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Neurology Flashcards

1
Q

Cerebellar signs

A

Dysdiadochokinesia
Ataxia
Nystagmus
Intention tremor
Slurred speech
Hypotonia

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2
Q

Differentials for ataxia

A

Acute - post-infection cerebellitis, tumour, drugs (phenytoin), stroke, head injury
Intermittent - migraine
Chronic - Friedrich’s ataxia, ataxia telangiectasia, Wilson’s disease, ataxic cerebral palsy, multiple sclerosis

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3
Q

Viral causes of cerebellitis

A

VICE
Varicella
Influenza
Coxsackie
Enterovirus

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4
Q

Definition of hydrocephalus

A

Increased volume of CSF, usually with increased pressure and with some degree of dilatation of the ventricles

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5
Q

3 causes of hydrocephalus

A

Obstructive
Decreased absorption of CSF
Overproduction of CSF (only really due to papilloma of the choroid plexus - incredibly rare)

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6
Q

What is communicating hydrocephalus

A

CSF can flow from 4th ventricular foramina to subarachnoid space but is not absorbed by arachnoid granulations

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7
Q

What is non-communicating hydrocephalus

A

There is no communication between the ventricles and sub-arachnoid space

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8
Q

Causes of non-obstructive hydrocephalus

A

Infection: encephalitis, meningitis, intrauterine toxoplasmosis
IVH or intracranial bleed
Sagittal sinus thrombosis
(Arachnoid villi hypoplasia)

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9
Q

Causes of obstructive hydrocephalus

A

Tumour
Dandy-walker cyst
Arnold chiari malformation
Aqueduct stenosis
Vein of Galen malformation
Achondroplasia

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10
Q

What is a Dandy walker cyst

A

absence or deficiency of cerebella vermis and fourth ventricular foramina outlets leads to cystic dilatation of 4th ventricle

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11
Q

What is the Arnold-Chiari malformation

A

Small posterior fossa, downward displacement of cerebellar tonsils and medulla into foramen magnum - often associated with spina bifida

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12
Q

Aqueduct stenosis
Genetics?
Clinical features?

A

X-linked or sporadic.
X-linked - short 1st metacarpal + thumb flexed in opposition
(child will have hydrocephalus/macrocephaly and overlapping fingers)

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13
Q

How does achondroplasia cause hydrocephalus

A

bony abnormalities causes obstruction of CSF flow

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14
Q

Normal ratio of white cells to red cells in peripheral blood

A

1:500

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15
Q

CSF glucose

A

Usually less than 2/3 blood glucose

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16
Q

Most common organisms implicated in meningitis neonatal period

A

GBS
Listeria
E. coli

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17
Q

Neurological sequelae in meningitis.

Pneumococcal?
Haemophilus?

A

Neurological sequelae most common in pnuemococcal
Effect of dexamethasone greatest in Haemophilus

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18
Q

Hypsarrythmia on EEG may be seen in:

A

prenatal: Tuberous sclerosis, infantile spasms
perinatal: Birth asphyxia
post-natal: Head injury, PKU,

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19
Q

How is diagnosis of SSPE confirmed

A

High anti-measles Ab titire in CSF

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20
Q

Herpes simplex encephalitis on EEG

A

Periodic complexes in leads over the temporal lobes

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21
Q

Classification of IVH

A

Grade I: Bleed in germinal matrix
Grade II: Bleed extending into the lateral ventricle, no ventricular dilatation
Grade III: Bleed extending into ventricles (>50%) with ventricular dilatation
Grade IV: Parenchymal involvement

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22
Q

Brudzinski’s sign

A

+ve = hip and knee flexion when neck is flexed (due to neck stiffness)

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22
Q

Kernig’s sign

A

Patient lying flat with knee flexed at 90 degrees, pain on extension of knee is positive Kernig sign

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23
Q

Neurological causes of the floppy infant

A

Congenital myotonic dystrophy
Congenital myasthaenia
SMA
Congenital myopathy

learn differences between these

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24
Poor prognostic indicators in spina bifida
Hydrocephalus Incontinent of faeces or urine Paralysis of legs Lumbar kyphosis
25
Side effects of phenytoin
Megaloblastic anaemia Rickets (secondary to vitamin D metabolism) Gum hypertrophy
26
Neurological complications of B12 deficiency
Subacute combined degeneration of the cord **peripheral neuropathy** Optic neuritis Dementia
27
Drugs that cause peripheral neuropathy
metronidazole isoniazid Nitrofurantoin Vincristine/vinblastine (chemo) amiodarone Cisplatin (chemo) Phenytoin (seizures) AZT (HIV)
28
Facial features in congenital myotonic dystrophy
Thin face Inverted V shape upper lip high arched palate Thinning hair in temporal regions ptosis
29
Features of myotonic dystrophy
Eyes: cataracts Learning impairment testicular atrophy IDDM cardiomyopathy (most die from arrhythmias) Myotonic handshake (slow to release hand grasp)
30
Juvenile myasthenia gravis
Fatigueability Ophthalmoplegia Bulbar symptoms
31
Rx of myasthenia gravis
Pyridostigmine (acetylcholinesterase) steroids plasmaphoresis thymectomy (thymomas are feature)
32
Pattern of weakness: Myopathy Neuropathy
Myopathy = proximal weakness Neuropathy = distal weakness
33
Features of Duchenne muscular dystrophy on examination
prominent calves (pseudohypertrophy) Gower sign Trendelenburg (waddling) gait kyphosis, scoliosis or lumbar lordosis tip-toe walking Bilateral pes cavus (high arch in foot)
34
Genetics of BMD + DMD
X-linked
35
Miller-Fisher syndrome
Areflexia, ataxia, ophthalmoplegia
36
Key features of Landau-Kleffner
normal language development until acute regression in comprehension and verbal auditory agnosia. Able to interact in socially normal manner Up to 80% go on to have seizures (EEG may show continuous spike wave during slow wave sleep)
37
Friedrich's ataxia Inheritance Onset Typical features (*neuro signs*) Deformity Other systems
Autosomal recessive 5-15 years Spasticity + upgoing plantars (corticospinal tracts) Broad based gait (ataxia) Absent ankle reflexes Pes cavus, scoliosis Cardiomyopathy, optic atrophy
38
3 subtypes of cerebral palsy
Hemiplegia Quadriplegia Diplegia
39
Diagnosis of NF-1
≥6 cafe au lait patches (>5mm pre-puberty, >15mm post-puberty) Axillary freckling ≥2 neurofibromas OR 1 plexiform neurofibroma (more diffuse, on face and neck) Optic glioma ≥2 Lisch nodules Fibrous dysplasia of bone ≥1 relative with neurofibromatosis | *2 or more of above*
40
Incidence of NF-1
1 in 2,500
41
On examinaiton in NF
Short stature Kyphoscoliosis Macrocephaly
42
Follow up in NF-1
1. Annual full physical examination - 2. BP monitoring (because of renal artery stenosis, phaeochromocytoma) 2. Annual vision exam 3. Tumour surveillance ## Footnote (risk of Wilms, medulloblastoma, neuroblastoma, astrocytoma, ependymoma, meningioma, phaeochromocytoma, medullary Ca thyroid)
43
NF-1 cf. NF-2
1st clinical manifestation: cataracts (not cafe au lait patch) no learning impairment (NF-1 has learning impairment) schwannoma most common neoplasm Features hearing loss, tinnitus
44
NF-2 diagnosis
1. Bilateral acoustic neuromas 2. Unilateral acoustic neuroma + 1st degree relative with NF-2 3. Unilateral acoustic neuroma OR 1st degree relative with NF-2 + 1 of: *meningioma, ependymoma, non-vestibular schwannoma, cataract*
45
DDx for cafe au lait
NF-1 and NF-2 Tuberous sclerosis Turner syndrome McCune-Albright syndrome Russell-Silver syndrome
46
Gottron's patches | Juvenile dermatomyositis
47
Heliotrope rash | Juvenile dermatomyositis
48
Subungal fibroma | Tuberous sclerosis
49
Adenoma sebaceum | Tuberous sclerosis
50
Ash leaf patch | Tuberous sclerosis
51
Examination findings in Sturge-Weber
Port wine stain *(naevus flammeus)* in the distribution of the sensory divisions of the trigeminal nerve
52
Incidence of Sturge-Weber
1 in 20,000 to 1 in 50,000
53
Features of Sturge Weber
Vascular neurocutaneous syndrome which leads to headaches, seizures, hemiparesis, glaucoma (due to malformations in vessels of the eye), intellectual disability Seizures onset from 1 month to 1 year. Often focal clonic, contralateral to PWS
54
Features of UMN facial nerve palsy Causes of UMN facial nerve palsy
Contralateral weakness below eyebrows, brow sparing Cerebral palsy, bleed, tumour
55
Features of LMN facial nerve palsy Causes of LMN facial nerve palsy
Ipsilateral weakness with no sparing of the brow **(no ptosis)** Causes: Idiopathic, infection (Ramsay-Hunt = Herpes Zoster) mastoiditis or mastoid surgery, GBS
56
Incidence of Charcot-Marie-Tooth disease (Peroneal muscular atrophy, HMSN Type IIa) Genetics
3.8 in 100,000 Autosomal dominant (and AR but rarer)
57
CMT onset
late childhoo/early adolescence but gait abnormality may be present from 1-2 years
58
Features of CMT on examination
Anterior compartment of LLs become wasted Progressive weakness of ankle dorsiflexion and eventually foot drop Drop foot gait **- look for orthotics!!** Pes cavus deformity Defects in vibration, proprioception, pain + tempearature + motor and autonomic
59
Tests in CMT Diagnosis
Test = EMG Sural nerve biopsy diagnostic
60
Tell me about Bell's palsy
Common in all age groups Usually occurs around 2 wks after viral infection Activation or reactivation of HSV, varicella-zoster
61
(Rarer) DDx for unilateral (or bilateral) facial nerve palsy
Infection: Lyme disease, EBV, CMV Malignant: leukaemic infiltration, schwannoma, neurofibromas Other: hypertension, T1DM, trauma
62
Prognosis of Bell's palsy
90% make good recovery over weeks <10% have residual weakness
63

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