Spine Flashcards
Network of valveless veins extends along the vertebral column from the pelvic venous plexus to the intracranial venous sinuses without passing through the lungs
Batson plexus
Region most affected by vertebral bacterial osteomyelitis
Lumbar spine
Area of the spinal cord affected by HTLV-1
Posterolateral cord
Most common location of spinal DAVFs
Low thoracic cord or conus medullaris
True spinal intramedullary AVMs are located where?
dorsal surface of the lower half of the spinal cord
Intradural perimedullary and subpial AVMs are located where?
Anterior parts of the cervical enlargement
What is Foix ALajouanine Myelopathy?
Amyotrophic paraplegia from severe necrosis of both gray and white matter of the LS region and a marked increase in the number of small vessels with thickened , cellular and fibrotic walls (angiodysplastic)
Extensive vascular malformation of the spinal cord with associated cutaneous vascular nevus overlying the AVM/ presence of hemangiectatic hypertrophy of finger or hand
Klippel- Trenaunay- Weber- Syndrome
Observed in patients subjected to high underwater pressure then ascend too rapidly
Caissons disease
Caisson disease affects mainly which segment and area of the spinal cord
upper thoracic SC, posterior column
Most common type of Hereditary Spastic Paraplegia
SPG4: with mutation of gene encoding for protein SPASTIN
Protein encoded by SPG-3
ATLASTIN
HSP with developmental delay or dementia associated with mutation of ALDH3A2? What protein is coded?
Sjogren- Larsson Syndrome
codes for aldehyde dehydrogenase
pathology of SPG
degeneration of corticospinal, gracile fascicles, spinocerebellar tract
What is CRASH syndrome and which SPG type is it seen?
Corpus callosum hypoplasia, retardation, adducted thumbs, spinal paraplegia, hydrocephalus
seen in SPG-1
What is MASA syndrome and which SPG type is it seen?
Mental retardation, aphasia, shuffling gait, adducted thumbs
seen in SPG-1
True or false: in primary lateral sclerosis, degenerative changes are also seen in percentile gyrus and prefrontal gyrus
True.
Pure lower motor neuron syndrome
Progressive Muscular Atrophy
Protein implicated in Familial Primary Sclerosis
ALSIN
Who si SOD 1 mutation harmful?
It causes a toxic gain of function, enhancing free radical formation
Which motor neuron are relatively resistant to degeneration in ALS? Explain.
Extraocular motor neurons and Onuf’s nuclei
High expression of Calbindin-D28k or Parvalbumin in there motor neurons
Genes implicated in ALS with FTD
genes: c9orf72
FUS and TARDP5P
protein:fused sarcoma
First mutation found in Familial ALS and enzyme involved
SOD-1 mutation, Cu-Zn superoxide dismutase
Gene implicated in childhood SMA
Survival motor neuron 1 (SMN-1) responsible for 95-98% of childhood SMA
Gene implicated in adult SMA
Survival motor neuron 2 (SMN-2)
What is the effect of a higher number of SMN-2 copies in severity of phenotype? (more or less severe?)
less severe phenotype
How would SMA commonly present?
childhood or adult?
symmetric or asymmetric?
distal or proximal?
Childhood, symmetric, proximal
with atrophy
Pathology of SMA
degeneration of anterior horn cells
X linked disorder with unusual pattern of distal muscular atrophy with prominent bulbar signs.
Also with unusual mild sensory neuronopathy due to degeneration of dorsal root ganglion
Kennedy syndrome
Which vitamin deficiency is S-adenosyl-L-methionine (SAM) affected and why is it important?
Seen in vitamin B12 deficiency where there is a defect in methionine synthesis. Methionine is the precursor of SAM.
SAM acts as a methyl donor for methylation of Myelin Basic Protein
Small eosinophilic granular intraneuronal inclusions seen in ALS
Bunina bodies
Most common initial presentation of ALS
foot drop
CK levels in:
SMA I
SMA II
SMA III
SMA I: normal
SMA II: normal
SMA III: elevated x 10
Motor neurone disease predominantly limited to lower cranial nerves?
What is involved mutation?
Fazio- Londe disease
Mutation of SLC52A3: riboflavin transporter
What is Hihrayama’s Disease?
Also knwon as Juvenile Monomelic Amyotrophy
MND that predominantly affects muscles innervated by C7-T1
Genetics: Kennedy Syndrome
CAG trinucleotide repeat expansion in the androgen receptor (AR)
What other disease has a similar genetic mutation as Kennedy Syndrome?
Huntington Disease
More than half of acute spinal epidural abscesses is due to what organism?
Staphylococcus aureus
Pathology of HIV myelopathy
Microvacuolations, spongiform
Areas of preference of these viruses?
Coxsackie?
Herpes zoster?
West Nile?
Coxsackie: anterior horn and motor nuclei of brainstem
Herpes zoster: dorsal ganglion
West Nile: anterior horn
In syphilis: difference of tabes dorsalis vs syphilitic SC meningoencephalitis?
Tabes dorsalis: posterior column affected
Syphilitic SC meningoencephalitis: lateral columns affected
What is Elsberg syndrome?
Acute lumbosacral radiculitis with urinary retention in HSV-2 and CMV
