1
Q
What is a tremor?
A
Rhythmical, oscillatory movement.
2
Q
What is dystonia?
A
Sustained contraction of agonist and antagonist muscles → abnormal posture, slow movements, sometimes tremor.
3
Q
What is chorea?
A
Irregular, purposeless, brief jerky movements
4
Q
What is athetosis?
A
Slower, writhing form of chorea
5
Q
What is ballismus?
A
Violent, flinging movements, usually of proximal limbs; extreme chorea.
6
Q
What is myoclonus?
A
Sudden, unpredictable, shock-like jerk
7
Q
What is asterixis?
A
Sudden loss of tone; “negative myoclonus.”
8
Q
What are tics?
A
Jerks or semi-purposeful, stereotyped movements/facial grimaces, often preceded by a feeling of compulsion.
9
Q
What is akathisia (acathisia)?
A
Complex stereotyped movements associated with inner restlessness; usually tardive.
10
Q
What is dyskinesia?
A
Abnormal involuntary movement; often drug-induced.
11
Q
What is a stereotypy?
A
Repetitive motor movements (e.g., rocking, hand wringing, tongue protrusion).
12
Q
What does tardive mean?
A
Movement disorder arising after prior exposure to neuroleptic medication.
13
Q
What is exaggerated physiological tremor?
A
Mild, fine postural tremor that may worsen with age, stress, hyperthyroidism, or β-agonist drugs. Usually no other neurological signs.
14
Q
What is essential tremor (ET)?
A
• Postural 4–12 Hz tremor of hands and forearms, also appearing on finger-nose testing or writing.
• Often symmetrical.
• Can involve head (yes–yes), jaw, tongue, voice.
• Usually familial (≈50%), may improve with alcohol.
• Rest tremor is uncommon; if present, review diagnosis.
• Diagnosis: clinical features + exclusion of other causes (no tremorogenic drugs, normal tone, coordination, and thyroid/ Wilson’s labs).
15
Q
What is essential tremor (ET)?
A
Postural 4–12 Hz tremor of hands/forearms, may involve head, jaw, tongue, voice; often familial; improves with alcohol.
16
Q
Which features help distinguish ET from Parkinson’s tremor?
A
ET is mostly postural, symmetrical, with normal tone, coordination, and no bradykinesia.
17
Q
First-line drug for ET?
A
Propranolol (if no asthma) or primidone.
18
Q
Non-drug treatments for severe ET?
A
Botulinum toxin (limited) or deep brain stimulation (VIM thalamus).
19
Q
How does Parkinsonian tremor differ from essential tremor?
A
Usually asymmetrical, rest tremor > action tremor, legs can be involved, postural tremor appears later (re-emergent).
20
Q
What is a re-emergent tremor?
A
Postural tremor in Parkinson’s that appears after a delay.
21
Q
What is an “indeterminate tremor”?
A
Tremor difficult to classify as ET or Parkinson’s; diagnosis may become clear over time.
22
Q
Features of dystonic tremor?
A
Often in neck, arm, hand, or face; non-rhythmic, directional, associated dystonia, may have sensory trick, action/position-dependent.
23
Q
Which tremor is usually isolated to the head?
A
Dystonic tremor.
24
Q
Treatments for dystonic tremor?
A
Trihexyphenidyl or procyclidine (limited effect, side effects common); botulinum toxin injections, especially for head tremor.
25
Key feature of drug-induced tremor?
Often appears after long-term use of a medication; review all drugs, withdrawal may take time for improvement.
26
What characterizes cerebellar tremor?
Predominantly on posture and movement; associated with clumsiness or unsteadiness rather than tremor alone.
27
What is the hallmark of cerebellar tremor?
Intention tremor—worse as the movement approaches the target.
28
Associated features of cerebellar tremor?
Incoordination, dysarthria, gait ataxia.
29
Causes of cerebellar tremor?
Structural, degenerative, genetic, or inflammatory cerebellar pathology.
30
Drug treatment for cerebellar tremor?
None effective; limb weights may provide symptomatic relief.
31
Key features?
Fragile X Tremor–Ataxia Syndrome
Late-onset intention tremor and ataxia; white matter changes in middle cerebellar peduncles; FMR1 gene.
32
Characteristics? lmes (Rubral) Tremor
Slow, irregular rest and intention tremor, postural; delayed onset after brainstem/thalamic lesion.
33
Treatment response?
Holmes (Rubral) Tremor
Limited improvement with levodopa or dopamine agonists
34
What is it?
Primary Writing Tremor
Task-specific tremor during writing; may be variant of ET or dystonia
35
When can neuropathies cause tremor?
Tremor and Neuropathies
Demyelinating peripheral neuropathies, especially with anti-MAG antibodies.
36
Typical presentation? Orthostatic Tremor
High frequency (14–16 Hz) leg tremor on standing, unsteadiness relieved by walking.
37
Diagnosis clue?
Orthostatic Tremor
EMG of quadriceps shows high-frequency tremor; may sound like helicopter on auscultation.
38
Treatment options?
Orthostatic Tremor
Gabapentin, clonazepam, primidone, levodopa (limited effect).
39
Palatal Tremor
Key feature?
Clicking in ears due to rhythmical tremor of the palate.
40
Palatal Tremor
Types?
Essential palatal tremor (no MRI abnormality), symptomatic (dentate–olivary lesions).
41
Palatal Tremor
Treatment?
Botulinum toxin into palatal muscles.
42
Hallmarks
Functional Tremor
Abrupt onset, variable, inconsistent pattern, often associated with other functional disorders.
43
Mechanism of dystonia?
Co-contraction of agonist and antagonist muscles causing abnormal posture and jerky movements.
44
Sensory trick in dystonia?
Geste antagoniste—using touch to control abnormal posture.
45
Classification by age of onset?
Early onset (<25 yrs, often limb, can generalise; DYT1 gene)
Late onset (>25 yrs, usually cervical or blepharospasm, often remains focal/segmental).
46
Anatomical distribution of dystonia?
Focal, segmental, generalised, hemidystonia
47
Causes of dystonia?
Primary/idiopathic or secondary (drug-induced, neurodegenerative, structural).
48
In cervical dystonia, what are the common names based on abnormal neck position?
Torticollis (turn), laterocollis (lean), anterocollis (forward), retrocollis (backward).
49
What are the key clinical features of cervical dystonia?
Neck pain, abnormal posture, possible tremor, worse with walking/driving, hypertrophy of sternocleidomastoid/splenius capitis.
50
How is cervical dystonia treated?
Anticholinergics (occasionally helpful), regular botulinum toxin injections (most effective).
51
What is the clinical presentation of blepharospasm?
Involuntary blinking and forced closure of both eyes, non-painful.
52
What is an important differential diagnosis in blepharospasm?
Distinguish from ocular causes like corneal inflammation.
53
How is blepharospasm treated?
Botulinum toxin injections.
54
What are the clinical features of spasmodic dysphonia?
Strangulated, variable speech.
55
How is spasmodic dysphonia treated?
Endoscopic botulinum toxin injections into overactive laryngeal muscles.
56
How common is limb-onset dystonia in adults and what should it prompt?
Rare; should prompt a search for secondary causes.
57
Give an example of a task-specific dystonia.
Writer’s cramp.
58
What is the key feature of task-specific dystonia?
Occurs only during a specific task
59
What is the typical presentation of dopa-responsive dystonia?
What is the typical presentation of dopa-responsive dystonia?
60
Which genetic mutation is most commonly associated with dopa-responsive dystonia?
Mutations in GTP-cyclohydrolase 1.
61
How is dopa-responsive dystonia treated?
Dramatic response to levodopa, starting at 50 mg/day (with decarboxylase inhibitor), titrated up to 1000 mg/day if needed.
62
Why is dopa-responsive dystonia important in clinical practice?
It is rare but treatable; any patient with dystonia should be considered for a levodopa trial.
63
What is the typical age of onset for myoclonus–dystonia?
Early adult life.
64
What are the key clinical features of myoclonus–dystonia?
Myoclonic movements (predominantly arms) with mild cervical dystonia or writer’s cramp.
65
Which gene is associated with myoclonus–dystonia?
DYT11
66
What unusual factor improves myoclonus–dystonia symptoms?
Alcohol
67
What is the typical onset of DYT1-associated dystonia in childhood?
Starts in the limbs and progresses to neck and face.
68
How does DYT1 dystonia usually present in adults?
As focal dystonia, most commonly cervical dystonia or blepharospasm.
69
What is the most common cause of secondary dystonia?
Parkinson’s disease and dopaminergic drugs used for treatment.
70
Which drug class is known to cause tardive or acute dystonia?
Dopamine antagonists (e.g., antipsychotics).
71
When dystonia occurs in parkinsonism-plus syndromes, what usually reveals the diagnosis?
Other features of the underlying disease (e.g., multiple system atrophy, corticobasal degeneration, progressive supranuclear palsy).
72
Are other causes of secondary dystonia common?
No, they are rare, and dystonia is usually only part of a broader clinical presentation.
73
What movement disorders can result from basal ganglia injury due to perinatal hypoxia or kernicterus?
Dystonia or choreoathetoid cerebral palsy.
74
What neurological presentations can occur in Wilson’s disease?
Tremor, dystonia, chorea, or akinetic-rigid syndrome, often with cognitive and personality changes.
75
What eye finding is almost always present in neurological Wilson’s disease?
Kayser–Fleischer rings
76
How is Wilson’s disease diagnosed?
Low plasma caeruloplasmin, increased 24 h urinary copper, Kayser–Fleischer rings; liver biopsy if uncertain
77
What is the conventional treatment for Wilson’s disease?
Chelation with penicillamine or trientine, or zinc to prevent absorption; newer agent tetrathiomolybdate may be effective.
78
What are the typical early and late presentations of PKAN?
Classical: rapid progression in first decade; Atypical: slower progression in second decade.
79
What movements are prominent in PKAN?
Chorea, dystonia, tics, facial dystonia, blepharospasm, tongue and mouth movements.
80
What is the characteristic MRI sign in PKAN?
“Eye of the tiger” sign (high intensity anterior globus pallidus with low intensity posteriorly).
81
Is there a specific treatment for PKAN?
No specific treatment available.
82
What is neuroferritinopathy and how does it present?
Rare late-onset disorder similar to Huntington’s disease, associated with low ferritin.
83
What structural lesions can cause dystonia?
Stroke, inflammation, infection, or neoplasm affecting basal ganglia.
84
What is the difference between chorea, athetosis, and ballismus?
Chorea: irregular dance-like movements; Athetosis: slower writhing movements; Ballismus: violent flinging of proximal limbs.
85
Which drugs commonly cause chorea?
Levodopa, dopamine agonists, dopamine antagonists; less commonly lithium, OCPs, corticosteroids.
86
How does Huntington’s disease present?
Progressive chorea, tics, dystonia, myoclonus; frontal behavioral changes, personality change, dementia.
87
How is Huntington’s disease diagnosed?
Genetic testing; caudate atrophy on MRI suggestive
88
How is chorea in Huntington’s managed?
Tetrabenazine or atypical antipsychotics (risperidone, olanzapine); multidisciplinary care and psychiatric support
89
What are the features of neuroacanthocytosis?
Chorea, dystonia, tics, cognitive issues, seizures, neuropathy; acanthocytes seen on blood film.
90
Is there a specific treatment for neuroacanthocytosis?
No
91
When does Sydenham’s chorea occur?
1–2 months after group A β-haemolytic streptococcus infection in children.
92
Can chorea occur in autoimmune or systemic conditions?
Yes—SLE, antiphospholipid syndrome, pregnancy (chorea gravidarum), HIV-related.
93
Which metabolic disorders can cause chorea?
Hyperthyroidism, polycythemia rubra vera, non-ketotic hyperglycemia, liver failure (acquired hepatolenticular degeneration).
94
How can structural brain lesions cause hemichorea or hemiballismus?
Lesions affecting subthalamic nucleus and its connections (stroke, tumor, demyelination).
95
How is symptomatic chorea or ballismus treated?
Often with dopamine antagonists, e.g., haloperidol.
96
What is myoclonus?
A very brief shock-like jerk of a muscle.
97
What are the two types of myoclonus based on muscle activity?
Positive myoclonus: sudden muscle contraction; Negative myoclonus (asterixis): sudden loss of muscle tone.
98
Which type of myoclonus is most commonly seen in metabolic encephalopathy?
Negative myoclonus (asterixis), especially in hepatic encephalopathy.
99
How can cortical myoclonus be identified?
EEG shows time-locked cortical discharges; somatosensory evoked potentials are enlarged.
100
How does spinal myoclonus appear on EEG and somatosensory evoked potentials?
Both are normal.
101
Which conditions are associated with myoclonus in epilepsy?
Juvenile myoclonic epilepsy and progressive myoclonic epilepsies (Unverricht–Lundborg, Lafora disease, neuronal ceroid lipofuscinosis, sialidoses).
102
Which drugs can worsen myoclonic seizures?
Carbamazepine, phenytoin, gabapentin, tiagabine
103
What is the first-line treatment for myoclonic epilepsies?
Sodium valproate.
104
Name other drugs used in myoclonic epilepsy
Lamotrigine, levetiracetam, zonisamide, clonazepam, clobazam.
105
What is Lance–Adams myoclonus?
Post-hypoxic myoclonus following significant hypoxia, often after cardiac arrest.
106
How is Lance–Adams myoclonus treated?
Sodium valproate, clonazepam, or levetiracetam.
107
What is opsoclonus–myoclonus syndrome?
An immune-mediated disorder with “dancing eyes” and myoclonus; can be paraneoplastic or post-infectious.
108
Which cancers are associated with paraneoplastic opsoclonus–myoclonus in children and adults?
Children: neuroblastoma; Adults: lung, breast, ovary.
109
Name other encephalopathies that can present with myoclonus.
Creutzfeld–Jakob disease, Hashimoto’s encephalopathy, subacute sclerosing panencephalitis, mitochondrial cytopathies.
110
Which drugs can cause myoclonus?
Most antipsychotics, lithium, most antidepressants, some antiepileptics, and others.
111
What is essential myoclonus?
Myoclonus occurring in isolation, with no other neurological features or identifiable cause.
112
What are the two patterns of spinal myoclonus?
1) Jerks in muscles of contiguous spinal levels, often due to intrinsic spinal cord disease, syringomyelia, or tumour.
2) Propriospinal myoclonus: jerks involving the trunk, especially abdominal muscles, usually in middle-aged men, often worse on lying down, with no identifiable cause.
113
Which spinal pathology can cause jerks in contiguous spinal levels?
Intrinsic spinal cord disease, syringomyelia, or spinal tumours.
114
What is the typical demographic and trigger for propriospinal myoclonus?
Middle-aged men; worsens on lying down
115
What are symptomatic treatments for spinal myoclonus?
Clonazepam, sodium valproate, piracetam, levetiracetam, ethosuximide, zonisamide.
116
What are tics?
Brief, sudden, shock-like movements preceded by a compulsion or urge to move.
117
What distinguishes simple tics from complex tics?
Simple tics: brief, often facial or vocal movements.
Complex tics: more elaborate movements involving face, limbs, or vocalisations.
118
Can patients voluntarily suppress tics?
Yes, but only for a brief period.
119
What is the typical course of tics in childhood?
Common and often resolve spontaneously.
120
What is Tourette’s syndrome?
Childhood onset of multiple motor and vocal tics, with ~50% having associated obsessive–compulsive behaviours.
121
What causes adult-onset tics?
Often drug-induced (neuroleptics), or associated with neurodegenerative, metabolic, post-anoxic, or multifocal brain pathologies like antiphospholipid syndrome
122
Do all patients with tics need drug treatment?
No, many are satisfied with an explanation of their tics.
123
Which α-agonists can be used for tics?
Clonidine (50–500 μg/day) and guanfacine.
124
Which dopamine antagonists are used for tics?
Risperidone, aripiprazole, haloperidol. Use the minimum effective dose.
125
What other agents may be used for tics?
Botulinum toxin, clonazepam, tetrabenazine.
126
What are motor stereotypies or habit spasms?
Repeated movements such as rocking, hand wringing, or clapping, often seen in learning difficulties, autism, or psychiatric disorders.
127
How are motor stereotypies different from tics?
They are usually straightforward to recognize, continuous, and not preceded by a premonitory urge.
128
What does the term “dyskinesia” usually refer to?
Drug-induced movements, though it can also describe most abnormal movements collectively.
129
What are common features of tardive dyskinesias?
Orofacial movements (chewing, tongue), akathisia (restlessness, pacing), variable onset relative to neuroleptic exposure.
130
Do tardive dyskinesias always improve when the causative drug is stopped?
No, they often persist despite stopping or changing the drug.
131
Which autoimmune encephalopathy is associated with acute dyskinesia?
Anti-NMDA receptor encephalitis
132
What are the typical features of anti-NMDA receptor encephalitis?
Seizures, decreased consciousness, severe orofacial dyskinesias, chorea, dystonia.
133
What proportion of patients with anti-NMDA receptor encephalitis have an underlying tumour?
Over 50%.
134
What other conditions can cause acute encephalopathy with dyskinesia?
Drug-induced movement disorders, Hashimoto’s encephalopathy, encephalitis lethargica, metabolic encephalopathies (renal, liver, thyroid, hyperglycaemia), Creutzfeldt–Jakob disease.
135
Which tumor is most commonly associated with anti-NMDA receptor encephalitis?
Ovarian teratoma, which should be treated.
136
What were the clinical features of encephalitis lethargica?
Sleep disorder, parkinsonism, dystonia, tics, oculogyric crises.
137
Is there a specific diagnostic test for encephalitis lethargica?
No
138
What is major hyperekplexia?
Dominantly inherited stiff-legged gait; falls “like a log” when startled; related to GLRA-1 mutations.
139
How does minor hyperekplexia differ?
Increased startle without other signs; usually no clear genetic abnormality.
140
How is hyperekplexia treated?
Clonazepam; genetic testing available
141
What else can cause excessive startle?
Rarely brainstem disease (MS, stroke, etc.).
142
What triggers paroxysmal kinesigenic dyskinesia?
Sudden movement; onset usually in childhood; responds well to carbamazepine.
143
What triggers paroxysmal non-kinesigenic dyskinesia?
Fatigue, coffee, or alcohol; may improve with clonazepam
144
Which rare paroxysmal dyskinesia is associated with a potassium channel defect?
Generalized epilepsy with paroxysmal dyskinesia (KCNMA1).
145
How are functional movement disorders recognized?
Variable, triggered/suppressed by unusual tricks; history often includes minor injuries; investigations often normal.
146
Why avoid the term “psychogenic” in these disorders?
It presupposes a psychological cause and may hinder management.
147
How is restless legs syndrome diagnosed?
Based on history; no abnormal movements on exam.
148
What are the key features?
Urge to move legs, burning/tingling sensations, periodic limb movements in sleep.
149
What are common associations?
Neuropathy, renal failure, pregnancy, low ferritin, family history.
150
How is restless legs syndrome treated?
Iron replacement if low ferritin; dopamine agonists (pramipexole, ropinirole); gabapentin, pregabalin, clonazepam, zolpidem.
151
What is this syndrome associated with
Painful Feet and Moving Toes
Neuropathy or radiculopathy; movements often noticed by the examiner rather than patient.
152
What is hemifacial spasm?
Rhythmic contraction of facial muscles, usually around one eye.
153
What usually causes hemifacial spasm?
Usually microvascular compression at the facial nerve root entry zone; rarely brainstem demyelination.
154
How is hemifacial spasm treated?
Botulinum toxin injections; severe or resistant cases may need microvascular decompression.
Neurology
flashcards
Decks in class (38)
# Cards
-
Headache113
-
Headache cases13
-
Weakness59
-
Epilepsy131
-
The Rule of 4 and Brainstem Localisation36
-
Epilepsy133
-
Into To Neuro51
-
Raised ICP102
-
Epilepsy CPC31
-
Stroke178
-
Movement Disorders93
-
Stroke39
-
Traunatic Brain Injury90
-
Glascoma Scale13
-
CNS Tumors115
-
Neuroradiology156
-
Neurocascular43
-
Neurosurgery Red Flags93
-
Surgical Stroke38
-
The Spine29
-
Spinal Pathology103
-
Spinal Cord Compression30
-
Spinal Lesion88
-
Spina Bifida83
-
Neuro Exam33
-
Spinal Quiz26
-
Stroke Localisation45
-
Chronic Dizziness62
-
Coma63
-
Denentia125
-
Disorders Of The Spinal Cord132
-
Hyperkinetic Movements154
-
Hypoxic Ischemic Brain Injury20
-
Infections Of The Nervous System77
-
Motor Neuron Disease38
-
Muscle Disease33
-
Peripheral Berve Disease37
-
Spinal Cord Lesions44
