Diseases Block 4 Flashcards
Injury to FEF
Sx: Loss of voluntary saccades to contralateral side and deviation of eyes towards lesion. Loss of ability to move gaze away from stimulus.
Sx: can resolve over time (days/weeks) due to plasticity
Premotor Cortex lesion
Lesion produces inability:
- respond properly to stimuli (i.e. can’t use toothbrush but know how)
- Plan appropriate movements based on circumstances (can’t reach under glass for treat)
- Lean new sensory-motor associations
- Steer arm accurately
Supplementary Motor Area Lesion
Lesion results in:
- reduction in volitional movements
- loss of suppression of motor programs triggered by visual stimuli (alien hand syndrome and utilization behavior)
- Neglect of affected limb
Alien Hand Syndrome
Contralateral “semi-purposeful” movements that are outside patient’s volitional control
Seen in lesion to SMA
Utilization behavior
Use of objects in an inappropriate setting (excessive response to external stimuli
Seen in lesion to SMA
Multiple Sclerosis
Autoimmune inflammation and demyelination of CNS. Patients can present with optic neuritis (sudden loss of vision), MLF syndrome (internuclear opthalmoplegia), hemiparesis, hemisensory symptoms, or bladder/bowel incontinence. Relapsing and remitting course. Most often affects women in their 20s and 30s; more common in whites.
Genetic and environmental. Linked to HLA-DR2 and some IL-2 and IL-7 receptor polymorphisms
Findings: increased protein (IgG) in CSF. Oligoclonal bands are diagnostic. MRI is gold standard. Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with preservation of axons.
Charcot’s triad: SIN Scanning speech, Intention tremor, Incontinence, Internuclear opthalmoplegia, Nystagmus.
Treatments: Beta- Interferon or immunosuppressant therapy. Symptomatic treatment for neurogenic bladder, spasticity, pain.
Acute Disseminated Encephalomyelitis (ADEM)
Diffuse, monophasic demyelination following a viral infection; usually in children
Rapid onset headache, lethargy, coma
Fatal in 10%; rest recover completely
May be an acute autoimmune rxn vs myelin
Acute Necrotizing Hemorrhagic Encephalomyelitis
Fulminant CNS demyelination in young adults/children
that is preceded by URI.
Fatal in many patients; significant deficits in survivors
May be a hyperacute variant of ADEM
Central Pontine Myelinolysis
Symmetric loss of myelin in basis pontis and part of pontine tegmentum. Results in rapidly evolving quadriplegia.
Monophasic disease.
Caused by rapid correction of hyponatremia
Treahcer Collins Syndrome
Deficiency of neural crest cells migrating into the first arch.
Sx: Mandibulofacial dysostosis, Abnormal development of first arch structures, Hypoplasia of jaw; middle and external ear deformities, palate and eyelid defects.
“Fishmouth appearance”
Conductive hearing loss.
Autosomal dominant or teratogenic cause.
Pierre Robin Syndrome
Deficiency of neural crest cells migrating into first arch.
Sx: Abnormal development of first arch structures. U shaped cleft palate.
Micrognathia (small jaw) and large, posteriorly placed tongue leads to airway obstruction.
Genetic or environmental etiology.
Rx: Tracheostomy to maintain airway and bilateral mandibular lengthening.
Goldenhar Syndrome
1st and 2nd arch syndrome. Possibly caused vascular insult etiology.
Sx: hypoplastic maxilla, mandible, and temporal bone. Ear may be absent, vertebral abnormalitis, dermoid tumors of the eye.
Digeorge Syndrome
No thymus or parathyroids ( = diminished immunity, hypocalcemia)
Failure of neural crest migration into arches leading to 3rd and 4th pouch failure and hypoplasia of 1st arch (cleft palate, low set ears, poor feeding, delayed speech)
Heart defects, poor circulation, poor muscle tone.
CATCH 22: Microdeletion of Chr. 22q11.2 Cardiac abnormality (especially tetralogy of Fallot) Abnormal facies Thymic aplasia Cleft palate Hypocalcemia/Hypoparathyroidism
Lateral cervical (branchial) cyst or fistula
When cervical sinus formed by 2nd, 3rd, and 4th clefts persists.
Thyroglossal Duct Cyst
Midline cystic mass
Preceding URI
Mobile with tongue protrusion and swallow.
Treatment:
- treat infx first
- avoid incision and drainage
- Excision of entire tract, cyst
- Remember anatomic relationships with nerves
Branchial Cleft Cyst or Lateral Cervical Cyst
Lateral Cystic neck mass
Preceding URI
Treatment:
- treat infx first
- avoid incision and drainage
- Excision of entire tract, cyst
- Remember anatomic relationships with nerves