migraine, drugs
mc in newborns dt metabolic
hypoxia, ischemia more in premies
hypoglycemia more if mom is undxd DM insulin dependent(50 is not abn BS for baby
infx can cause hypoglycemia, HSV is very bad in neonates and causes siezures by itself
(4 drops colostrum will raise babies blood sug to normal)
or focal nervous sys problem
shaking can cause hemorrhage ddx shaken baby syndrome
RISKS for recurrent nonepileptic seizures::: hx of prev siez febrile or otherwise, recent withdrawal of anticonvulsant meds, major growth spurt without meds adjustment, brain tumor, neurodegenerative dz, hx of neuro insult, post vaccination, infx mc or neuro disorder
Tx: if febrile and known hx of febrile seizures: tylenol and sleep, but consider ER always esp if doc can't put eyes and hands on kid, or if first siezure and cause not sure, or if parents are freaked even if you're not
EPILEPSY
altered LOC, motor activity, change in sensation, autonomic fx
accelerated rate of synchronicity of nervous impulses??
*need good pediatric neurologist for referral list!
partial/focal seizure, single hemisphere*
simple partial has no impairment of consciousness
complex partial has altered LOC, may start as simple
both can end with grand mal convulsions
generalized siezures starts in both hemispheres symmetrically and simultaneously (???)
consciousness disturbed immediately
petit mal incl absence seizure is nonconvulsive, generalized, consciousness disturbed, no convulse
Hx: aura? sequence of events, general appearance, triggers?, duration is difficult to peg
Hx: post ictal state?
TX: refer to neurologist, need EEG while seizing for conclusive dx
Tx: picking meds is a progressive process, try one if not work try another
Tx: NDs may help manage SEs, don't try to manage grand mal s neurologist, lawsuit waiting to happen
BENIGN SLEEP MYOCLONUS
rhythmic jerking while sleeping or v drowsy
mc in kids under 3
sim to adult twitch when falling asleep and just as b9
may last longer than typical siezure
strike difference btw this and siezure: goes away with waking, doesn't happen while awake
EEG always normal
HEADACHES
common
ddx: trauma, tension, migraine, acute illness, trigeminal neuralgia
migraines more in kids than you'd think, often BL, dull, throbbing, aura, n/v, etc, relief by sleep
common migraine is mc headache in children and adolescents, stress exacerbates
regular Monday h/a: headache is not the issue, school is
complicated migraine has neuro changes same as in adults
most start at puberty onset, early would be age 8-10
some want off drugs, others circle around, remind pts that there are options
hx: concussions? football? bike? falls?
q: what age OK to do headers in soccer? not so dangerous dt no mo change in head, no direct answer
ddx: dental infx? sinusitis, tumor, hydrocephalus, undxd visual prob
vision: "kids don't know not everybody sees blurry", elementary school screenings are crap,
need specialist screening if any suspicion, fhx
dx: based on hx, acute, chronic, repetitive, location, onset, duration, associated, response to tx
pe: eent, brudzinski's, jaw, mouth, face, ears, neuro exam usu n with migraine or tension
consider referral: if can't find prob and nothing helps
LEARNING DISORDERS, disabilities
common
terms used when performance is substantially below expectation for age and intelligence
many forms that don't fit categories
dyslexia
parents come with concerns of their own or from school
they may not want a dx or label
is there a problem? behind in school?
loss of previously acquired skills is bad sign
misbehavior mb willful
outcast kid may become quirky but high performing adult
many kids have certain areas with deficits and other areas where they are strong
if you don't tx the deficit area, deficits may become general
ddx: hearing loss dt ear infx? vision problem? bipolar is overdxd, substance abuse?
ddx: adhd, odd, borderline mental retardation
dx: refer to developmental pediatrician who coordinates care with specialists
ddx: lead poisoning? anemia? meds? antiepileptics, bronchodilators-->wired
ddx: hypothyroid, absence and partial complex siezures can be missed by teachers
ddx: malnutrit, anorexia nervosa, chronic illness, poor growth
ddx: psychosoc, child abuse, bullying account for much dread of school
tx: through 4.5-5 can refer to early intervention program (in OR I think)
tx: school age you can use school system
but parents don't like school to own the dx, would rather pay to have the information and power
tx: REFER if siezure or neurodegen, depression or another psych dx, physical dysmorphology, possible genetic disorder, hearing or visual impairment, neuro ped doc
SLEEP DISORDERS
APNEA
restless sleep
onset age 2-6
"children should not snore"
consider: allergies, enlarged "ginormous" tonsils, gerd, obesity, poor growth
sx: fatigue
consider: refer to HEENT doc
tx: she has recommended removal of tonsils, worked wonders in 4-5 kids in 15 years in her practice
SLEEPWALKING
abrupt onset, benign
common in school age, 15-30% do it at least once, fewer do it often
bumbling, rumbling, walkers usu return to bed
resolves by adolescence
NIGHT TERRORS
very disturbing to parents too
child screams, sits up, sweats, breathes hard, glassy eyes, inconsolable
will sleep again after a while and won't remember
resolves with age
mb at a regular interval after bedtime
tx: find trigger, most common is being overtired
homeop: calc carb, calms forte
CEREBRAL PALSY
nonprogressive motor impairment dt dmg to developing brain
50% in premies
etio usu unknown, usu dxd at birth or early in infancy
sx: toe walking, stuttering/dysfluency, motor probs, airway, sensory and cognitive probs mb present
STUTTERING
if child doesn't blink or have physical sx with stutter, don't worry, let child finish thought, don't tell child what to do, don't ask a lot of questions or add stress, REFER if it lasts over 3-4 months, occurs in every sentence, if child is disturbed by stutter, if FHx, if sx of physical struggle while trying to speak or child starts avoiding speech
MUSCULAR DYSTROPHIES
Duchennes 1/3500 live male births, x linked
many other types
waddling gait, lordosis, poor prog, die in 20's
change in existing skills-->refer
ATAXIA AND CLUMSINESS
lack of coordination usudt cerebellar dysfx
etio: metab, tumor, infx, intox, ear infx, thyroid, head trauma
chronic: fhx or tumor
kids are clumsy, if it comes and goes don't worry so much, more with growth spurts
******************************
I. seizures
A. epilepsy vs. seizures
1. anyone can have a seizure without having epilepsy
a. 4-6% of kids will have at least one seizure by age 16
1. careful hx will often indicate cause
b. DDX of non-epileptic seizures
i. idiopathic
ii. febrile
iii. manifestation of previous illness or injury
iv. sxs of an acute condition
v. some oddball causes of non-epileptic seizures
c. risks for recurrent non-epileptic seizures
2. in newborns, they can be due to metabolic problems or focal CNS lesions
a. intracranial causes
b. metabolic causes
c. infectious causes
B. Epilepsy
1. definition: epileptic seizures are marked by altered consciousness often accompanied by motor activity and or sensory alterations. “a transient involuntary alteration of consciousness, behavior, motor activity, sensation, or autonomic function due to an excessive rate and hypersynchronicity of neuronal discharges.” (from 5 minute ped consult)
2. seizure types
a. accurate classification of seizure type often critical to getting appropriate care
i. partial aka focal
a. simple
b. complex
ii. generalized
a. consciousness disturbed immediately
b. bilateral motor manifestations
c. grand mal is an obvious example
d. petit mal is a non-convulsive generalized seizure
3. diagnosis
a. history may give indication, but EEG is definitive
b. physical descriptions are not definitive
c. diagnosis makes a difference
C. Management and Treatment
1. epilepsy should be managed by a neurologist
2. other seizures can be managed depending on the causes
D. Benign sleep myoclonus
1. rhythmic myoclonic jerking when child is sleeping or drowsy.
2. similar to jerking when falling asleep
3. may look like a seizure
4. will not respond to anti-convulsants
II. Headaches
A. Differential diagnosis
1. head injury/ trauma
2. tension headache
4. migraine
a. onset usually gradual
b. often bilateral
c. generally dull, throbbing
d. migraine types
i. classic
ii. common
iii. complicated
5. acute illness
6. trigeminal neuralgia
7. brain tumor or other structural lesion, hydrocephalus, abscess
B. good history can be the key
1. acute vs chronic or repetitive
2. duration, location, onset, associated symptoms
3. response to therapy
4. FH, PMH
5. stress
6. preceding events
C. PE
1. vitals; funduscopic exam
2. signs of meningeal irritation
3. jaw, mouth, face, ears
4. neuro exam
D. consider referral with any chronic, recurrent, persistent HA
III. Learning Disorders
A. in the Primary care setting
1. parents will often come to you with their concerns
2. determining if there is a problem
a. falling behind expected levels of performance in school
b. loss of previously acquired skills/ deterioration of performance
3. determining possible sources
a. recent changes
b. long-term problems
c. things to watch for in the primary care setting
4. pediatrician often responsible for coordination of care among other providers
a. through preschool age, early intervention
b. school age, often through the school system
c. psych referral often needed
d. always refer if
i. hx indicates seizures or neurodegenerative disorder
ii. significant depression or other psych dx
iii. physical dysmorphology or FH might indicate genetic disorder
iv. hearing or visual impairment seems apparent
v. consider referral to neurodevelopmental specialist if no cause isolated
IV. Sleep Disorders
A. apnea
1. children should not snore as a rule
B. somnambulism
1. usually abrupt onset
2. typically see bumbling, purposeless movements, often some speaking
3. typically sleepwalkers return to bed
C. night terrors
1. common in preschool children
2. often very disturbing for parents
a. child suddenly sits up and screams
b. agitated, sweating, rapid breathing, aroused but glassy-eyed
c. child is inconsolable, unlike nightmare
d. child generally returns to sleep after a while and does not recall the event in the morning
e. resolve spontaneously as children age
V. Cerebral Palsy
A. non-progressive motor impairments that result from damage to or dysfunction of developing brain
1. various classifications, including spastic, dyskinetic, ataxic, mixed
B. Associated concerns
1. sensory impairment
2. cognitive
3. neurological
4. Musculoskeletal
5. Respiratory
6. GI
7. GU
8. dental
VI. Stuttering
A. developmental dysfluency very common in preschool kids
1. most will be transient
B. refer to speech pathologist if
1. it goes on more than a few months
2. it occurs in every sentence
3. child clearly reacts to the stutter
4. FH of persistent stuttering
5. child shows signs of physical struggle when speaking
6. stutter makes the child reluctant to speak or frustrated
VII. Motor pathologies
A. muscular dystrophies
1. hereditary disorders causing progressive weakness and muscle degeneration
a. typically slowly progressive disorders involving all muscle groups, though some more than others
b. generally have loss of muscle cells, necrosis of muscle fibers, accumulation of connective tissue between muscle fibers. Can affect smooth and cardiac muscle as well
2. multiple types, classified by clinical criteria including age of onset, muscles involved, severity of disorder, associated conditions
3. Duchenne type most commonly presents in boys aged 3-7
a. proximal muscle weakness results in waddling gait
b. toe-walking common
c. lordosis exaggerated
d. frequent falls
e. difficulty standing or climbing stairs
4. prognosis poor for Duchenne type
B. ataxia and clumsiness
1. true ataxia
a. generally associated with cerebellar dysfunction
b. cerebellum is very delicate and sensitive to disruption
c. acute ataxia
i. history of insult
intoxication
trauma
recent infection
current infection
migraine
labyrinthitis
stroke
seizure
family history
d. chronic ataxia
i. associated with
brain tumor
FH
metabolic diseases
2. clumsiness
a. often presents with concerned parents
b. common in young children
c. generally transient
d. ddx includes
visual problems
intoxication
muscular dystrophy
hypothyroidism
infection
trauma
C. Spinal Musclar Atrophy (SMA)
1. progressive weakness caused by disorder of motor neurons
a. severe type starts before 6 months
b. less severe starts by age 2
c. prognosis poor, most die of respiratory failure.
2. generally see hypotonia, poor head control, generalized weakness with early onset cases.
3. late motor developmental milestones and hypotonia cause for concern
4. genetic, FH may contribute
