Neurology Flashcards
What characterizes a static condition?
seen in first few months and do not change over time
What characterizes a progressive condition?
degenerative disease or neoplasm that worsens over time
What characterizes an intermittent or brief disease?
Epileptic or migraine-like symptoms. You either have it or you don’t.
What characterizes a saltatory condition?
bursts of symptoms followed by partial recovery, some periods are worse than others
Ophthalmoplegia and Limb Weakness
Myasthenia gravis, Botulism, Myotonic dystrophy, Miller Fisher variant of GB sydnrome
Facial and Bulbar Weakness
All of the same conditions as ophthalmoplegia and limb weakness plus polio and facioscapulohumeral dystrophy
Spinal muscular atrophy syndrome
AR, Chr 5, progressive weakness of LMN due to degeneration of anterior horn cells, most are present at infancy
What disorder in adults is spinal muscular atrophy syndrome similar to?
ALS– Flaccid weakness (symmetric), decreased spontaneous movement, hypotonia, decreased DTR, fasciculation, progress to decreased facial expressions with increased drooling. death due to respiratory dys or infection.
What is important to remember about patients with spinal muscular atrophy syndrome?
They have normal cognitive, social, and language development
What is acute transverse myelitis? Where is it usually located?
It is acute inflammation of gray and white matter of one or more spinal cord segments, usually thoracic
What is the cause of acute transverse myelitis?
Unknown, but related to multiple sclerosis, infections, autoimmune or post-infectious inflammation, vasculitis, and certain drugs
What are the symptoms of acute transverse myelitis?
Onset of hours to weeks of bilateral motor, sensory, and sphincter deficits below the level of the lesion. Usually comorbid with back pain, sudden paresthesias, weakness.
How is a diagnosis of acute transverse myelitis made?
MRI and CSF analysis. MRI will reveal lighter areas of localized inflammation on the peripheral surfaces
What is Guillain Barre Syndrome also known as? What is it described as? What causes it?
Acute idiopathic polyneuritis; inflammatory peripheral neuropathy; caused by post infectious (respiratory or GI) campylobacter jejuni (maj), cytomegalovirus, EBV, and mycoplasma pneumoniae
What are the hallmarks of GB syndrome?
Rapid loss of motor strength (ascending), symmetric weakness, many have sensory symptoms with few sensory findings, loss of DTRs EARLY ON
What other symptom might someone with GB syndrome have?
Autonomic nerve dysfunction
How is a diagnosis of Guillain Barre Syndrome made?
Elevated CSF protein with no increased WBC
EMG
What is the mortality of someone with Guillain Barre Syndrome?
1-2% if treated properly, 20% end up on ventilator
What is the treatment for Guillain Barre Syndrome?
IV Immunoglobulins, plasmaphoresis
What is myasthenia gravis?
Chronic disease of rapid fatigability of striated muscle. It is usually immune mediated and creates a NM blockade. The post-synaptic muscle membrane (motor end plate) is less responsive due to circulating Ach nicotinic receptor binding antibodies.
What age groups can you see myasthenia gravis?
begins in teenage years
What are hallmark symptoms of myasthenia gravis?
Ptosis, diplopia, ophthalmoplegia, weakness of face/extremities, dysphagia. Symptoms worsen throughout the day or with exertion. No primary complaint of muscle pain or sleepiness
How do you diagnose myasthenia gravis?
CT scan for thymoma, serology for autoantibodies, NCS/EMG (repetitive), tensilon (AchE) challenge test
How do you treat myasthenia gravis?
AChE inhibitors, immunosuppressors
What is the most common hereditary NM disease?
Duchenne + Becker Muscular dystrophy
What is the sign for Duchenne Becker Muscular Dystrophy? What kind of heredity is it?
Gower’s Sign, sex-linked recessive
What are some hallmarks of DMD?
Mild intellectual impairment, hypertrophy of calves, proliferation of connective tissue
How do you diagnose DMD?
Muscle biopsy, absence of dystrophin (an Ig protein associated with muscle fiber pm), EMG, serum CPK levels-markedly elevated
What is the primary treatment for DMD?
symptomatic with glucocorticoids, gene therapy is new, also combined with creatine and aminoglycosides
What is the scapulohumeral muscular dystrophy?
AKA Emery-Dreifuss MD, it is a group of myopathies that affect the muscles of the hip and shoulder
When is the onset of Emery-Dreifuss MD?
Mid to late childhood and early adolescence, usually wheelchair bound by age 30
What is Limb-Girdle muscular dystrophy?
X-linked recessive long arm Xq28. It includes other mutations that cause myotubular myopathy
What are two other myotubular myopathies that are associated with the Xq28 chromosome?
Adrenoleukocystrophy and Block-Sulzberger (pigment changes)
What is the age of onset for Limb-Girdle MD?
5-15 years, cardiomyopathy is severe
What are some symptoms of pediatric brain tumors?
Headache, nausea and vomiting, loss of balance, speech problems, sleepiness, personality changes, seizures, increased head circumference in infants
What are the three types of gliomas we discussed?
Astrocytoma, brain stem glioma, and ependymomas
What percentage of brain tumors in children are meduloblastomas?
15%
What chromosome is Type 1 Neurofibromatosis (NF) carried on? What is its classification?
AD on 17; peripheral
What chromosome is Type 2 Neurofibromatosis carried on? What is its classification? What are its characteristics?
AD on 22; central; incurable disease-multiple intracranial and spinal tumors.
What are the physical characteristics of NF?
Cafe-au-lait spots, numerous macules greater than .5 cm present at birth. Neurofibromas form in late adolescence along nerves. There is also axillary and groin freckling and a greater incidence of CNS tumors.
Is there any cognitive impairment with NF?
There is a range of cognitive impairment
How do we treat NF?
Surgical excision of tumors
Which fontanelle closes first? second? third?
posterior; sphenoid; anterior
What characterizes an upper motor neuron lesion?
Spasticity, negative fasciculations, and increased tone (CNS)
What characterizes a lower motor neuron lesion?
Flaccidity, positive fasciculations, decreased tone (PNS)
Moro reflex
sudden head extension then extension and flexion of limbs, subsides at 0-6 months, originates in the brainstem
Rooting reflex
tactile stimulus near the mouth then pursuing stimulus, subsides at 0-6 months, originates in the brainstem
Trunk incurvation reflex
Stroke skin near vertebrae and display curvature of the spine opposite, subsides at 0-6 months, originates in the spinal cord
Cerebral Palsy classification
70% of cases are caused prenataly, usually idiopathic. 4-9% may be due to asphyxia at delivery. NOT HEREDITARY.
How do you treat cerebral palsy?
OT/PT/ST, anti-spasmodics such as dantrolene, some benzos. Botulism toxin
What is the most frequent permanently disabling birth defect?
Spina bifida
In what condition are Arnold-Chiari malformations present? And what are they?
Spina bifida, cerebellar herniation through foramen magnum
What is the clinical presentation of spina bifida?
occipital HA that radiates up, becomes worse with valsalva
What is the treatment for spina bifida?
Pain treatment or surgical decompression
What other medical condition is spina bifida occasionally a/w?
hydrocephalus and 4th ventricle obstruction
What is the most severe type of spina bifida? What are its characteristics?
Meningiomyelocele; SC or nerve roots are exposed on the back, area covered with sores and CSF leaks, leg paralysis and incontinence, Chiari malformations
What is the rarest form of spina bifida? What are its characteristics?
Only meninges are exposed in a cyst-like sac, underlying sc is intact, no HC or neurologic deficits
What is Dandy-Walker syndrome?
Enlargement of the 4th ventricle, 90% have hydrocephalus, usually a posterior fossa cyst, 50% have normal IQ
How do you treat hydrocephalus?
Loop diuretic, acetazolamide, surgery with a shunt and removal of the obstruction
