Neurology Quiz: ALS, Chiari & CES Symptoms
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Questions and Answers

What is a key symptom that differentiates ALS from Chiari malformation?

  • Occipital headaches
  • Progressive motor weakness
  • Sensory loss (correct)
  • Cerebellar dysfunction

Which MRI finding is typically associated with Chiari malformation?

  • Increased intracranial pressure
  • Downward herniation of the cerebellar tonsils (correct)
  • Lateral ventricle enlargement
  • Cervical spine degeneration

What symptom commonly distinguishes a spinal cord tumor from syringomyelia?

  • Rapid progression of neurological deficits (correct)
  • The cape-like distribution of symptoms
  • Involvement of sensory modalities
  • Dissociated sensory loss

What distribution pattern of sensory loss is characteristic of peripheral neuropathy?

<p>Stocking-glove distribution (B)</p> Signup and view all the answers

Which imaging modality is the first line for diagnosing syringomyelia?

<p>MRI of the cervical spine (D)</p> Signup and view all the answers

What pattern of symptoms should prompt consideration of peripheral neuropathy?

<p>Distal sensory loss without evidence of central involvement (D)</p> Signup and view all the answers

What is a primary risk of delaying surgery in cases of CES?

<p>Permanent neurological deficits (C)</p> Signup and view all the answers

What is the common pitfall when differentiating syringomyelia from multiple sclerosis (MS)?

<p>Identifying the cape-like distribution of sensory loss (B)</p> Signup and view all the answers

Which symptom is considered a 'red flag' indicating the need for urgent referral in CES?

<p>Saddle anesthesia (D)</p> Signup and view all the answers

What is the definitive investigation for diagnosing CES?

<p>MRI (C)</p> Signup and view all the answers

Which symptom is most closely associated with Chiari malformation?

<p>Neck pain (B)</p> Signup and view all the answers

Which of the following symptoms is NOT characteristic of CES?

<p>Post-operative physiotherapy (A)</p> Signup and view all the answers

What role does rehabilitation play in the care of CES patients?

<p>It improves mobility and strength (A)</p> Signup and view all the answers

What is the primary focus when differentiating CES from lumbar disc herniation?

<p>Presence of saddle anesthesia and bowel/bladder dysfunction (C)</p> Signup and view all the answers

Which of the following management steps is crucial in CES?

<p>Immediate surgical consultation (D)</p> Signup and view all the answers

What potential ongoing issue may patients experience despite timely surgical intervention for CES?

<p>Residual bladder, bowel, or sexual dysfunction (D)</p> Signup and view all the answers

What is the most common type of Normal Pressure Hydrocephalus (NPH)?

<p>Idiopathic NPH (D)</p> Signup and view all the answers

Which of the following conditions can lead to Secondary NPH?

<p>Head trauma (D)</p> Signup and view all the answers

What clinical feature is typically the earliest symptom of Normal Pressure Hydrocephalus?

<p>Magnetic gait (B)</p> Signup and view all the answers

What neuroimaging finding is characteristic of NPH?

<p>Ventricular enlargement without cortical atrophy (C)</p> Signup and view all the answers

What is the purpose of a lumbar puncture in diagnosing NPH?

<p>To assess response to CSF removal (D)</p> Signup and view all the answers

Which age group is at a higher risk of developing Normal Pressure Hydrocephalus?

<p>Individuals over 60 years (A)</p> Signup and view all the answers

Which of the following is NOT a feature of cognitive decline in NPH?

<p>Rapid cognitive deterioration (D)</p> Signup and view all the answers

What is the typical finding in CSF analysis during lumbar puncture for NPH?

<p>Normal pressure and cell count (C)</p> Signup and view all the answers

What is the typical imaging finding in Normal Pressure Hydrocephalus (NPH)?

<p>Ventriculomegaly without significant cortical atrophy (A)</p> Signup and view all the answers

Which symptom is not typically associated with a subdural hematoma?

<p>Urinary incontinence (D)</p> Signup and view all the answers

What is the mainstay treatment for Normal Pressure Hydrocephalus (NPH)?

<p>Ventriculoperitoneal (VP) shunting (D)</p> Signup and view all the answers

Which combination of symptoms is key for diagnosing Normal Pressure Hydrocephalus (NPH)?

<p>Gait disturbance, urinary incontinence, and cognitive decline (C)</p> Signup and view all the answers

In differentiating NPH from Parkinson’s disease, which feature is indicative of NPH?

<p>Magnetic gait with minimal arm swing (B)</p> Signup and view all the answers

What follow-up measure is crucial after VP shunt placement?

<p>Regular monitoring for complications (D)</p> Signup and view all the answers

Which of the following is a common misconception when diagnosing NPH?

<p>It presents similarly to Alzheimer’s disease (B)</p> Signup and view all the answers

What type of changes are observed in EEG during Creutzfeldt-Jakob Disease?

<p>Periodic sharp wave complexes (A)</p> Signup and view all the answers

What is the primary symptom of Restless Leg Syndrome (RLS)?

<p>An uncontrollable urge to move the legs (D)</p> Signup and view all the answers

During which time period do the symptoms of RLS primarily occur?

<p>Nighttime or periods of inactivity (D)</p> Signup and view all the answers

Which age group is most commonly affected by Restless Leg Syndrome?

<p>Middle-aged and older adults (A)</p> Signup and view all the answers

What type of disorder is Restless Leg Syndrome classified as?

<p>Neurological disorder (B)</p> Signup and view all the answers

Which neurological pathway is believed to be involved in the aetiology of RLS?

<p>Dopaminergic pathways (D)</p> Signup and view all the answers

What percentage of the general population is estimated to be affected by Restless Leg Syndrome?

<p>5-15% (D)</p> Signup and view all the answers

Which gender is reported to be more commonly affected by RLS?

<p>Women (D)</p> Signup and view all the answers

What effect does resting have on the symptoms of Restless Leg Syndrome?

<p>Symptoms worsen with rest (A)</p> Signup and view all the answers

Which symptom is most prominently associated with Alzheimer's Disease in the early stages?

<p>Language difficulties (A)</p> Signup and view all the answers

What is the key differentiator for Parkinson's Disease compared to other conditions?

<p>Presence of motor symptoms like rigidity (B)</p> Signup and view all the answers

Which factor is a critical indicator of Vascular Dementia?

<p>Multiple lacunar infarcts on imaging (A)</p> Signup and view all the answers

What unique symptom might suggest Lewy Body Dementia?

<p>Visual hallucinations (C)</p> Signup and view all the answers

What should you consider if a patient recently changed medications and exhibits cognitive impairment?

<p>Medication side effects (A)</p> Signup and view all the answers

In which scenario is frontotemporal dementia most likely to be considered?

<p>If behavioral changes and personality alterations are more prominent than memory loss (B)</p> Signup and view all the answers

What type of symptoms distinguishes Vascular Dementia from other forms of dementia?

<p>Stepwise cognitive decline with focal neurological signs (A)</p> Signup and view all the answers

What is an important imaging finding that indicates Alzheimer's Disease?

<p>Cortical atrophy, especially in the temporal lobes (C)</p> Signup and view all the answers

Flashcards

Syringomyelia

A rare neurological condition characterized by a fluid-filled cavity (syrinx) within the spinal cord. It typically affects the central nervous system, causing sensory loss and motor weakness, predominantly in a "cape-like" distribution.

Amyotrophic Lateral Sclerosis (ALS)

An inherited disorder that affects the motor neurons in the brain and spinal cord, leading to progressive muscle weakness and atrophy without sensory loss.

Chiari Malformation

A condition where the cerebellar tonsils (part of the cerebellum) herniate into the spinal canal. It often leads to syringomyelia, especially Type I, and causes symptoms like occipital headaches, neck pain, and signs of cerebellar dysfunction.

Spinal Cord Tumor

A tumor within the spinal cord that can present with symptoms similar to syringomyelia, including motor and sensory deficits. However, back pain and rapid progression are often present.

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Peripheral Neuropathy

A group of disorders that affect the peripheral nerves, leading to sensory loss and weakness. It typically presents with a stocking-glove distribution of sensory loss, affecting both pain and vibration sensations.

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Cape-Like Sensory Loss

A distinctive pattern of sensory loss that occurs in syringomyelia, affecting pain and temperature sensation in a "cape-like" distribution over the shoulders and upper back.

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Dissociated Sensory Loss

A type of sensory loss particularly common in syringomyelia, where there is a loss of pain and temperature sensation but preserved touch, proprioception, and vibration senses.

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MRI of the Cervical Spine

The initial diagnostic modality for syringomyelia, providing detailed information about the spinal cord structure and the presence of a syrinx.

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What is Normal Pressure Hydrocephalus (NPH)?

A condition where there is an abnormal accumulation of cerebrospinal fluid (CSF) in the brain's ventricles, leading to ventricular enlargement without a significant increase in intracranial pressure.

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What is Idiopathic (Primary) NPH?

This type of NPH is the most common and occurs when the cause of CSF buildup is unknown. It's often linked to age-related changes in CSF absorption.

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What is Secondary NPH?

This type of NPH occurs when a known underlying condition, like head trauma or brain tumor, obstructs the flow or absorption of CSF.

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What is the most common symptom of NPH?

The most common symptom is a difficulty with walking, often described as a 'magnetic gait'—the feet seem stuck to the floor.

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What is another common symptom of NPH?

It starts as a feeling of urgency or frequency and may progress to loss of bladder control.

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What are the cognitive symptoms of NPH?

Memory problems, slow thinking, and lack of motivation can be symptoms. It's often more gradual than Alzheimer's disease.

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How is NPH diagnosed with neuroimaging?

A brain scan, usually an MRI or CT, helps identify enlarged ventricles (ventriculomegaly) while the brain's tissue remains relatively normal.

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Why is a lumbar puncture done?

A spinal tap is used to remove some CSF to see if removing fluid improves symptoms. It helps predict how well a shunt might work.

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Alzheimer's Disease

A neurodegenerative disease with early prominent memory loss, language difficulties, and visuospatial deficits.

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Parkinson's Disease

A neurodegenerative disease characterized by resting tremors, bradykinesia, rigidity, and postural instability.

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Vascular Dementia (VaD)

A type of dementia characterized by a stepwise decline in cognitive function often with focal neurological deficits.

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Frontotemporal dementia (FTD)

A dementia characterized by behavioural changes and personality changes more prominent than memory loss.

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Lewy Body Dementia (LBD)

A dementia that causes visual hallucinations, fluctuations in cognition, and Parkinsonism.

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Alzheimer's Disease (Imaging)

Cognitive impairment and urinary incontinence may be symptoms. MRI shows cortical atrophy in the temporal lobes.

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Vascular Dementia (Imaging)

Imaging typically shows multiple lacunar infarcts or white matter changes.

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Parkinson's Disease (Symptoms)

Parkinsonism is a key feature, and cognitive impairment and urinary incontinence occur later.

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Subdural Hematoma

A collection of blood that forms between the dura mater and the arachnoid mater of the brain, often caused by head trauma.

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Creutzfeldt-Jakob Disease

A rapidly progressive dementia characterized by severe cognitive decline, involuntary muscle jerks, and specific EEG changes.

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Normal Pressure Hydrocephalus (NPH)

A condition where the brain ventricles are enlarged, leading to gait disturbance, urinary incontinence, and cognitive decline.

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Ventriculoperitoneal (VP) Shunting

Treatment for NPH that involves surgically inserting a shunt to drain excess cerebrospinal fluid from the ventricles.

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Magnetic Gait

A gait pattern seen in NPH characterized by short, shuffling steps with minimal arm swing.

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Ventriculomegaly with Preserved Cortical Mantle

A distinctive imaging finding in NPH where the brain ventricles are enlarged but the brain tissue itself appears normal.

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NPH Triad

The classic triad of symptoms seen in NPH: gait disturbance, urinary incontinence, and cognitive decline.

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Cauda Equina Syndrome (CES)

A condition where the spinal cord is compressed, typically by a herniated disc, causing neurological deficits like saddle anesthesia, bowel and bladder dysfunction.

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Saddle Anesthesia

A loss of sensation in the area around the anus and genitals, resembling a saddle shape.

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MRI of the Lumbar Spine

In CES, the primary diagnostic tool to confirm the diagnosis and reveal the extent of compression.

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Urgent Surgical Consultation

CES is a surgical emergency requiring immediate decompression to prevent permanent neurological damage.

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Rehabilitation Post CES

Physiotherapy and occupational therapy can help improve mobility, strength, and daily life functions after CES.

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Residual Symptoms in CES

Even with prompt surgery, some individuals might experience ongoing bowel, bladder, or sexual dysfunction.

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Differentiating CES from Lumbar Disc Herniation

Distinguish CES from other conditions that cause back pain and neurological deficits, such as a lumbar disc herniation.

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Red Flags in CES

Be mindful of red flag symptoms in CES, such as saddle anesthesia, urinary retention, and bilateral lower limb weakness.

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Restless Leg Syndrome (RLS)

A neurological disorder characterized by an irresistible urge to move the legs, often accompanied by uncomfortable sensations.

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Akathisia

A symptom of RLS, described as a compelling need to move the legs to relieve an uncomfortable sensation.

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Nocturnal Symptoms

The primary symptom of RLS, where the urge to move is strongest at night, often interfering with sleep.

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Worsening Symptoms with Rest

The effect of RLS where discomfort increases when the patient is lying down or sitting for extended periods.

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Prevalence

A characteristic of RLS, affecting about 5-15% of the general population.

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Age of Onset

RLS can affect people of any age, but it's more common in middle-aged and older adults.

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Gender Impact

RLS affects women more often than men.

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Aetiology

The unknown cause of RLS, likely involving the dopaminergic pathways in the brain.

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Study Notes

UKMLA and PLAB Exam: Neurology Notes

  • Syringomyelia is a chronic condition where a fluid-filled cavity (syrinx) forms in the spinal cord.
  • It can lead to progressive damage, particularly affecting the central and anterior spinal cord tracts.
  • Often associated with Chiari malformations or spinal cord injuries.
  • If the syrinx extends to the brainstem (medulla), it's called syringobulbia.
  • Syringomyelia is strongly linked to Arnold-Chiari malformation (Type I), causing downward displacement of the cerebellar tonsils, obstructing cerebrospinal fluid (CSF) flow.

Pathophysiology

  • Syrinx Formation: The syrinx typically develops in the cervical or thoracic spine and expands, compressing surrounding spinal cord tissue.
  • Affected Pathways: Disrupts pain and temperature sensation (spinothalamic tract) and motor function (anterior horn cells), potentially leading to muscle weakness and wasting.

Clinical Features

  • Dissociated Sensory Loss: Primarily loss of pain and temperature sensation in a "cape-like" distribution over the shoulders, arms, and upper chest.
  • Loss of Reflexes: Diminished or absent reflexes in upper limbs due to anterior horn cell damage.
  • Bilateral Upgoing Plantar Reflexes: Indicates involvement of corticospinal tracts in advanced cases.
  • Light Touch, Vibration, and Position Sense: These senses are usually preserved initially but can be affected as the syrinx enlarges, impacting dorsal columns.

Motor Deficits

  • Weakness and wasting of small hand muscles due to anterior horn cell involvement.
  • Lower motor neuron (LMN) pattern of weakness.
  • As the condition progresses, upper motor neuron (UMN) signs may appear in lower limbs, like hyperreflexia and spasticity, if the syrinx involves corticospinal tracts.

Other Symptoms

  • Horner's Syndrome: Ptosis (drooping eyelid), miosis (constricted pupil), and anhidrosis (lack of sweating) on the affected side, caused by sympathetic pathway disruption.
  • Pain: Can be severe and exacerbated by coughing or straining.

Investigation

  • MRI of the Spine: Gold standard for diagnosis, visualising the syrinx, and assessing associated abnormalities (e.g., Chiari malformation).
  • CT Scan: Useful for evaluating bony abnormalities, though less sensitive than MRI for detecting the syrinx.
  • Dynamic MRI: Used to assess cerebrospinal fluid (CSF) flow, particularly if associated conditions like Chiari malformations are suspected.

Management

  • Surgical Intervention: Indicated in symptomatic patients or if the syrinx is enlarging. Goals include restoring normal CSF flow and decompressing the syrinx (e.g., posterior fossa decompression in Chiari malformations or direct syrinx drainage).
  • Monitoring: Asymptomatic patients or those with mild symptoms may be monitored periodically with MRI to track syrinx size and progression.

Differential Diagnosis

  • Multiple Sclerosis (MS): Episodes of neurological deficits frequently involving optic neuritis, characterized by wide CNS locations with demyelinating plaques.
  • Amyotrophic Lateral Sclerosis (ALS): Presents with progressive upper and lower motor neuron signs, but typically doesn't affect sensory modalities.
  • Chiari Malformation: Often associated with syringomyelia, particularly type I. Presents with occipital headaches, neck pain, and cerebellar dysfunction. Other conditions, such as spinal cord tumors or various inflammatory conditions, should also be considered in the diagnostic process.
  • Spinal Cord Tumor: May mimic syringomyelia presenting with similar motor and sensory deficits but often with back pain and more rapid progression.

Transient Ischemic Attack (TIA)

  • A temporary period of neurological dysfunction caused by focal brain, spinal cord, or retinal ischemia without infarction.
  • Symptoms usually resolve within 24 hours (often less than 1 hour).
  • Frequently a warning sign for future strokes, with significant risk within the first 48 hours.

Risk Factors

  • Cardiovascular disease
  • Hypertension
  • Diabetes
  • Hyperlipidemia
  • Smoking
  • Atrial Fibrillation
  • History of ischemic heart disease

Pathophysiology of TIA

  • Transient occlusion of cerebral or retinal blood vessels, frequently due to thromboembolism.
  • Common sources include atherosclerotic plaques in carotid arteries or cardiac emboli (especially atrial fibrillation).

Clinical Presentation of TIA

  • Sudden onset of focal neurological deficits (e.g., weakness, numbness, vision loss).

Diagnosis of TIA

  • Clinical assessment (history and neurological examination).
  • Imaging (e.g., CT Head, MRI, Carotid Doppler Ultrasound, CT/MR Angiography, Echocardiogram).

Differential Diagnosis of TIA

  • Migraine with Aura: Migraine aura progresses over minutes while TIA symptoms are abrupt.
  • Stroke: TIA symptoms resolve within 24 hours while stroke symptoms persist.

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UKMLA and PLAB Exam: Neurology Notes Updated PDF

Description

Test your knowledge on key neurological conditions including ALS, Chiari malformation, and cauda equina syndrome (CES). This quiz covers critical distinguishing symptoms, imaging findings, and clinical considerations for diagnosis and management. Perfect for medical students and professionals in neurology.

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