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?

Stocking-glove distribution Signup and view all the answers

Which imaging modality is the first line for diagnosing syringomyelia?

MRI of the cervical spine Signup and view all the answers

What pattern of symptoms should prompt consideration of peripheral neuropathy?

Distal sensory loss without evidence of central involvement Signup and view all the answers

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

Permanent neurological deficits Signup and view all the answers

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

Identifying the cape-like distribution of sensory loss Signup and view all the answers

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

Saddle anesthesia Signup and view all the answers

What is the definitive investigation for diagnosing CES?

MRI Signup and view all the answers

Which symptom is most closely associated with Chiari malformation?

Neck pain Signup and view all the answers

Which of the following symptoms is NOT characteristic of CES?

Post-operative physiotherapy Signup and view all the answers

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

It improves mobility and strength Signup and view all the answers

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

Presence of saddle anesthesia and bowel/bladder dysfunction Signup and view all the answers

Which of the following management steps is crucial in CES?

Immediate surgical consultation Signup and view all the answers

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

Residual bladder, bowel, or sexual dysfunction Signup and view all the answers

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

Idiopathic NPH Signup and view all the answers

Which of the following conditions can lead to Secondary NPH?

Head trauma Signup and view all the answers

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

Magnetic gait Signup and view all the answers

What neuroimaging finding is characteristic of NPH?

Ventricular enlargement without cortical atrophy Signup and view all the answers

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

To assess response to CSF removal Signup and view all the answers

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

Individuals over 60 years Signup and view all the answers

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

Rapid cognitive deterioration Signup and view all the answers

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

Normal pressure and cell count Signup and view all the answers

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

Ventriculomegaly without significant cortical atrophy Signup and view all the answers

Which symptom is not typically associated with a subdural hematoma?

Urinary incontinence Signup and view all the answers

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

Ventriculoperitoneal (VP) shunting Signup and view all the answers

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

Gait disturbance, urinary incontinence, and cognitive decline Signup and view all the answers

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

Magnetic gait with minimal arm swing Signup and view all the answers

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

Regular monitoring for complications Signup and view all the answers

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

It presents similarly to Alzheimer’s disease Signup and view all the answers

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

Periodic sharp wave complexes Signup and view all the answers

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

An uncontrollable urge to move the legs Signup and view all the answers

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

Nighttime or periods of inactivity Signup and view all the answers

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

Middle-aged and older adults Signup and view all the answers

What type of disorder is Restless Leg Syndrome classified as?

Neurological disorder Signup and view all the answers

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

Dopaminergic pathways Signup and view all the answers

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

5-15% Signup and view all the answers

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

Women Signup and view all the answers

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

Symptoms worsen with rest Signup and view all the answers

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

Language difficulties Signup and view all the answers

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

Presence of motor symptoms like rigidity Signup and view all the answers

Which factor is a critical indicator of Vascular Dementia?

Multiple lacunar infarcts on imaging Signup and view all the answers

What unique symptom might suggest Lewy Body Dementia?

Visual hallucinations Signup and view all the answers

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

Medication side effects Signup and view all the answers

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

If behavioral changes and personality alterations are more prominent than memory loss Signup and view all the answers

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

Stepwise cognitive decline with focal neurological signs Signup and view all the answers

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

Cortical atrophy, especially in the temporal lobes Signup and view all the answers

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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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.