Neuroscience - Block 1 - Neuro Pathologies

davidwurbel7's version from 2016-02-09 16:22


Question Answer
Symptoms include sudden fever, headaches, stiff neck and back, confusion, drowsiness, clumsiness. Prognosis varies; typical episode last 1-2 weeks but can leave lasting neurological deficitsEncephalitis
Inflammation not due to bacterial infection. Mostly viral but other causesAseptic meningitis
Characterized by ipsilateral cranial nerve deficits, and contralateral body deficitsBrainstem Lesions
Congenital neural tube defects with progressive neurological damageSpinal Dysraphism
Neither spinal cord nor meninges herniateSpina Bifida Occulta
Spinal cord and/or meninges herniatesSpina Bifida Cystica
Meninges herniatesMeningocele
Meninges + spinal cord herniatesMyelomeningocele
Skin at the site of the defect may be normal, show a dimple, fatty deposits or hair growth, have a birthmark (hemangioma) may be indications ofSpina Bifida Occulta
Symptoms include loss of bladder or bowel control, Partial or complete lack of sensation, Partial or complete paralysis of the legs, Weakness of the hips, legs, or feet of a newborn, Hydrocephalus in 15-25% of children leading to seizures. Deformed feet or legs, such as clubfoot and curved spineMyelomeningocele
Protrusion of meninges and cord forced out by a syrinx (cyst)Syringomyelocele
Most severe type of spina bifida where the spinal cord is open. Neural tissue is only covered by a thin membraneMyelocele (Myeloschisis)
There is a cleft through the entire spine. Complete defect involving the entire spine from top to bottomRachischisis
High levels of Alpha Fetaprotein (AFP) in amniotic fluid and is indicative of possibleNeural Tube Defects
Epithelium-lined tract from the skin to down to the spinal cord or arachnoid materCongenital Dermal Sinus
Herniation of meninges (but not brain) through the craniumEncephalocele
Serious cranial birth defect where a baby is born without parts of the brain and skullAnencephaly
An abnormal increase in CSF within the ventricular system, with the following increase in ICP, Expansion of cerebral ventricles and/or atrophy of the brainHydrocephalus
The most common cause of HydrocephalusAqueductal Stenosis
Failure of the brain to divide properly into the right and left hemispheresNonsyndromic Holoprosencephaly
Posterior frontal lobes and parietal lobes unseparated with the basal forebrain normalMIHV
Fusion of the lateral ventricles. Also fused with the 3rd ventricle, Fusion of the fornices, Absence of septum pellucidum. Possibility of a normal corpus callosumLobar Holoprosencephaly
Absent septum pellucidum. Monoventricle. Rudimentary falx cerebri. Incomplete interhemispheric fissure. Partial or complete fusion of the thalami. Absent olfactory tracts and bulbs. Agenesis or hypoplasia of the corpus callosum. Incomplete hippocampal formationSemi-Lobar Holoprosencephaly
Monoventricle. Fused thalami. Absent corpus callosum. Absent interhemispheric fissue. Absent septum pellucidum. Absence of 3rd ventricle. MCA and ACA replaced by tangled internal carotid and basilar arteries. Severe facial malformationsAlobar Holoprosencephaly
Cysts or cavities fill with CSFPorencephaly
Signs and symptoms include Macrocephaly (large head) or microcephaly (small head). Slight or incomplete paralysis. Delayed growth and development. Hypotonia (low muscle tone). Muscle shrinkage or shortening. Cognitive impairments. SeizuresPorencephaly
Absence of folds in the cerebral cortexLissencephaly
This is caused by defective neuronal migrationLissencephaly
Signs and symptoms include severe psychomotor retardation, Unusual facial appearance, Difficulty swallowing, Failure to thrive, Muscle spasms, Seizures and Deformed hands and fingersLissencephaly
Death of myelinated neurons around the lateral ventricles due to softening of the brain tissuePeriventricular Leukomalacia
This is caused due to a lack of oxygen or blood to the periventricular areaPeriventricular Leukomalacia
No obvious signs or symptoms but at risk for motor disorders, Delayed mental development, Coordination problems, Vision and hearing impairmentsPeriventricular Leukomalacia
Genetic disease that causes benign tumors in the brain, but also kidneys, heart, eyes, lungs, and skinTuberous Sclerosis
Cortical tubers, Subependymal nodules (SEN), Subependymal giant astrocytomas (SEGA) which can block ventricular system leading to increase in ICP, Headaches, Blurred vision, NauseaTuberous Sclerosis
Extension of the cerebellar tonsils (the lower part of the cerebellum) into the foramen magnum without involving the brain stemArnold-Chiari Type I
Extension of cerebellar and brain stem tissue into the foramen magnum, and cerebellar vermis only partially complete or absentArnold-Chiari Type II
The cerebellum and brain stem herniate through the foramen magnum and into the spinal cordArnold-Chiari Type III
Involves an incomplete or underdeveloped cerebellumArnold-Chiari Type IV
Enlargement of the 4th ventricle, partial or complete absence of the vermis, and cyst formation in posterior fossaDandy-Walker Syndrome
Corpus callosum is partially or completely absentAgenesis of the Corpus Callosum
Missing tissue in the eye appearing gaps in one of several parts of the eye, especially the iris, retina, retina choroid, or optic nervesColoboma
Untreated syphilis. Generally occurs more in thoracic/lumbar regions - lower extremities/regions are more affected. Stabbing pains in lower limbs. Hypersensitivity to touch, heat, cold. Loss of sensation in skin and trunk. Loss of awareness of a full bladder. Loss of proprioceptive information. Loss of deep pain sensation. Ataxia and hypotonia of lower limbs. Loss of reflexesTabes Dorsalis
Degeneration of both UMN (specifically the corticospinal tract, more on that later) and LMN’s. Loss of voluntary control of muscles, loss of bowel and bladder control. Eye muscles usually, but not always, spared. Cognition and ANS usually intact. Death in 3-5 yrs due to respiratory arrestAmyotrophic Lateral Sclerosis (ALS)
Displaying the emotion effect of an emotion without feeling the actually emotionsPseudobulbar Affect
Degeneration of LMN’s (mostly spinal, only occasionally bulbar), resulting in ‘flaccid’ paralysis, atrophyPoliomyelitis
Developmental abnormality in formation of the central canal. Usually occurs in cervical segments. Symptoms include - Segmental losses of pain and temperature senses. Some impairment of touch. Expansion of lesion can eventually result in paralysis and atrophy of upper limb musclesSyringomyelia
Inflammation of the spinal cord. Usually only at one level. Caused by infection (viral or bacterial), such as herpes or syphilis. Symptoms are similar to spinal cord transection and LMN (lower motor neuron) damage. Inflammation results in demyelinationTransverse Myelitis
Symptoms include Paresis (hemiparesis), Babinski sign ‘present’, Hoffman’s sign present, Abdominal reflex absent, Cremasteric reflex absent, Spasticity, which is hyper-reflexia of DTR’s, clonus (repetitive movement), and increased muscle tone (rigidity)Corticospinal Tract (CST) Lesion
Weakness/paralysis/’paresis’. Spasticity (with its components). Babinski (+), Hoffman’s (+), loss of abdominal and cremasteric reflexesCorticospinal Tract Lesion
Weakness/paralysis/’paresis’. Spasticity (with its components). Babinski (-), Hoffman’s (-), no loss of abdominal and cremasteric reflexesExtrapyramidal UMN Lesion
Damage to anterior horns, nerve rootsLower Motor Neuron Damage
Symptoms include Flaccid paralysis, Atrophy of muscles, Loss of reflexes. Muscular fasciculation (muscle ‘twitches’ seen during slow destruction of LMN’s, like ALS). Muscular contracture (shortening of muscles). Reaction of degeneration (decreased response of muscle to electrical stimulation)Lower Motor Neuron Damage
Symptoms occur ONLY at the level of lesionLower Motor Neuron Damage
Occurs after major damage to the spinal cord. Cord functions below the lesion are lost or depressed. Persists for less than 24 hours to several weeksSpinal Cord Shock Syndrome
Motor Symptoms - Bilateral LMN paralysis at level of lesion, with muscle atrophy. Bilateral UMN signs below lesion. Sensory Symptoms - Bilateral loss of all sensation at and below the lesionComplete Cord Transection Syndrome
Motor Symptoms - Bilateral LMN symptoms at lesion. Bilateral UMN symptoms below the lesion, but NOT Babinski, or loss of abdominal or cremasteric (CST not damaged). Sensory Symptoms - Bilateral loss of pain, temperature and light touch (spinothalamic tracts) at/below level of lesion. Propioceptive loss (maybe), spinocerebellar tractsAnterior Cord Syndrome
Occlusion of the anterior spinal artery. Anterior horns, some extrapyramidal tracts, and spinothalamic tracts damaged, CST and dorsal columns sparedAnterior Cord Syndrome
Motor Symptoms - Bilateral LMN symptoms. Bilateral UMN paralysis with ‘sacral sparing’ (remember the somatotopic organization). Hoffman's (+), Babinski (-) Sensory Symptoms - Bilateral loss of pain, temperature and light touch, also with ‘sacral sparing’. Dorsal column symptoms may be presentCentral Cord Syndrome
Occurs after hyperextension injury in an individual with long-standing cervical spondylosisCentral Cord Syndrome
Motor Symptoms Ipsilateral LMN symptoms at level of lesion. Ipsilateral spastic paralysis, Babinksi, hyperreflexia, below level of lesion. Sensory Symptoms - Ipsilateral loss of two-point discrimination, vibratory and proprioceptive senses (dorsal column). Contralateral loss of pain, temperature, touch, 1-2 segments below the lesion (spinothalamic). Complete sensory loss, ipsilateral, at the level of lesionBrown-Sequard Syndrome
LMN symptoms only damagePoliomyelitis
Symptoms of both LMN and UMN damage are presentALS
All corticospinal tracts damaged only.Familial Spastic Paraparesis
Dorsal columns and corticospinal tracts damage. Loss of UMN and loss of some proprioceptionSubacute Combined Degeneration
Dorsal columns, corticospinal and spinocerebellar tracts. Complete loss of proprioceptionSpinocerebellar Degeneration
Caused by pernicious anemia, B12 deficiency. Symptoms include paresthesias, leg stiffness, weakness, lethargy, fatigue, ataxia. Results in degeneration of myelin and eventually the neurons. Occurs most frequently but is not limited to the cordSubacute Combined Degeneration
Onset at 5-15yrs. Muscle weakness and ataxia in arms and legs. Vision impairment, hearing loss. Slurred speech. Aggressive scoliosis (curvature of the spine). Diabetes mellitus or carbohydrate intolerance. A heart condition (enlarged heart)Friedreich’s Ataxia
Onset at 15-40yrs. Weakness in the arms and legs. Spasticity. Staggering, lurching gait, often mistaken for drunkenness. Difficulty with speech and swallowing. Nystagmus, diplopia, polyuriaMachado-Joseph Disease
Damage above T12. Bladder functions at segmental level without control from higher centers. Urination is involuntary, frequent and caused by spasms in the lower limbs. The sensation of fullness is lackingSpastic Bladder
Due to direct injury to the peripheral innervation of the bladder, or to lesions of the cord around S2-4. Retention with overflow incontinenceAtonic (flaccid) Bladder