Neurological Diseases Flashcards
Cerebral Palsy
group of problems that affect body movement and posture
non-progressive and usually related to brain injury
Etiology: anoxia during birth, genetic condition, or intrauterine infection
Associated with: seizures, mental retardation, hearing and speech problems
Types of CP
Spastic: 80% - decreased spontaneous movements and increased muscle tone (exaggerated reflex, stiff)
- commando crawl, delayed walking, toe walking
- diplegia, hemiplegia, quadriplegia
Dyskinetic: 15% - problem controlling limb movements
- hypotonia as infant, speech problems, difficulty feeding, and ataxic
Mixed Forms
Vision Issues in CP
relate to severity of CP
Optic Neuropathy in 10% mild CP vs 65% severe CP
Avg VA: 20/100 H > Myopia 55% Accommodation Dysfunction 60% strab (overall ET more common) BUT spastic (XT more common clinically) VFD + poor VMI + CVI
CVI
Cortical/Cerebral Vision Impairment
damage to the BRAIN (not eye)
- detect with MRI/CT
may have normal eye exam but decreased VA
- strong color preference
- movement to see objects
- delayed visual responses
- light gazing
- VF preference
- no visually guided reach
Etiology: any disorder that affects BRAIN
- hypoxia, CVA, infection, structural, metabolic conditions
Nystagmus
rhythmic, biphasic oscillation in eyes due to sign of vision loss, CNS disorder, response to external stimuli, body rotation, or inner ear disorder
unilateral < bilateral
Note the amplitude, frequency, conjugacy, and type
Hx: age of onset, head nodding, head turn, meds (anti-epileptics, drugs, ETOH), development status, birth and family history
Neuro Signs: seizures, poor balance, failure to thrive (develop), large head, vomit, dizzy, HA
Types of Nystagmus
Jerk: fast and slow
Pendulum: equal velocity both ways
Nystagmus Exam
VA with a frosted occluder or high plus lens bc you don’t want to fully occlude (latent)
Motilities in all 9 gazes
Pupils, ON assessment (DFE + CV), OKN, consider MRI
Nystagmus Tx
Correct RE, Prisms (BO to stimulate convergence), Biofeedback, Sx to move eyes to null point, Education, Low Vision
Infantile/Congenital Nystagmus
- onset
- manifestations
onset 2-4 mos old and may improve with age
pendular is classic (can be jerk at endgaze) or jerk
Nystagmus Blockage Syndrome: develop ET and converge eyes with head turn to depress nystagmus
Congenital ET Syndrome: have associated ET from congenital nystagmus
Symptomless
Latent: will appear once 1 eye is covered
OKN Inversion
- normal is eyes follow tape and beat opposite quickly
- congenital: eyes beat in direction of tape
Fixation: worse at distance and better at near bc convergence
Upgaze remains horizontal
Null Point: head tilt with minimal nystagmus
Forms of Infantile/Congenital Nystagmus
Motor Forms: efferent pathway disorder of oculomotor systems involved in fixation (behind eye to brain)
- may be genetic
Sensory Forms: ON abnormalities, Ocular Albinism, Cataracts, tend to be pendular
Acquired Nystagmus
pendular/jerk or see-saw (one up and one down; super rare and caused by midbrain lesion)
Down Beat: cerebellar lesion
Up Beat: cerebellum, medulla, midbrain lesion
Spasmus Nutans can occur
Spasmus Nutans
Triad: Head Tilt (Torticollis) + Head Nod + Monocular or Asymmetrical Nystagmus
*MONOCULAR NYSTAGMUS: ORDER MRI because it can be caused by a tumor in third ventricle or optic chiasm
starts 4-14 mos and resolves by 5 years old (usually benign)
Glioma
APD, Optic Atrophy/Edema, Large Head, Cafe Au Lait (NF1) , Neuro dysfunction
Retinal Disease
DDX for Spasmus Nutans
Achromatopsia (no CV) and Congenital Stationary Night Blindness
Headache
Hx: onset, duration, frequency, location, associated signs, relief?, systemic meds, intensity (1-10)
Visual causes: reading, trying to see far, squinting
- will not wake up with HA, vomit, systemic findings, before lunch, when playing
Exam: history guides testing!!
- Refraction, Binocular + Accommodation Testing if indicated, DFE with attention to ON (SVP and ON edema), VF (VFD)
How to differentiate between true ON edema and pseudo edema
Causes of ON Edema: Papilledema (increased ICP) from hydrocephalus or PTCS/IIH and Neuroretinitis (infection)
- HA + Diplopia > Vomit, Blur, Stiff neck
Causes of Pseudo Edema: drusen or crowded nerves
Papilledema
optic disc swelling with increased ICP (looks bad but sees good : VA ~20/20)
can have increased ICP without swelling + retinal folds (Paton’s folds) and enlarged blind spot in VF
Early: blurred disc margin (sup.inf first) and absent SVP
(normal in 30%)
Late: retinal hemorrhage and exudates in retina
PTCS
Pseudotumor Cerebri Syndrome
Primary: IIH (idiopathic intracranial HTN)
- thin, mildly obese women with PCOS
Secondary: cerebral venous anomalies, Meds (tetracycline, steroid withdrawal, hormones (GH and levonorgesterol), medical condition like DS, Turner syndrome, Addisons)
- more common in children 53-78%
Dx: MRI 1st and if no tumor, do a spinal tap to check the ICP (bc PTCS should show no tumor)
Adults with PTCS
mild obese, F>M, HA, only CN VI (6)
Child with PTCS
F=M, other CNP, no role of obesity until puberty
PTCS Eval
DFE (check SVP), B-scan to rule out Drusen, ON OCT to rule out drusen and edema, VF (bigger blindspot), if suspect papilledema, order MRI to rule out tumor
Modified Dandy
Criteria for Dx PTCS
signs of increased ICP, no neuro signs (CN VI), CSF >25 cmH20 and normal/small ventricles
PTCS Dx Criteria
papilledema, normal neuro (CNP 6 and 7), normal parenchymal MRI/MRV, normal CSF composition, CSF >28cmH20 in sedated child and >25cmH2o in adult/non-sedated
if normal nerve, other imaging abnormality
steps when child does not see 20/20
pinhole, pupils (neuro), CV and VF (neuro/retina)
CT (constant strab - Amblyopia, Incomitant strab - Neuro, and intermittent strab (rule out other cause)
Ret: amblyogenic factors
Anterior Segment: look at lens and cornea
DFE!!!!
Streff Syndrome
Non-malingering + no obvious pathology
80% pre-pubescent females ages 8-14 years old
VA range: 20/20 to 20/200 but does NOT complain about vision loss
decreased VA bilaterally in D/N but near is worse
abnormal CV and VF
low + refraction
Stress Theory: increased HR/BP/stomach acid/sweat/attention/arousal/emotional lability (rapid changes in emotion)
ask about stress at home, seeing something awful, upset at life etc
