Section 1

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When does anterior fontanelle close?

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Last updated

6 years ago

Date created

Mar 1, 2020

Cards (68)

Section 1

(50 cards)

When does anterior fontanelle close?

Front

by 2.5 years of life

Back

What day is the notochord formed? what does it do?

Front

day 17 and induces the primitive streak to form the neural plate that later develops the neural groove and neural folds

Back

What is a Hamartoma

Front

disorganized cells in the proper location for that cell type

Back

When do the commissures form?

Front

8-17 weeks

Back

When does myelination begin?

Front

fifth fetal month and proceeds caudad to cephalad, dorsal to ventral, and central to peripheral, and sensory before motor

Back

What tissue or structure dictates cleavage of the telencephalon and development of midline facial structures?

Front

prechordal mesoderm

Back

Cleidocranial dysostosis

Front

occurs with retention of mandibular teeth, delayed closure of fontanelles, wormian bones, and midline defects

Back

What does Nissl stain for? What does it bind too?

Front

Neuron cell bodies (binds nucleic acid)

Back

When does posterior neuropore close?

Front

26 days

Back

When does Metopic suture close?

Front

around 1 year

Back

What is the most frequent cephalocele?

Front

Occipital cephalocele—the most frequent among Caucasians and in Europe and North America. There is a female predilection. The cephalocele protrudes between the foramen magnum and the lambdoid suture. It is associated with myelomeningocele (7%), diastematomyelia (3%), Chiari II and III malformations, Dandy-Walker malformation, and Klippel-Feil syndrome

Back

What are Marinesco bodies?

Front

Marinesco bodies are seen in normal brain. They are eosinophilic and contain chiefly ubiquitin. They are located mainly in the nucleus of melanin cells of the brainstem.

Back

What is Arrhinencephaly?

Front

the absence of the olfactory bulbs and tracts with normal cortex and gray matter in place of the corpus callosum. It is associated with holoprosencephaly and Kallmann syndrome (anosmia, hypogonadism, and mental retardation).

Back

When does posterior and sphenoid fontanelles close?

Front

2-3 months

Back

When does secondary neurulation occur? And what does defects of secondary neurulation cause?

Front

4-5 weeks; spinal dysraphisms below L1/2

Back

1. Pachygyria 2. Polymicrogyria

Front

1. overall decreased number of gyri and those that are present are enlarged 2. wrinkled-appearing brain with many small gyri. It forms occasionally after neuronal migration from neural injury. Usually, there are only four layers of cortex in the abnormal gyri. It may be focal or widespread.

Back

What are the two astrocytic reactions to injury and what characterizes them?

Front

1. Secondary (reactive) astrocytosis—occurs after stroke, degenerative diseases, etc. There rarely are mitotic figures unless there is neoplastic disease. Gemistocytic astrocytes are large reactive astrocytes with eccentric nuclei. Fibrillary astrocytes appear later, have smaller cell bodies, and more fibers. Rosenthal fibers are eosinophilic masses in the astrocytic processes and are increased with Alexander disease, pilocytic astrocytomas, and reactive astrocytosis. 2. Primary astrocytosis—the proliferation of astrocytes after astrocytic disease such as hepatic encephalopathy with Alzheimer II astrocytes (large nuclei, gray, glycogen inclusions)

Back

Lissencephaly

Front

smooth brain. In the complete form, the cerebral hemispheres have no sulci. In the incomplete form, there are several shallow sulci. It is associated with in utero infections

Back

What is the main component of astrocytic intermediate filaments?

Front

Glial fibrillary acidic protein (GFAP)

Back

Viral Inclusion bodies

Front

1. Intranuclear—herpes simplex virus 1 (HSV-1; Cowdry type A, eosinophilic, in neurons, astrocytes, and oligodendrocytes; seen early in the disease), cytomegalovirus (CMV), and measles (subacute sclerosing panencephalitis [SSPE]) 2. Intracytoplasmic—rabies (Negri bodies), SSPE, and CMV

Back

Ectopias

Front

neurons in locations brain tissue should not be in (i.e., the subarachnoid space). They are associated with dysraphism and hydranencephaly

Back

Schizencephaly

Front

a gray matter-lined cleft extending from the pia to the ventricle. The cleft may be filled with CSF (opened lip) or collapsed (closed lip)

Back

What are microglia?

Front

macrophages from outside the CNS. With mild injury, there may be rod cells with cigar-shaped nuclei.

Back

What are ependymal cell's reaction or response to injury?

Front

DO NOT proliferate with injury but when damaged are replaced by subependymal astrocytes

Back

What is ferrugination

Front

dead neurons become encrusted with Fe and Ca salts --> red neurons

Back

What is central chromatolysis?

Front

occurs after an injury to an axon near the cell body. The Nissl substance disappears, the nucleus becomes eccentric, and the cell body enlarges. It is seen in the anterior horn cells with anterior nerve root compression and Guillain-Barré syndrome. The cells may progress to death or recover.

Back

What are dermal sinus tracts?

Front

epithelium-lined tracts caused by faulty segmental disjunction. 60% extend from the skin to the spinal canal, although they may end in the subcutaneous tissue, dura, spinal cord, or nerve roots. 50% end in epidermoids or dermoids. More than 50% are lumbar and the next most frequent site is occipital. There is no gender predilection. Symptoms are mainly a result of infection. They are associated with skin dimples, hyperpigmentation, hairy nevi, and capillary malformations.

Back

A problem at what stage causes vascular malformations and neurocutaneous syndromes.

Front

Neuronal proliferation and differentiation

Back

What are the 5 characteristics of progressive or reversible neuronal injury seen?

Front

1. Central chromatolysis 2. Neurofibrillary tangles 3. Neuronal storage of lipids or carbohydrates 4. Inclusion bodies (viral or degenerative/metabolic) 5. Marinesco bodies

Back

When does the anterior neuropore close? what does it form?

Front

24 days - lamina terminalis

Back

During development, if mesenchyme enters the neural tube and forms lipomas and/or lipomyelomeningoceles, what is this called?

Front

Dysjunction

Back

Brain and spinal cord are formed from neuroectoderm at how many weeks?

Front

3-8 weeks

Back

When do the neural folds fuse?

Front

22 days to form the neural tube

Back

True melanin is made by what? and located where?

Front

tyrosinase and located in the leptomeningeal melanocytes of the ventral medulla and cervical cord. (form the primary CNS melanomas)

Back

What do the ectodermal placodes form?

Front

they overly the neural tube and form the olfactory epithelium and cranial nerve 5, 7 to 10 ganglia

Back

What are pia/arachnoid formed from?

Front

neuroectoderm

Back

A problem at what stage causes callosal agenesis, schizencephaly, and heterotopias

Front

Cellular migration

Back

What does silver stain for?

Front

Cell processes

Back

What are Heterotopias?

Front

normal neurons in abnormal CNS locations (i.e., in the centrum semiovale, along the lateral ventricles or in the cerebellar white matter). They may be laminar or nodular and usually do not enhance

Back

Abnormalities at what stage cause holoprosencephaly, septooptic dysplasia, and Dandy-Walker malformation?

Front

Ventral induction

Back

What is dura formed from?

Front

mesodermal elements

Back

Why does partial agenesis of corpus callosum always include rostrum and splenium?

Front

rostrum and splenium are formed last

Back

When does the germinal matrix form? what does it produce? and when does it involute?

Front

7 weeks; produces neurons and glia; 30 weeks

Back

What is a Choristoma

Front

correctly organized cells in the wrong location

Back

What are neurofibrillary tangles?

Front

Argyrophilic linear densities accumulate in the cell bodies and processes. They can be detected on silver stain. The neurofibrils are made up of hyperphosphorylated tau proteins, neurofilaments, and microfilaments (actin). They are much more numerous with Alzheimer disease, postencephalitic Parkinson disease, progressive supranuclear palsy, and aluminum toxicity.

Back

What is Septooptic dysplasia?

Front

(de Morsier syndrome)—occurs with mild lobar holoprosencephaly, absence of the septum pellucidum, schizencephaly, and hypoplastic optic nerves. It is associated with seizures, visual symptoms, hypothalamic-pituitary dysfunction (precocious puberty), enlarged ventricles, and hypotelorism

Back

When does mastoid fontanelle close?

Front

by 1 year

Back

When does primary neurulation occur? and what does it form?

Front

3-4weeks. Neural plate, neural groove, and neural folds

Back

What is ventral induction?

Front

the growing brain's influence on the overlying mesoderm causing it to grow

Back

What is myelomeningocele associated with?

Front

Chiari II malformation (100%), hydrocephalus (80%), lipoma (75%), syringomyelia (50%), diastematomyelia (40%), scoliosis (20%), kyphosis (10%), orthopedic deformities, and callosal dysgenesis

Back

Section 2

(18 cards)

Cowden Syndrome

Front

Multiple hamartoma syndrome due to mutations of the phosphatase and tensin (PTEN) gene on chromosome 10q

Back

Chiari III malformation

Front

hindbrain herniation into an encephalocele, usually occipital or high cervical

Back

What is Chiari I malformation? and what are is measurement definitions based on age?

Front

peg-like tonsils extending below the foramen magnum (6 mm below the foramen magnum at age <10 years, 5 mm <30 years, 4 mm <80 years, and 3 mm <90 years). If the tonsils extend >12 mm below the foramen magnum, all are symptomatic and from 5-10 mm, 70% are symptomatic.

Back

Porencephaly

Front

a cleft not lined with gray matter but by gliotic white matter that forms after an insult to an otherwise normal brain

Back

Chiari II malformation associated abnormalities

Front

Skull and dura—lacunar skull ("lückenschädel," with scooped out appearance), small posterior fossa, low-lying torcula and transverse sinus, large foramen magnum, concave petrous temporal bones, short concave clivus, and a thin falx cerebri with occasional interdigitating gyri through fenestrations in the falx cerebri (Fig. 3.13) Hindbrain—herniation of the vermis, nodulus, uvula, and pyramis through the foramen magnum, medullary kinking (70%), enhancing ectopic choroid, upward cerebellar herniation, and tectal beaking CSF spaces—tubular fourth ventricle, large third ventricle with enlarged massa intermedia, colpocephaly (large atria and occipital horns), aqueductal stenosis, small cisterna magna, and hydro-cephalus (90%) Cerebral hemispheres—heterotopias, polymicrogyria, and callosal dysgenesis Spine—myelomeningocele (100%), syringomyelia (50-90%), diastematomyelia, and incomplete C1 arch (70%). It is NOT associated with lipomyelomeningocele.

Back

Unilateral lambdoid synostosis must be distinguished from what?

Front

positional plagiocephaly - not present at birth

Back

Difference between hydromyelia and syringomyelia?

Front

dilation of central canal lined by ependymal cells whereas syringomyelia is surrounded by astrocytes

Back

Crouzon syndrome

Front

most frequent craniofacial syndrome, autosomal dominant or sporadic inheritance, shallow orbits, exophthalmos, midface hypoplasia, malformed ears, agenesis of the corpus callosum, less severe mental retardation than with Apert syndrome, and increased incidence of hydrocephalus. More than one suture is involved, and they may have oxycephaly, turricephaly, or dolichocephaly. The sphenofrontal synostosis produces exophthalmos; associated with mutations of the fibroblast growth factor receptor 2.

Back

Most common craniosynostosis?

Front

Sagittal synostosis—most common (50%), male predominance, causes scaphocephaly or dolichocephaly

Back

Chiari I malformation is associated with what other brain abnormalities?

Front

None! but is associated with skeletal abnormalities in 25% of cases: basilar invagination (25-50%), Klippel-Feil syndrome with fused cervical vertebrae (5-10%), atlantooccipital fusion (5%), and cervical spina bifida occulta (5%)

Back

Unilateral megalencephaly

Front

hamartomatous overgrowth of one hemisphere with ipsilaterally enlarged ventricle and cortex. It is associated with seizures.

Back

Apert syndrome

Front

second most common craniofacial syndrome, autosomal dominant or sporadic inheritance, turricephalic head, maxillary hypoplasia, orbital hypertelorism, syndactyly, mental retardation, deafness, flat nose, and vertebral and skeletal abnormalities. Only the coronal suture is involved. There are GI, GU, and cardiac abnormalities, and increased incidence of frontal encephaloceles; also associated with mutations of the fibroblast growth factor receptor.

Back

Chiari IV malformation

Front

cerebellar hypoplasia, an entity distinct from the other chiari malformations

Back

Chiari II malformation

Front

thought to develop when the neural folds do not completely meet and there is abnormal ventricular CSF flow into the amnion with collapse of the ventricles.

Back

Chiari malformations of all types present differently at different age groups: 1. Infant 2. Child 3. Adolescent 4. Adult

Front

Infants—hydrocephalus and brainstem compression with apnea, decreased gag reflex, nystagmus, and spasticity Children—nystagmus, spastic paralysis, and bulbar dysfunction Adolescents—progressive spasticity and cape-like pain and temperature loss in the upper limbs Adults—occipital headache, neck and arm pain, and nystagmus

Back

Lhermitte-Duclos disease

Front

hypertrophied cerebellar granular cell layer and increased myelin in the molecular layer of the cerebellum with thick folia. There is mass effect on the fourth ventricle. The lesion is considered a hamartoma. There may be calcifications, hydrocephalus, and folia with increased signal intensity on T2-weighted MRI. It is associated with Cowden syndrome where patients have facial trichilemmomas, fibromas of the oral mucosa, hamartomatous polyps of the gastrointestinal (GI) tract and breast, and thyroid tumors. Cowden syndrome is due to mutations of the phosphatase and tensin (PTEN) gene on chromosome 10q.

Back

Dandy-Walker malformation

Front

posterior fossa cyst continuous with the fourth ventricle with partial or complete vermian absence. It may be due to failure of development of the superior medullary velum (roof of the fourth ventricle) combined with fourth ventricle outlet atresia. It is associated with a large posterior fossa, high tentorium and transverse sinus, lambdoid-torcula inversion, hydrocephalus (80%), callosal agenesis (25%), heterotopias, schizencephaly, cephaloceles, dolichocephaly, cardiac abnormalities, and polydactyly

Back

Klippel-Feil Syndrome is associated with what deformity?

Front

Sprengel deformity (elevation of the scapula) & Chiari I malformation

Back