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USMLE STEP 1 Neurology Exam Practice Questions and Answers (100% Pass)

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USMLE STEP 1 Neurology Exam Practice Questions and Answers (100% Pass) The notochord induces what to differentiate into what? - Answer️️ - Induces overlying ECTODERM to differentiate into NEUROECTODERM and form NUERAL PLATE Neural plate then gives rise to? - Answer️️ -Neural tube and neural crest cells Notochord becomes what? - Answer️️ -Nucleus pulposus of the intervertebral disks in adults Alar Plate - Answer️️ -Dorsal [Sensory] Same orientation as spinal cord Basal Plate - Answer️️ -Ventral [Motor] Same orientation as the spinal cord How does FGF affect chordin and noggin? - Answer️️ -FGF regulates chordin and noggin to down regulate BMP which leads to neural plate induction Three Primary Vesicles - Answer️️ -1. Forebrain [Prosencephalon] 2. Midbrain [Mesencephalon] 3. Hindbrain [Rhombencphaln] ©SOPHIABENNET@2024/2025 Tuesday, August 20, 2024 10:21 AM 2 The Forebrain gives rise to: - Answer️️ -1. Telencephalon [Cerebral hemispheres, lateral ventricles] 2. Diencephalon [Thalamus, Third Ventricle] The Midbrain gives rise to: - Answer️️ -1. Mesencephalon [Midbrain, aqueduct] The Hindbrain gives rise to: - Answer️️ -1. Metencephalon [Pons, cerebellum, upper part of the fourth ventricle] 2. Myelencephalon [Medulla, Lower part of the fourth ventricle] CNS/PNS Origins - Answer️️ -Neuroectoderm = CNS neurons, ependymal cell [Inner lining of ventricles, makes CSF], oligodendrocytes, astrocytes Neural crest cells = PNS, schwann cells Mesoderm = Microglia [Like Macrophages, originate from Mesoderm] Neural tube defects - Answer️️ -NEUROPORES fail to fuse [4th week] → Persistent connection between amniotic cavity and spinal canal Associated with: 1. Low folate levels before conception and during pregnancy 2. ↑ a-fetoprotein levels [AFP] in amniotic and maternal serum 3. ↑ AChE in amniotic fluid [Helpful confirmatory test] -- Fetal AChe in CSF transudates across defect into amniotic fluid ©SOPHIABENNET@2024/2025 Tuesday, August 20, 2024 10:21 AM 3 Spina bifida occulta - Answer️️ -Failure of bony spinal canal to close, NO STRUCTURAL HERNIATION Usually seen at lower vertebral levels DURA INTACT Associated w/ tuft of hair or skin dimple at level of bony defect NORMAL AFP Meningocele - Answer️️ -Meninges [BUT NO NEURAL TISSUES] herniates through bony defect Meningomyelocele - Answer️️ -Meninges and neural tissue herniate through bony defect Associated w/ Arnold-Chiari Type II Malformation Anencephaly - Answer️️ -Malformation of the ANTERIOR NEURAL TUBE → 1. No forebrain 2. Open calvarium Clinical Findings: 1. ↑ a-fetoprotein levels [AFP] 2. Polyhydramnios [No swallowing center in brain] 3. Associated w/ MATERNAL TYPE I DIABETES 4. Maternal folate supplementation ↓ risk ©SOPHIABENNET@2024/2025 Tuesday, August 20, 2024 10:21 AM 4 Holoprosencephaly - Answer️️ -Failure of LEFT AND RIGHT HEMISPHERES TO SEPARATE Usually occurs during the 5th and 6th week May be related to mutations in the SONIC HEDGEHOG

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©SOPHIABENNET@2024/2025 Tuesday, August 20, 2024 10:21 AM


USMLE STEP 1 Neurology Exam Practice
Questions and Answers (100% Pass)

The notochord induces what to differentiate into what? - Answer✔️✔️-
Induces overlying ECTODERM to differentiate into NEUROECTODERM
and form NUERAL PLATE

Neural plate then gives rise to? - Answer✔️✔️-Neural tube and neural crest
cells

Notochord becomes what? - Answer✔️✔️-Nucleus pulposus of the
intervertebral disks in adults

Alar Plate - Answer✔️✔️-Dorsal [Sensory]

Same orientation as spinal cord

Basal Plate - Answer✔️✔️-Ventral [Motor]

Same orientation as the spinal cord

How does FGF affect chordin and noggin? - Answer✔️✔️-FGF regulates
chordin and noggin to down regulate BMP which leads to neural plate
induction

Three Primary Vesicles - Answer✔️✔️-1. Forebrain [Prosencephalon]

2. Midbrain [Mesencephalon]

3. Hindbrain [Rhombencphaln]




1

, ©SOPHIABENNET@2024/2025 Tuesday, August 20, 2024 10:21 AM

The Forebrain gives rise to: - Answer✔️✔️-1. Telencephalon [Cerebral
hemispheres, lateral ventricles]

2. Diencephalon [Thalamus, Third Ventricle]

The Midbrain gives rise to: - Answer✔️✔️-1. Mesencephalon [Midbrain,
aqueduct]

The Hindbrain gives rise to: - Answer✔️✔️-1. Metencephalon [Pons,
cerebellum, upper part of the fourth ventricle]

2. Myelencephalon [Medulla, Lower part of the fourth ventricle]

CNS/PNS Origins - Answer✔️✔️-Neuroectoderm = CNS neurons,
ependymal cell [Inner lining of ventricles, makes CSF], oligodendrocytes,
astrocytes

Neural crest cells = PNS, schwann cells

Mesoderm = Microglia [Like Macrophages, originate from Mesoderm]

Neural tube defects - Answer✔️✔️-NEUROPORES fail to fuse [4th week] →
Persistent connection between amniotic cavity and spinal canal

Associated with:

1. Low folate levels before conception and during pregnancy

2. ↑ a-fetoprotein levels [AFP] in amniotic and maternal serum

3. ↑ AChE in amniotic fluid [Helpful confirmatory test]

-- Fetal AChe in CSF transudates across defect into amniotic fluid



2

, ©SOPHIABENNET@2024/2025 Tuesday, August 20, 2024 10:21 AM

Spina bifida occulta - Answer✔️✔️-Failure of bony spinal canal to close, NO
STRUCTURAL HERNIATION

Usually seen at lower vertebral levels

DURA INTACT

Associated w/ tuft of hair or skin dimple at level of bony defect

NORMAL AFP

Meningocele - Answer✔️✔️-Meninges [BUT NO NEURAL TISSUES]
herniates through bony defect

Meningomyelocele - Answer✔️✔️-Meninges and neural tissue herniate
through bony defect

Associated w/ Arnold-Chiari Type II Malformation

Anencephaly - Answer✔️✔️-Malformation of the ANTERIOR NEURAL
TUBE →

1. No forebrain

2. Open calvarium

Clinical Findings:

1. ↑ a-fetoprotein levels [AFP]

2. Polyhydramnios [No swallowing center in brain]

3. Associated w/ MATERNAL TYPE I DIABETES

4. Maternal folate supplementation ↓ risk


3

, ©SOPHIABENNET@2024/2025 Tuesday, August 20, 2024 10:21 AM

Holoprosencephaly - Answer✔️✔️-Failure of LEFT AND RIGHT
HEMISPHERES TO SEPARATE

Usually occurs during the 5th and 6th week

May be related to mutations in the SONIC HEDGEHOG signaling pathway

Moderate form = Cleft lip/palate

Severe form = Cyclopia

Seen in:

1. PATAU SYNDROME

2. FETAL ALCOHOL SYNDROME

Chiari Type II Malformation - Answer✔️✔️-Posterior fossa malformation

Significant HERNIATION of the CEREBELLAR TONSILS and VERMIS
through FORAMEN MAGNUM with AQUEDUCTAL STENOSIS and
HYDROCEPHALUS

Patients often present w/:

1. Lumbosacral meningomyelocele

2. Paralysis below the defect

Dandy-Walker Malformation - Answer✔️✔️-AGENESIS of CEREBELLAR
VERMIS with CYSTIC ENLARGEMENT of the 4th ventricle

Fills enlarged posterior fossa

Associated w/:


4

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