Curriculum

17 Sections
1548 Lessons
100 Days

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U01.01 Biochemistry
This biochemistry review for USMLE Step 1 covers essential topics including metabolism, enzymology, and molecular biology.
126
U01.02 Immunology
Prepare for USMLE Step 1 Immunology with a comprehensive review covering foundational concepts, immune system mechanisms, and key disorders to solidify your understanding and boost exam readiness.
42
U01.03 Microbiology
Review essential microbiology topics for the USMLE Step 1, covering key pathogens, mechanisms of disease, and antimicrobial principles critical for mastering infectious disease concepts.
172
U01.04 General Pathology
Explore key concepts in general pathology essential for the USMLE Step 1. This review covers fundamental principles, disease mechanisms, and clinical correlations to help you excel in your medical exam.
35
U01.05 Pharmacology
Master key pharmacology concepts for the USMLE Step 1 with a focus on drug mechanisms, therapeutic uses, adverse effects, and interactions essential for high-yield exam performance.
43
U01.06 Public Health Sciences
Review essential Public Health Sciences topics for the USMLE Step 1, covering epidemiology, biostatistics, health policy, and ethics to strengthen your understanding of foundational public health principles.
53
U01.07 Cardiovascular system
Comprehensive review of essential cardiovascular system topics to help you excel in the USMLE Step 1 exam, covering key concepts from anatomy to pathology.
73
U01.08 Endocrine system
Master essential concepts of the endocrine system for USMLE Step 1, covering hormone regulation, feedback mechanisms, and key disorders to boost your exam readiness.
51
U01.09 Gastrointestinal system
Review key topics in the gastrointestinal system for the USMLE Step 1, covering essential concepts in anatomy, physiology, pathology, and pharmacology to reinforce your understanding and test readiness.
131
U01.10 Hematology and Oncology
This review covers essential Hematology and Oncology topics for the USMLE Step 1, including hematopoiesis, coagulation, anemias, leukemias, lymphomas, and neoplastic syndromes.
148
U01.11 Musculoskeletal, Skin, and Connective Tissue
Review essential topics for the USMLE Step 1 Musculoskeletal, Skin, and Connective Tissue systems, covering anatomy, physiology, pathology, and pharmacology fundamentals critical for exam success.
178
U01.12 Neurology and Special Senses
Master the foundational concepts of Neurology and Special Senses with our USMLE Step 1 review, covering essential topics like neuroanatomy, neurophysiology, and sensory pathways.
143
U01.13 Psychiatry
Psychiatry for the USMLE Step 1 focuses on understanding the pathophysiology, diagnosis, and treatment of psychiatric disorders, with an emphasis on key concepts like neurotransmitter imbalances, mental health disorders, and pharmacology.
83
U01.14 Renal System
The Renal System review for USMLE Step 1 covers key concepts including renal physiology, nephron function, acid-base balance, electrolyte regulation, and renal pathology, crucial for understanding both normal and abnormal kidney functions.
81
U01.15 Reproductive System
The USMLE Step 1 Reproductive System review covers key concepts in human reproduction, including anatomy, physiology, endocrinology, and common pathologies affecting both male and female reproductive health.
124
U01.16 Respiratory System
The Respiratory System review for the USMLE Step 1 covers key concepts in anatomy, physiology, pathophysiology, and pharmacology essential for understanding respiratory diseases and treatments.
61
U01.17 Rapid Review
he USMLE Step 1 Rapid Review covers essential topics across multiple disciplines, providing a concise, high-yield overview to reinforce key concepts and optimize your exam preparation.
5

Neurocutaneous disorders

Neurocutaneous disorders are a group of genetic conditions that affect the skin and nervous system. These disorders often present with characteristic skin lesions and neurological symptoms. Below is a detailed overview of some common neurocutaneous disorders, including their genetic basis, presentation, and clinical features.

1. Sturge-Weber Syndrome

Genetics:

Congenital, non-hereditary anomaly of neural crest derivatives.
Somatic mosaicism of an activating mutation in one copy of the GNAQ gene.

Presentation:

Capillary vascular malformation (port-wine stain): Present in the V1/V2 distribution of the trigeminal nerve.
Ipsilateral leptomeningeal angioma: Leads to seizures/epilepsy and intellectual disability.
Episcleral hemangioma: Results in increased intraocular pressure (IOP), potentially causing early-onset glaucoma.

Key Features:

Sporadic inheritance pattern.
Port-wine stain, tram-track calcifications (opposing gyri), unilateral presentation, intellectual disability, glaucoma, and epilepsy.
Mutation in GNAQ gene.

Key Terms	Mnemonic
Port-wine stain	Sturge-Weber: Sporadic, Staining
Tram-track calcifications	Tram tracks in the brain
Intellectual disability	Inability to develop
Glaucoma	Glaucoma onset early

2. Tuberous Sclerosis

Genetics:

Autosomal dominant inheritance.
Mutations in TSC1 on chromosome 9 (hamartin) and TSC2 on chromosome 16 (tuberin).

Presentation:

Hamartomas in the CNS and skin, including:
- Angiofibromas (papules on face).
- Ash-leaf spots (hypopigmented macules on skin).
- Shagreen patches (rough patches on the skin).
- Cardiac rhabdomyomas (benign tumors of the heart).
Renal angiomyolipomas (benign kidney tumors).
Intellectual disability.
Seizures.

Key Features:

Subependymal giant cell astrocytomas and ungual fibromas.
Presence of ash-leaf spots, shagreen patches, and angiofibromas.

Giant cell astrocytomas, are key for diagnosis.	Mnemonic
HAMARTOMAS	Hamartin, Angioma, Mental retardation, Angiomyolipomas, Rhabdomyomas
Subependymal astrocytomas	Giant cell astrocytomas, key for diagnosis.

3. Neurofibromatosis Type I (NF1)

Genetics:

Autosomal dominant inheritance with 100% penetrance.
Mutation in the NF1 tumor suppressor gene on chromosome 17, encoding neurofibromin, a negative regulator of RAS.

Presentation:

Café-au-lait spots.
Cutaneous neurofibromas.
Lisch nodules (pigmented iris hamartomas).
Optic gliomas, pheochromocytomas.
Seizures, focal neurologic signs (often from meningioma).
Bone lesions (e.g., sphenoid dysplasia).

Key Features:

Also called von Recklinghausen disease.
CICLOPSS: Café-au-lait spots, Intellectual disability, Cutaneous neurofibromas, Lisch nodules, Optic gliomas, Pheochromocytomas, Seizures.

Key Terms	Mnemonic
Café-au-lait spots	Café skin lesions.
Intellectual disability	Inability to process.
Lisch nodules	Lesions on the iris.
Optic gliomas	Optic nerve tumors.

4. Neurofibromatosis Type II (NF2)

Genetics:

Autosomal dominant inheritance.
Mutation in the NF2 tumor suppressor gene (Merlin) on chromosome 22.

Presentation:

Bilateral vestibular schwannomas.
Juvenile cataracts.
Meningiomas and ependymomas.

Key Features:

NF2 affects 2 ears, 2 eyes.

Key Terms	Both ears were affected.
Bilateral schwannomas	Both ears affected.
Juvenile cataracts	Juvenile onset.
Meningiomas	Meninges affected.

5. Von Hippel-Lindau Disease

Genetics:

Autosomal dominant inheritance.
Deletion of the VHL gene on chromosome 3p.

Presentation:

Hemangioblastomas in the retina, brainstem, cerebellum, and spine.
Angiomatosis (benign tumors with blood vessels).
Bilateral renal cell carcinomas.
Pheochromocytomas.

Key Features:

Numerous benign and malignant tumors.
Mutation in VHL gene.
HARP: Hemangioblastomas, Angiomatosis, Renal cell carcinomas, Pheochromocytomas.

Key Terms	Mnemonic
Hemangioblastomas	High vascularity, Hyperchromatic nuclei.
Angiomatosis	Tumors with blood vessels.
Renal cell carcinoma	Renal malignancy.

Points to Remember

Genetic inheritance plays a key role in neurocutaneous disorders.
Skin lesions such as café-au-lait spots, port-wine stains, and ash-leaf spots are commonly seen.
Tumors, both benign (e.g., neurofibromas, angiomyolipomas) and malignant (e.g., renal cell carcinoma, pheochromocytomas), are characteristic of many of these conditions.
Early diagnosis and management are crucial for improving quality of life and preventing complications such as seizures and glaucoma.

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USMLE Step 1 Review Series

Curriculum

Neurocutaneous disorders

1. Sturge-Weber Syndrome

Genetics:

Presentation:

Key Features:

2. Tuberous Sclerosis

Genetics:

Presentation:

Key Features:

3. Neurofibromatosis Type I (NF1)

Genetics:

Presentation:

Key Features:

4. Neurofibromatosis Type II (NF2)

Genetics:

Presentation:

Key Features:

5. Von Hippel-Lindau Disease

Genetics:

Presentation:

Key Features:

Points to Remember

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