Neurology Practice Questions

Q: The clinical manifestation of Sydenham chorea includes all except?

Tremors. **Explanation:** Sydenham chorea (St. Vitus’ Dance) is a major Jones criterion for **Acute Rheumatic Fever (ARF)**, resulting from autoimmune basal ganglia inflammation following a Group A Streptococcal infection. **Why "Tremors" is the correct answer:** Tremors are rhythmic, oscillatory movements. In contrast, Sydenham chorea is characterized by **arrhythmic, involuntary, purposeless, and jerky movements** (Chorea). Tremors are not a feature of this condition; their presence should prompt a search for alternative diagnoses like Wilson’s disease or hyperthyroidism. **Analysis of other options:** * **Choreiform movements (Option A):** These are the hallmark of the disease. They typically involve the extremities and face ("grimacing") and disappear during sleep. * **Hung-up reflexes (Option B):** This is a classic neurological sign in Sydenham chorea where the relaxation phase of a deep tendon reflex (usually the knee jerk) is prolonged due to a superimposed choreic contraction. * **Emotional lability (Option C):** Neuropsychiatric symptoms, including inappropriate crying, laughing, or irritability, often precede the motor symptoms and are a core clinical manifestation. **High-Yield Clinical Pearls for NEET-PG:** * **Milkmaid’s Grip:** Irregular contractions of the hand muscles while squeezing the examiner’s fingers. * **Jack-in-the-box Tongue:** Inability to keep the tongue protruded (it darts in and out). * **Pronator Sign:** When arms are extended overhead, the palms turn outwards due to hypotonia. * **Latent Period:** It has the longest latent period of all ARF manifestations (1–6 months). * **Gender:** It is more common in adolescent females.

Q: Which of the following is true regarding Gower sign?

Use of hands on thighs to stand up. **Explanation:** **Gower sign** is a classic clinical maneuver observed in children with proximal muscle weakness, particularly involving the pelvic girdle and hip extensors (Gluteus maximus). 1. **Why Option A is correct:** To rise from a supine or sitting position, the patient must compensate for weak hip extensors. They first assume a "tripod" position, then use their hands to "climb up" their own legs and thighs to reach an upright posture. This specific action of using the hands on the thighs is the defining physical finding of the sign. 2. **Why Options B and C are "Incorrect" (in the context of this specific question):** While Gower sign is indeed seen in **Duchenne Muscular Dystrophy (DMD)** and is caused by **proximal muscle weakness**, the question asks "Which of the following is true regarding Gower sign?" In many standardized medical exams, if the options describe the definition, the etiology, and the association, and an "All of the above" option exists, it is usually the intended answer. However, if the key identifies **Option A** as the specific correct answer, it emphasizes the **clinical definition** of the sign over its associations. **High-Yield Clinical Pearls for NEET-PG:** * **Primary Muscle Involved:** Gluteus maximus (hip extensor). * **Differential Diagnosis:** While pathognomonic for DMD, it is also seen in Becker muscular dystrophy, Spinal Muscular Atrophy (SMA), and inflammatory myopathies like Dermatomyositis. * **DMD Genetics:** X-linked recessive; deletion of the Dystrophin gene (largest human gene) on chromosome Xp21. * **Associated Finding:** Pseudohypertrophy of the calves (due to fatty/fibrous infiltration of the gastrocnemius). * **Laboratory Marker:** Creatine Kinase (CK) levels are massively elevated (often >10,000 U/L) in the early stages of DMD.

Q: MacEwen sign is seen in which condition?

Hydrocephalus. **Explanation:** **MacEwen Sign (Cracked-Pot Sign)** is a clinical diagnostic sign used in pediatrics to detect increased intracranial pressure (ICP) and separation of cranial sutures. **Why Hydrocephalus is correct:** In **Hydrocephalus**, the accumulation of cerebrospinal fluid (CSF) leads to ventricular enlargement and increased ICP. In infants and young children whose cranial sutures have not yet fused, this pressure causes the sutures to widen. When the skull is percussed (tapped) near the junction of the frontal, temporal, and parietal bones, it produces a resonant, "cracked-pot" sound. This is due to the altered transmission of vibrations through the separated sutures and thinned table of the skull. **Why other options are incorrect:** * **Encephalitis & Meningitis:** While these inflammatory conditions can cause increased ICP and a bulging fontanelle, they typically do not cause the chronic, significant separation of sutures required to produce a positive MacEwen sign unless they lead to secondary obstructive hydrocephalus. * **Microcephaly:** This condition involves an abnormally small head due to failure of brain growth. The sutures often close prematurely (craniosynostosis) or are tightly apposed, which is the physiological opposite of the mechanism behind MacEwen sign. **High-Yield Clinical Pearls for NEET-PG:** * **Setting:** MacEwen sign is best elicited in infants before the closure of fontanelles, but it can also be seen in older children with thinned skull bones. * **Other signs of Hydrocephalus:** Setting-sun sign (downward gaze), bulging anterior fontanelle, and dilated scalp veins. * **Transillumination Test:** Used in infants to differentiate hydrocephalus from hydranencephaly. * **Cushing’s Triad:** A late sign of increased ICP consisting of bradycardia, hypertension, and irregular respirations.

Q: A 3-year-old boy presents with a several-month history of increasing difficulty walking. His parents note an increased inward curvature of the lower spine during ambulation and a more waddling gait. Physical examination reveals these findings, along with enlarged calves. What is the most likely diagnosis?

Muscular dystrophy. The clinical presentation described is a classic textbook case of **Duchenne Muscular Dystrophy (DMD)**, the most common hereditary neuromuscular disease in children. ### **Explanation of the Correct Answer** The diagnosis is based on a triad of physical findings: 1. **Pseudohypertrophy of Calves:** The "enlarged calves" are not due to muscle strength but the replacement of muscle tissue with fat and connective tissue (fibrosis). 2. **Waddling Gait:** This occurs due to weakness of the gluteus medius muscle (proximal muscle weakness), causing the pelvis to drop on the unsupported side. 3. **Lumbar Lordosis:** The "inward curvature of the spine" is a compensatory mechanism to maintain the center of gravity over the base of support due to weakened hip extensors (gluteus maximus). ### **Why Other Options are Incorrect** * **Occult Spina Bifida:** Usually presents with cutaneous markers (tuft of hair, dimple) over the lower back. While it can cause gait issues, it does not cause calf pseudohypertrophy. * **Brain Tumor:** Typically presents with signs of increased intracranial pressure (headache, vomiting) or ataxia. It would not cause isolated calf enlargement. * **Guillain-Barre Syndrome (GBS):** This is an **acute**, ascending paralysis following a viral illness. The "several-month history" in this vignette rules out the acute nature of GBS. ### **High-Yield Clinical Pearls for NEET-PG** * **Gower’s Sign:** The child uses their hands to "climb up" their own legs to stand from a sitting position (indicates proximal muscle weakness). * **Genetics:** X-linked recessive; mutation in the **Dystrophin gene** (the largest known human gene). * **Diagnosis:** Gold standard is Genetic testing (Deletion/Duplication analysis); Screening test is highly elevated **Serum Creatine Kinase (CK)**. * **Complications:** Dilated cardiomyopathy and respiratory failure are the leading causes of death.

Q: Which of the following conditions is associated with early complete fusion of all of the cranial sutures?

Ape syndrome. **Explanation:** The correct answer is **Apert syndrome** (often referred to in exams as "Ape syndrome"). **1. Why Apert Syndrome is Correct:** Apert syndrome is a Type I acrocephalosyndactyly syndrome caused by a mutation in the **FGFR2 gene**. It is characterized by **craniosynostosis**, which is the premature fusion of cranial sutures. Specifically, it often involves the early complete fusion of multiple sutures (coronal, sagittal, etc.), leading to a "tower-shaped" skull (turricephaly) and midface hypoplasia. A hallmark diagnostic feature is **symmetric syndactyly** (mitten-hand deformity) of the hands and feet. **2. Why the Other Options are Incorrect:** * **Chiari Malformations:** These are structural defects in the cerebellum and brainstem where brain tissue extends into the spinal canal. They are not primarily disorders of cranial suture fusion. * **Craniospinal Dysraphism:** This refers to a spectrum of neural tube defects (like spina bifida or encephaloceles) resulting from the failure of the neural tube to close during embryogenesis, rather than premature suture fusion. * **Osteosclerosis:** This is a general term for increased bone density (seen in conditions like Albers-Schönberg disease). While it affects bone mineralization, it does not specifically cause the early complete fusion of all cranial sutures as a primary feature. **Clinical Pearls for NEET-PG:** * **Crouzon Syndrome:** Similar to Apert but **without** syndactyly. * **Scaphocephaly:** Most common type of craniosynostosis (premature fusion of the **sagittal** suture). * **Apert Triad:** Craniosynostosis, midface hypoplasia, and symmetric syndactyly. * **Inheritance:** Most cases are sporadic, but it can be inherited in an **Autosomal Dominant** fashion.

Q: In which of the following conditions is the given appearance of calves typically observed?

Muscular dystrophy. ***Muscular dystrophy*** - **Calf pseudohypertrophy** is the classic hallmark of **Duchenne Muscular Dystrophy (DMD)**, caused by replacement of muscle fibers with **fat and fibrous tissue**. - This creates enlarged but weak calves, typically observed in boys aged 3-5 years with **progressive muscle weakness** and **delayed motor milestones**. *Spinal muscular atrophy* - Presents with **muscle wasting** and **atrophy**, not enlargement of the calves. - Characterized by **hypotonia**, **areflexia**, and **tongue fasciculations** due to anterior horn cell degeneration. *Myopathy* - Generally causes **muscle weakness** without the characteristic **calf enlargement** seen in DMD. - May present with **proximal muscle weakness** but lacks the specific pseudohypertrophic appearance. *Peripheral neuropathy* - Results in **distal muscle wasting** and **sensory loss**, not calf enlargement. - Typically presents with **glove and stocking** distribution of symptoms and **reduced reflexes**.

Q: A 10-year-old boy, who was apparently normal at birth, had delayed walking and difficulty in walking. As years passed, weakness increased, and he is now wheelchair-bound. What is your diagnosis?

Duchenne muscular dystrophy. **Explanation:** The clinical presentation of a 10-year-old boy with progressive muscle weakness leading to wheelchair dependency is classic for **Duchenne Muscular Dystrophy (DMD)**. **Why Duchenne Muscular Dystrophy (DMD) is correct:** DMD is an X-linked recessive disorder caused by the absence of the protein **dystrophin**. While patients appear normal at birth, symptoms typically manifest between ages 3–5 with delayed walking, frequent falls, and difficulty climbing stairs. The weakness is progressive and proximal, typically leading to the loss of independent ambulation (wheelchair bound) by age **10–12 years**. **Analysis of Incorrect Options:** * **Congenital Muscular Dystrophy:** Symptoms are present **at birth** or within the first few months of life (hypotonia, "floppy infant"), unlike this patient who was normal at birth. * **Becker Muscular Dystrophy (BMD):** This is a milder form where dystrophin is reduced or truncated rather than absent. Onset is later (usually >7 years), and patients typically remain ambulatory well into their **20s or 30s**. * **Limb-Girdle Muscular Dystrophy (LGMD):** This group of disorders affects the hip and shoulder girdles but lacks the specific X-linked inheritance pattern and rapid progression seen in DMD. It usually presents in late childhood or adolescence. **High-Yield Clinical Pearls for NEET-PG:** * **Gower’s Sign:** The child uses their hands to "climb up" their own legs to stand up (due to proximal muscle weakness). * **Pseudohypertrophy:** The calves appear large but are actually replaced by fat and connective tissue. * **Diagnosis:** Gold standard is **Genetic testing** (deletion of DMD gene). Screening shows massively elevated **Serum Creatine Kinase (CK)**. * **Cause of death:** Usually respiratory failure or dilated cardiomyopathy in the late teens or early 20s.

Q: Neurofibromatosis type 1 is most commonly associated with which of the following tumors?

Optic pathway glioma. **Explanation:** **Neurofibromatosis Type 1 (NF1)**, also known as von Recklinghausen disease, is an autosomal dominant neurocutaneous syndrome caused by a mutation in the *NF1* gene on **chromosome 17**. This gene encodes neurofibromin, a tumor suppressor that regulates the Ras signaling pathway. **Why Optic Pathway Glioma (OPG) is correct:** OPGs are the most common central nervous system tumors in children with NF1, occurring in approximately **15–20%** of patients. These are typically low-grade **pilocytic astrocytomas** (WHO Grade I). While often asymptomatic, they can lead to vision loss or precocious puberty (if the hypothalamus is involved). Their strong association makes OPG a formal NIH diagnostic criterion for NF1. **Analysis of Incorrect Options:** * **Brain stem gliomas:** While these occur with increased frequency in NF1 patients compared to the general population, they are significantly less common than optic pathway gliomas. * **Subependymal pilocytic astrocytoma:** This is likely a distractor. **Subependymal Giant Cell Astrocytomas (SEGA)** are classically associated with **Tuberous Sclerosis**, not NF1. * **Glioblastoma multiforme (GBM):** GBM is a high-grade (WHO Grade IV) malignancy. While NF1 patients have a higher lifetime risk of high-grade gliomas, the low-grade optic glioma is the most characteristic and frequent association. **High-Yield Clinical Pearls for NEET-PG:** * **Lisch Nodules:** Iris hamartomas (most common ocular finding in NF1). * **Sphenoid Wing Dysplasia:** A characteristic skeletal finding. * **Plexiform Neurofibroma:** Pathognomonic for NF1; carries a risk of transformation into **Malignant Peripheral Nerve Sheath Tumor (MPNST)**. * **NF2 Association:** Remember that **Bilateral Acoustic Neuromas** (Vestibular Schwannomas) are the hallmark of NF2 (Chromosome 22), not NF1.

Question 1

A child presented with clinical features of demyelination. The chances of progression to Multiple Sclerosis is least with which of the following?

Accepted Answer

Absent oligoclonal bands

Answer

Bilateral visual loss

Answer

Poor recovery

Answer

Complete cord transaction

Question 2

The clinical manifestation of Sydenham chorea includes all except?

Accepted Answer

Tremors

Answer

Choreiform movements

Answer

Hung up reflexes

Answer

Emotional liability

Question 3

Which of the following is true regarding Gower sign?

Accepted Answer

Use of hands on thighs to stand up

Answer

Seen in Duchenne muscular dystrophy

Answer

Due to proximal muscle weakness

Answer

All of the above

Question 4

MacEwen sign is seen in which condition?

Accepted Answer

Hydrocephalus

Answer

Encephalitis

Answer

Meningitis

Answer

Microcephaly

Question 5

All of the following are true about meningocele except?

Accepted Answer

Immediate surgical treatment is required to prevent meningitis in all patients.

Answer

The spinal cord is usually normal and assumes a normal position in the spinal canal.

Answer

Most meningoceles are well-covered with skin and pose no threat to the patient.

Answer

Brilliantly transilluminant.

Question 6

A 3-year-old boy presents with a several-month history of increasing difficulty walking. His parents note an increased inward curvature of the lower spine during ambulation and a more waddling gait. Physical examination reveals these findings, along with enlarged calves. What is the most likely diagnosis?

Accepted Answer

Muscular dystrophy

Answer

Occult spina bifida

Answer

Brain tumor

Answer

Guillain-Barre syndrome

Question 7

Which of the following conditions is associated with early complete fusion of all of the cranial sutures?

Accepted Answer

Ape syndrome

Answer

Chiari malformations

Answer

Craniospinal dysraphism

Answer

Osteosclerosis

Question 8

In which of the following conditions is the given appearance of calves typically observed?

Accepted Answer

Muscular dystrophy

Answer

Spinal muscular atrophy

Answer

Myopathy

Answer

Peripheral neuropathy

Question 9

A 10-year-old boy, who was apparently normal at birth, had delayed walking and difficulty in walking. As years passed, weakness increased, and he is now wheelchair-bound. What is your diagnosis?

Accepted Answer

Duchenne muscular dystrophy

Answer

Congenital muscular dystrophy

Answer

Becker muscular dystrophy

Answer

Limb-girdle muscular dystrophy

Question 10

Neurofibromatosis type 1 is most commonly associated with which of the following tumors?

Accepted Answer

Optic pathway glioma

Answer

Brain stem gliomas

Answer

Subependymal pilocytic astrocytoma

Answer

Glioblastoma multiforme

Neurology — MCQs

Neurology — MCQs

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