Neurology Quiz: Ataxia Disorders

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Questions and Answers

At what age does Friedreich's ataxia typically onset?

3rd decade

4th decade

2nd decade

1st decade (correct)

Which of the following clinical manifestations is characteristic of Marie's ataxia?

Severe atrophy of limbs

Spastic paralysis

Tremors and seizures

Nystagmus and dysarthria (correct)

What type of ataxia can be observed in Friedreich's ataxia?

Cerebral ataxia

Vestibular ataxia

Peripheral ataxia

Sensory ataxia (correct)

What is a common clinical feature of vestibular ataxia?

Vertigo Signup and view all the answers

Which cerebellar syndrome is mainly associated with swaying during standing?

Vestibulocerebellum syndrome Signup and view all the answers

What selective symptom is observed in neocerebellum syndrome in acute cases?

Severe hypotonia Signup and view all the answers

Which type of lesion can lead to sensory ataxia?

Brainstem lesions Signup and view all the answers

In Marie's ataxia, which of the following sensations is preserved?

Superficial sensations Signup and view all the answers

What does swaying during Romberg’s Test indicate?

Presence of sensory ataxia Signup and view all the answers

Which type of gait is associated with an archi-cerebellar lesion?

Wide base or drunken gait Signup and view all the answers

What is the primary goal of the restorative physical treatment mentioned?

To achieve functional gait and independence Signup and view all the answers

In the context of improving proprioception, which technique aims to enhance proximal stability?

Rhythmic stabilization Signup and view all the answers

When training for balance, what is the recommended sequence of tasks?

Simple first, then complex Signup and view all the answers

Which of the following is NOT a goal of treatment in improving functional gait?

Increased dependence on aids Signup and view all the answers

What characteristic defines neo-cerebellar lesions in terms of gait?

Deviation to one side or zigzag gait Signup and view all the answers

Which of the following activities would best improve proprioception according to the treatment methods?

Performing gait exercises on various surfaces Signup and view all the answers

What indicates moderate impairment in the grading system?

Able to accomplish activity but movements are slow and unsteady Signup and view all the answers

Which test assesses the ability to bring the tip of the forefinger to the nose?

Finger-to-nose test Signup and view all the answers

What does the heel-to-knee test assess?

Lower limb coordination Signup and view all the answers

What is the primary responsibility of the vestibulocerebellum?

Maintaining balance and postural control Signup and view all the answers

What phenomenon occurs when the resistance is suddenly released during the rebound test?

The forearm flies upwards Signup and view all the answers

Which of the following describes adiadokokinesis?

Inability to perform rapid alternating movements Signup and view all the answers

How does the cerebellum utilize sensory input for movement control?

It compares desired and actual outputs continuously Signup and view all the answers

What is the primary focus of the finger-to-finger test?

Assessing accuracy and speed in a movement Signup and view all the answers

What characterizes a feedforward control system in the cerebellum?

Inability to alter commands once sent Signup and view all the answers

What happens in the cerebellum during a dysmetric movement?

Cerebellum fails to correct mismatched movements Signup and view all the answers

During which activity might a patient exhibit deviation toward the affected side?

Walking along a straight line Signup and view all the answers

Which of the following tests evaluates proprioception by requiring a return to a predetermined position?

Heel-to-knee test Signup and view all the answers

What is the role of the spinocerebellum?

Aids in error detection and modulation of muscle tone Signup and view all the answers

Which function is performed by the cerebellum as an adaptive feed control system?

Preparation based on past sensory input and motor output Signup and view all the answers

Which cerebellar lobe is primarily associated with coordination and fine movement?

Cerebrocerebellum Signup and view all the answers

What is a characteristic feature of feedback control systems?

Adjustment based on comparing actual and desired outputs Signup and view all the answers

Which pattern of PNF involves movements that are symmetrical on both sides of the body?

Bilateral symmetrical Signup and view all the answers

What is the primary focus of the 'perception' component in coordination training?

To analyze performance feedback through sensory pathways Signup and view all the answers

Which vestibular exercise is designed to promote adaptation and substitution for vestibular dysfunction?

Cawthorne Cooksey exercises Signup and view all the answers

Which method is primarily aimed at improving coordination by using sensory mechanisms that remain intact?

Frenkel's exercises Signup and view all the answers

In vestibular habituation training, what is the recommended action after each repetition of an exercise that elicits vertigo?

Stay in the position until vertigo persists Signup and view all the answers

Which method is NOT typically used to decrease limb tremors?

Cawthorne Cooksey exercises Signup and view all the answers

Which of the following is NOT one of the graduations in Frenkel's exercises?

Standing then sitting Signup and view all the answers

What component of the 5Ps in coordination training involves practicing movements below peak performance to avoid fatigue?

Peak performance Signup and view all the answers

What is the first step of exercises performed from a supine position?

Flex & extend one leg, heel sliding down Signup and view all the answers

The goal of vestibular exercises is NOT to enhance which of the following?

Muscle strength Signup and view all the answers

What type of contraction is involved in 'Slow reversal' technique of PNF?

Isotonic contraction of the antagonist followed by isotonic contraction of the agonist Signup and view all the answers

What is the main purpose of the 'progression' component in coordination training?

To ensure exercises are reviewed and adapted as performance improves Signup and view all the answers

Which exercise is meant for improving balance in a sitting position?

Sliding heel to reach a mark on the floor Signup and view all the answers

What characterizes the 'Slow reversal hold' technique in PNF?

Isotonic contraction of the antagonist followed by isometric contraction of the antagonist Signup and view all the answers

How can balance be improved effectively in an antigravity position?

Employing a normal base of support Signup and view all the answers

Which of the following is NOT a recommended activity for coordination improvement?

Picking up randomly scattered objects Signup and view all the answers

Study Notes

Ataxia

Ataxia is the loss of coordination of voluntary movements

It may or may not be accompanied by disequilibrium, but does not include motor weakness

Cerebellum

The cerebellum is a part of the brain responsible for coordinating movement

It has three main lobes:

vestibulocerebellum (archicerebellum)

flocculonodular lobe

spinocerebellum (paleocerebellum)

anterior lobe

cerebrocerebellum (neocerebellum)

posterior lobe

The cerebellum maintains balance and postural control

It assists in error detection and modulating muscle tone

It plays a vital role in coordination, fine movement, action preparation, and timing.

It receives input from cortical, sensory, and proprioceptive areas, then projects this information to the thalamus

Damage to the cerebellum can cause significant motor impairment

The cerebellum receives sensory input and guides movement in a feedback and feed-forward manner

Feedback control systems continuously compare desired output to actual output to make adjustments during the movement

Feedback & Feed-forward Control

Feedback controllers continuously adjust the actual output to match the desired output

Feed-forward controllers send precise commands without feedback, allowing quicker movement execution

Coordination and Comparator

The cerebellum acts as a comparator checking voluntary signals against sensory signals from movement

Corrective feedback is provided to motor pathways if a mismatch is found

A cerebellar lesion may prevent the cerebellum from correcting movement errors leading to dysmetric movements

Compensator and Adaptive Control

The cerebellum acts as a compensator by adjusting reflexes to anticipate future movement

This is part of an adaptive feed-forward control system using prior sensory and motor output for prediction

Cerebellum Functions

Regulation of equilibrium

Maintaining muscle tone, affecting axial & proximal limbs. A lesion can lead to hypotonia.

Regulation (coordination) of voluntary movements- uses mechanisms including:

Comparator and error correction

Damping mechanism

Planning movement sequence & timing

Damping mechanism- the cerebellum sends braking signals to prevent overshooting of movements

A damaged cerebellum may result in intention kinetic tremor

Ataxia Types

Cerebellar ataxia

Sensory ataxia

Vestibular ataxia

Mixed ataxia

Hysterical ataxia

Cerebellar Ataxia Causes

Lesions to the cerebellum and/or its connections

Vestibulo-cerebellar dysfunction

Spino-cerebellar dysfunction

Cerebro-cerebellar dysfunction

Symptomatic (vascular, inflammatory, traumatic, or tumor)

Heridofamilial (Friedreich's or Marie's ataxia)

Ataxia Symptoms (Friedreich's and Marie's)

Age of onset: Friedreich's is in the 1st decade. Marie's is in the 2nd and 3rd decades

Cerebellar manifestations/deficits:

Friedreich's: mainly archicerebellar (walking & balance issues); spinocerebellar & pyramidal tract affected. Minimal or no deficits in deep reflexes & no impaired sensation.

Marie's: mainly neocerebellar (incoordination); pyramidal tract affected. Deep reflexes tend to be exaggerated, but sensation is preserved.

Sensory Ataxia

Can be observed in hereditary ataxias like Friedreich's

Can occur in other conditions like peripheral neuropathy, posterior column issues, brainstem lesions, thalamic syndrome, or parietal lobe lesions

Vestibular Ataxia

Ataxia due to lesions in the vestibular division of the 8th cranial nerve (e.g., Meniere's disease, labyrinthitis, acoustic neuroma)

Symptoms commonly include vertigo, tinnitus, and deafness.

Cerebellar Syndromes

Archicerebellum (vestibulocerebellum syndrome)- symptoms include problems with balance & posture (swaying, falling) and wide-based gait

Paleocerebellum-spinocerebellum syndrome- causes hypotonia & hyporeflexia impacting posture and coordination

Neocerebellum (cerebrocerebellum) syndrome- also causes hypotonia & hyporeflexia, affecting distal and proximal issues

Ataxia Symptoms- Other

Asthenia (generalized weakness) is a less common symptom, most prominent in patients with extensive & deep lesions

Incoordination:

Dysmetria: inability to judge range/direction of movement

Intention tremor (kinetic tremor): oscillation of movement that increases toward the end of the movement; seen in ongoing movements, not at rest.

Titubation: tremor affecting the head, upper trunk, and legs, follows lesion of the vermis

Decomposition of complex movements: distinct sequences rather than smooth movements

Rebound phenomenon: patient cannot quickly stop ongoing movement, limb forcefully moves or hits body

Dysdiadochokinesis: slow, irregular, and clumsy movements of rapidly alternating movements (such as pronation/supination)

Ataxia problems- Other

Problems with balance (postural sway, poor equilibrium)

Instability around pelvis which interferes with weight-bearing control

Orofacial dysfunction: nystagmus and dysarthria

Issues with visual fixation, head/trunk control, leading to problems with gait (wide-based or drunken gait)

Evaluation Principles

Bilateral assessments needed, even for unilateral lesions

Assess functional capabilities in a quiet environment, consideration of patient's age and emotional state

Examination of functional activity to include assistance needed and time to complete

Ataxia Assessment

Motor assessment (muscle tone, muscle tests)

Sensory assessment (superficial and deep sensation)

Range of motion (ROM)

Orofacial function (facial expression, lips, jaw closure, swallowing, speech) Coordination of movement assessed through:

Coordination (basic functional capabilities with equilibrium subtypes)

Alternate/reciprocal motion

Movement composition/synergy

Movement accuracy (e.g., dysmetria)

Fixation/limb holding

Equilibrium & postural holding

Special tests: Finger-to-nose, finger-to-finger, finger-to-doctor's finger, heel-to-knee, tandem gait, and Romberg's Test

Gait Assessment

Categorize gait according to lesion site (e.g., wide-based/drunken to unsteadiness/deviation to one side/zigzag):

Evaluate gait by speed, direction changes, balance loss frequency/distance walked & assistance needs

Goals of Treatment

Postural stability

Functional gait

Accuracy/coordination of limb movements

Treatment Principles

Graduated from simple to complex

Focus on proximal stability progressing to distal segments

Compensation aids and devices/assistive techniques when needed

Home exercise programs & recreation activities

Methods to improve proprioception

PNF techniques (rhythmic stabilization, reversal techniques, approximation)

Resistive exercises on varied surfaces (with eyes open/closed)

Using weighted objects, balance boards, and balls

Specific Techniques of PNF

Slow reversal (isotonic contraction of agonist and antagonist)

Slow reversal hold (isotonic contraction of antagonist followed by isometric contraction of antagonist then same agonist sequence)

Repetition of Assessment

Non-equilibrium tests, modification of range/speed/eye status during tests

Combined PNF patterns (bilateral symmetrical, bilateral asymmetrical, reciprocal same diagonal, reciprocal opposite diagonal).

Training of Coordination (5 phases)

Perception (correct performance) through sensory input (tactile & visual)

Precision (simplifying movements)

Perceptual practice (repetitive activity)

Peak performance- determine and practice movements below peak to prevent fatigue

Progression- revise performance as improvement occurs, transition to a functional goal

Vertigo Treatment Exercises

Vertigo is a sensation of body rotation, often paired with substitution and adapting vestibular function.

Vestibular habituation exercises

Cawthorne Cooksey exercises

Vestibular Habituation Training

Repeated stimulation of receptors causing vertigo

Determine position/movement eliciting vertigo

Select/practice movements which elicit vertigo

Repeat exercises five times twice daily

Maintain vertigo-eliciting positions until resolved

Cawthorne Cooksey Exercises

Graduated head and eye movements to train visual & somatosensory systems for vestibular adaptation

Exercises progress from least to most likely to elicit vertigo

Repeat 10-20 times per day, progressing from slow to faster movements

Methods for decreasing tremors/nodding/titubation

Tonic holding

Alternative isometric exercises

Weight-bearing exercises

Approximation

Weights on ankles/wrists

Rhythmic stabilization