Wrong

Front 1

what are the clinical features of tay sachs disease?

Back 1

psychomotor retardation, seizures, blindness, no hsm and cherry red spot

Front 2

What are the features of infantile Gaucher's disease?

Back 2

strabismus, ichthyotic skin, hyperextension of the neck, increased tone, dysphagia, myoclonus, massive splenomegaly

Front 3

Where do hyperintensities on MRI tend to occur for Leigh's disease?

Back 3

bilateral cerebral peduncles, thalamus and putamen

Front 4

What movement disorder also has skull thickening 2 to 3 times of normal?

Back 4

DRPLA-triplet repeat disorder Japan and Carolinab

Front 5

What are the features of metachromatic leukodystrophy?

Back 5

CNS + PNS dementia ataxia

Front 6

What is the deficiency in sialidosis type 1?

Back 6

alpha neuraminidase

Front 7

What is the deficiency in sialidosis type 1?

Back 7

alpha neuraminidase

Front 8

What are the clinical features of NCL?

Back 8

progressive myoclonic epilepsy
macular degen

Front 9

What is the neurologic manifestation of Farber's disease?

Back 9

MR

Front 10

What is the deficiency in abetalipoproteinemia?

Back 10

mttp microsomal triglyceride transfer protein

Front 11

What are the most common tumors that metastasize to the spinal cord?

Back 11

Bad lesions like killing glia
breast lung lymphoma kidney colorectal

Front 12

What is the enzyme deficiency associated with GM1 gangliosidosis?

Back 12

beta galactosidase

Front 13

What is Alport's syndrome?

Back 13

sensorineural hearing loss and interstitial nephritis

Front 14

What are the symptoms of sulfite oxidase deficiency?

Back 14

mr, aphasia, seizures, lens subluxation, intractable seizuere

Front 15

What mitochondrial disorder is associated with intestinal pseudoobstruction?

Back 15

MELAS

Front 16

What is the prominent efferent pathway from the amygdala?

Back 16

stria terminalis which teminates in the hypothalamus, nucleus of stria terminalis and medial preoptic nuclei

Front 17

amygdalofugal fibers pass through the inferior thalamic peduncle to the magnocellular division of the mediodorsal nucleus

Back 17

amygdalofugal fibers pass through the inferior thalamic peduncle to the magnocellular division of the mediodorsal nucleus

Front 18

Describe a central cord syndrome.

Back 18

The pattern of bilateral upper extremity weakness affecting distal muscles more than proximal ones with decreased pinprick
sensation with retained fine touch, ie, a dissociated sensory level in a "cape-like" distribution is most suggestive of a central cord
syndrome. A central cord lesion affecting the anterior commissure will affect the crossing spinothalamic tracts resulting in
sensory deficits only at the level of the lesion, without causing more caudal sensory deficits, and will spare vibration and light
touch since the dorsal columns are spared.

Front 19

What arteries are the thalamus supplied by?

Back 19

PCA, PCOMM and posterior choroidal arteries

Front 20

What are the preganglionic parasympathetic outflow arise from in cranial nerves?

Back 20

The cranial preganglionic outflow arises from visceral cell groups of the brain stem associated with the oculomotor, facial,
glossopharyngeal and vagus nerves. All of these are parasympathetic.

Front 21

Which cerebellar peduncle does the nucleus dorsalis of clarke travel in?

Back 21

The posterior spinocerebellar tract arises from the dorsal nucleus of Clarke. The fibers enter the cerebellum via the inferior
cerebellar peduncle. None of the impulses conveyed by this tract reaches conscious levels.

Front 22

What are the fibers from the STN (excitatory or inh) and what are their outputs?

Back 22

Axonal fibers arising from the subthalamic nucleus terminate on the globus pallidum, internal segment and the substantia nigra,
pars reticulata. Neurons of the subthalamus contain glutamate and exert excitatory input into the globus pallidum, pars internal.
This connection is one component of the indirect loop that decreases thalamocortical excitation.

Front 23

What are the 4 structures the anterior choroidal artery supplies?

Back 23

The anterior choroidal artery arises from the internal carotid artery distal to the origin of the posterior communicating artery. It
has a long subarachnoid course, enters the inferior horn of the lateral ventricle through the choroidal fissure, and supplies the
amygdala, hippocampal formation, globus pallidus, and the ventrolateral portion of the posterior limb and the entire
retrolenticular portion of the internal capsule.

Front 24

Which 3 cutaneous nerves are derived from the L1 dermatome?

Back 24

genitofemoral
ilioinguinal
iliohypogastric

Front 25

Which muscles act to flex the hip?

Back 25

iliacus, tensor fascia lata and psoas muscles

Front 26

Which muscles acts to internally rotate the thigh?

Back 26

iliacus which therefore connects to the iliotibial band

Front 27

What is a Chiari type 1 malformation?

Back 27

tonisillar herniation >5 mm below foramen magnum (some soures argue it could be less with symptoms)

Front 28

What is a Chiari type 2 malformation? **

Back 28

1. tectal beaking
2. lumbar myelomeningocoele
3. tonsillar herniation
4. cerebellar vermis displacement
5. downward displacement of torcula herophili (dist. from dandy walker)
+/- clival hypoplasia

Front 29

What is a Dandy-Walker malformation?

Back 29

1. vermis agenesis/hypoplasia
2. posterior fossa cyst with upward displacement of torcula herophili (dist. from chiari 2)
3. corpus callosum agenesis/hypoplasia

Front 30

What is a Chiari type 3 malformation?

Back 30

occipital encephalocoele

Front 31

What is a Chiari type 4 malformation?

Back 31

cerebellar hypoplasia

Front 32

What are the roots of tibialis posterior?

Back 32

L5, S1 (mostly L5)

Front 33

What are the roots of peroneus longus/brevis?

Back 33

L5/S1 (mostly L5)

Front 34

What are the roots for gluteus medius?

Back 34

L4-5-S1

Front 35

What are the roots for gluteus maximus?

Back 35

L5-S1-S2