neuro Flashcards
(148 cards)
1
Q
parkinsons plus
A
PSP MSA Lew body dementia Vascular parkinsons Cortico basal degenration
PD vs Parkinson plus
PD - symmetrical
Parksinsons plus - swallowing difficulties, change in personlity, vertical gaze palsy (unable to look down)
2
Q
PSP
A
posturnal instbaility truncal rigidity frequent falls asymetrical onset speech and swallow palsy
3
Q
Multisystem atrophy
A
Atonomic features
Postural instability
bladder/bowel dysfunction
cerebellar/pyramidal signs
4
Q
Vascular parkinsons
A
Parkinson’s worse in legs than in arms
vascular risk factors
5
Q
CJD
A
rapid cognitive decline in a young person with myoclonus
EEG in sporadic CJD may show significant abnormalities involving deep brain areas such as the thalami. The normal rhythms are gradually lost. Initially the changes are diffuse, and non-specific, developing into generalised bi- or triphasic periodic sharp wave complexes with a frequency of 1-2 per second. High voltage sharp waves may be synchronous with myoclonic jerks. In an appropriate clinical context, this EEG pattern is strongly suggestive of a diagnosis of CJD.
6
Q
Brain stem death tests
A
Brain stem death tests include:
Pupillary light response - CN II and III
Corneal reflex, response to supraorbital pressure - CN V and VII
Vestibulo-ocular reflex - CN III and VIII
Gag reflex - CN IX and X
Cough reflex - CN X
Absence of respiratory effort.
7
Q
Transient global amnesia
A
Most attacks last one to eight hours with a mean duration of 4.2 hours. Patients are disoriented to time and place with 60% to 90% exhibiting repetitive questioning, “Where am I?” which may last throughout the attack.
anterograde amnesia, with no clouding of consciousness or loss of personal identity.
Cognitive impairment is limited to amnesia, with no apraxia or aphasia. There is no recent history of head trauma, no history of seizures in the preceding two years.
8
Q
carotid endarctectomy
A
Carotid endartectomy is only indicated if there is >50% in some centres or else >70% stenosis of the symptomatic carotid artery. (Note the external carotid artery supplies the neck, face and skull and the 90% stenosis there is not responsible for the symptoms in this patient).
9
Q
vasculaf dementia
A
Vascular dementia tends to cause a step-wise deterioration in cognition, and one would expect a history or findings of previous cerebrovascular disease.
10
Q
Picks disease
A
Pick’s disease is a rare form of dementia characterised by degeneration of the frontal and temporal lobes.
Presentations are of frontal or temporal lobe syndromes, depending on the location of maximal lobar atrophy. Patients with frontal atrophy present with early personality change.
Predominant temporal lobe atrophy is associated with aphasia and semantic memory impairment.
Pathologically, Pick’s disease is associated with Pick bodies which are argyrophilic inclusion bodies within the neuronal cytoplasm.
EEG is relatively normal by contrast to Alzheimer’s disease.
11
Q
Neuroleptic malignant syndrome
A
The diagnosis is neuroleptic malignant syndrome (NMS) which can occur at any time during the treatment with antipsychotic medications.
Concomitant treatment with lithium or anticholinergics may increase the risk of NMS.
It is manifested by:
Fever
Rigidity
Altered mental status
Autonomic dysfunction, and
Elevated creatine phosphokinase concentration.
The creatine phosphokinase concentration is always elevated (>1000 IU/L−1), reflecting myonecrosis secondary to intense muscle contracture.1
Treatment includes withdrawal of the offending agent and reduction of body temperature with antipyretics.
Dantrolene, bromocriptine or levodopa preparations may be beneficial.
12
Q
cerebellar syndrome
A
The presence of anti-Hu antibody (a type of antineuronal antibody) supports a diagnosis a paraneoplastic syndrome.
Subacute cerebellum syndrome is one of the commonest paraneoplastic syndromes. It is usually associated with small cell lung carcinoma, breast and ovarian cancer.
CT chest, abdomen and pelvis and mammogram are required to look for a primary neoplasm.
A whole body positron emission tomography (PET) scan is preferabl
13
Q
Myasthenia gravis
A
myasthenia gravis presenting with ocular, bulbar and limb weakness.
Late onset myasthenia gravis is associated with thymic atrophy as opposed to thymic hyperplasia.
Forty percent of seronegative patients have an antibody found to postsynaptic protein muscle-specific kinase (MuSK).
Management:
1) pyridostigmine 60 mg QDS
2) if not responding then steroids, low dose
3) respiratory weakness/crisis - IV immunoglobulins or plasmapharesis
14
Q
hemiballismus
A
hemiballismus; the lesion is in the contralateral subthalamic nucleus.
Tetrabenezine
15
Q
hemiballismus
A
hemiballismus; the lesion is in the contralateral subthalamic nucleus.
Tetrabenezine
16
Q
Syringomyelia
A
cervical cord would cause lower motor neurone signs at the level of the syrinx, with dissociated pain and temperature loss and upper motor neurone signs in the legs.
17
Q
Botulism
A
Botulism occurs either from gut colonisation (e.g., ingestion of contaminated home-canned food) or an infected wound.
Clostridium botulinum spores are widespread in soil and aquatic sediment.
Typical initial features include:
Diplopia Ptosis Facial weakness Dysarthria, and Dysphagia. Later, respiratory difficulty and limb weakness occur.
Neuromuscular blockade causes the clinical features.
In botulism, the impaired cholinergic transmission also involves autonomic synapses, causing poorly reactive dilated pupils, dry mouth, paralytic ileus and occasionally bradycardia. Reflexes are depressed or absent, sensation is normal and cerebrospinal fluid (CSF) is normal in botulism.
18
Q
SDH
A
Subdural haematomas are as a result of blood within the outermost meningeal layer between the dura and arachnoid mater and results from tearing of the bridging veins.
19
Q
Extradural haemorrhage
A
Tearing of the middle meningeal artery results in an extradural haematoma.
20
Q
SAH
A
Rupture of the anterior and posterior communicating arteries results in subarachnoid haemorrhage.
21
Q
IIH
A
IIH typically affects young obese women.
Other risk factors include:
The oral contraceptive pill
Treatments for acne (tetracycline, nitrofurantoin, retinoids), and
Hypervitaminosis A.
Presentation is with a headache, which may be severe. There may be loss of peripheral vision and impaired visual acuity if papilloedema is severe. Reduction in colour vision is common and a significant number of patients develop a CN VI palsy.
CT scan is often normal; the diagnosis is confirmed by finding an elevated cerebrospinal fluid (CSF) opening pressure (more than 20 cmH2O). CSF protein, glucose and cell count will be normal.
The differential diagnosis includes venous sinus thrombosis; increased use of MRI has shown that small thromboses are commoner than previously thought in these patients.
MRI and/or MRI venography is essential in these patients.
22
Q
neuralgic amyotrophy
A
proceeded by an upper respiratory tract infection.
Pain around the shoulder is the presenting symptom that is usually very severe.
As the pain starts resolving, weakness begins and usually affects the muscles innervated by the upper brachial plexus (C5-6).
Treatment is conservative. It is usually a self-limiting condition (improvement over weeks to months).
There have been several cases of elevated cerebrospinal fluid protein with neuralgic amyotrophy.
23
Q
vertebrobasilar insufficiency
A
short episodes lasting minutes of loss of consciousness associated with brainstem features (diplopia, dysarthria and dizziness).
They are not posturally related, and on some occasions have been associated with reflex anoxic seizures.
There does not appear to be any history of confusion following these attacks or any precipitating factors. Hyperextension of the neck however can precipitate loss of consciousness, which may have occurred in the hairdressers.
everal vascular risk factors including hypertension, hypercholesterolaemia and probable glucose intolerance
24
Q
HSV encephalitis
A
HSV encephalitis which presents with fever, headache, vomiting, behavioural changes, confusion and seizures. MRI classically shows high signal in the temporal lobes.
25
LIsteria meningitis
Listeria meningitis affects immunosuppressed patients or patients at extremes of age. It can present with reduced conscious level, seizures and cranial nerve palsies
26
Meningococcal meningitis
Meningococcal meningitis presents with headache, neck stiffness, photophobia, vomiting and fever.
27
SAH
Subarachnoid haemorrhage classically presents with a thunderclap headache and neck stiffness
28
TB meningitis
TB meningitis presents gradually with headache, neck stiffness, photophobia, vomiting and fever. It occurs more commonly in those who are immunosuppressed, for example secondary to AIDS.
29
Anterior ischaemic optic neuropathy
Anterior ischaemic optic neuropathy causes persistent monocular visual loss associated with a relative afferent pupillary defect and is the most common cause in patients over the age of 50 years. There is normally a background of hypertension and diabetes.
30
antiphospholipid syndrome
Cerebral venous thrombosis
pulmonary hypertension with right heart strain, mild anaemia and thrombocytopenia with a prolonged activated partial thromboplastin time. The likely cause of the venous sinus thrombosis is anti-phospholipid syndrome leading to a hypercoaguable state.
31
Cerebral amyloid angiopathy
Cerebral amyloid angiopathy (CAA) would cause haemorrhagic strokes but would not explain the right heart strain.
32
ALS MND
The initial clinical manifestation of ALS may occur in any body segment (bulbar, cervical, thoracic or lumbosacral) and may manifest as upper motor neuron or lower motor neuron symptoms or signs. Twenty percent of patients will have onset in the bulbar segment, which most often presents with either dysarthria or dysphagia.
33
pituitary apoplexy
headache sudden onset
cavernous sinus compression (i.e. third and fifth nerve palsy
compression of the chiasm as suggested by the bitemporal hemianopia
expanding mass can compress the cavernous sinus with the Ocular nerves and the trigeminal nerve
SIADH due to tumor
34
anterior artery communicating aneurysms
anterior artery communicating aneurysms are asymptomatic unless they rupture, and so they are usually found either incidentally or when a patient presents with SAH. Some unruptured aneurysms can become symptomatic. Symptoms include headache (which may be severe and comparable to the headache of SAH), visual acuity loss, cranial neuropathies (particularly third nerve palsy), pyramidal tract dysfunction, and facial pain; they are felt to be due to the mass effect of the aneurysm.
35
Wallenberg's syndrome,
Wallenberg's syndrome, or lateral medullary syndrome, is due to infarction of the posterior inferior cerebellar artery resulting in ipsilateral V, IX, X nerve involvement, dissociated sensory loss and ataxia.
36
Weber's syndrome
Weber's syndrome is a mid brain stroke with ipsilateral third nerve palsy and contralateral hemiplegia.
37
VHL
bilateral cerebellar haemangiomas, which have led to compression of the fourth ventricle, and hydrocephalus manifested by headache, papilloedema and vomiting
renal cell carcinoma with secondary polycythaemia, as evidenced by right flank mass, haematuria
haemangioblastomas of the central nervous system (CNS) or retina, and the presence of one associated VHL tumour (renal cell carcinoma, islet cell tumours and adenomas)
38
Occlusion of the anterior cerebral artery
Occlusion of the anterior cerebral artery is rare and can cause transient incontinence, motor and sensory disturbance affecting the leg more than the arm and a transcortical aphasia.
39
Occlusion of basilar artery
Occlusion of basilar artery, posterior cerebral artery and vertebral arteries produces signs and symptoms of posterior and brainstem circulation syndromes.
40
craniopharyngioma
craniopharyngioma compressing the chiasm from above leading to a bilateral inferior quadrantinopia.
some cavernous sinus extension given the trigeminal nerve involvement and also compression of the pituitary stalk, leading to overproduction of prolactin
41
pituitary visual field defect
A pituitary adenoma is more likely to cause a superior bitemporal field defect initially, and one might expect to see signs or symptoms of pituitary dysfunction.
42
fridrivhs ataxia
Friedreich's ataxia is an autosomal recessive condition normally presenting within the first decade. The posterior columns, corticospinal and spinocerebellar tracts are affected leading to cerebellum dysfunction, spastic paraparesis and absent reflexes in lower limbs.
There is also myocardial muscle involvement leading to hypertrophy and heart failure. Bilateral pes cavus and kyphoscoliosis are common and some patients develop diabetes and optic atrophy.
43
Foster Kennedy syndrome
Foster Kennedy syndrome as a result of olfactory meningioma with optic nerve involvement. The presence of optic atrophy on one side with contralateral papilloedema is characteristic of Foster Kennedy syndrome as it is usually due to frontal tumour or tumour within the olfactory bulb compressing the ipsilateral optic nerve and causing raised intracranial pressure.
44
anterior ischaemic optic neuropathy
An anterior ischaemic optic neuropathy secondary to atherosclerosis or temporal arteritis would cause acute monocular visual loss associated with headache.
45
jacksonian seizure
A jacksonian seizure is also known as a focal (partial) motor seizure. In this condition an uncontrolled, spontaneous discharge of electricity from one motor cortex presents with contralateral motor signs. The patient has preserved consciousness as it is a partial seizure and after the seizure it is common to have a Todd's paralysis where the limb is weak.
46
Tuberous sclerosis
CT scan shows evidence of calcified phakomas in the periventricular white matter.
Combined with the clinical findings of spastic weakness and skin stigmata, and history of epilepsy with learning difficulties, the likely diagnosis is tuberous sclerosis. This condition can occur both spontaneously or is inherited in an autosomal dominant form.
Characteristic findings include:
Adenoma sebaceum
Learning difficulties, and
Seizures.
In addition patients can also have:
Retinal hamartomas
Shagreen patches
Ungual fibromas, and
Rhabdomyomas of the heart.
47
multifocal motor neuropathy (MMN).
marked asymmetrical lower motor neurone weakness affecting the right arm/forearm, in absence of sensory findings, with weakness disproportionate to the degree of wasting.
MMN is an autoimmune neuropathy associated in 80% of cases with an elevated anti-GM1 ganglioside antibody and typically presents with progressive weakness affecting an isolated limb without sensory involvement
Treatment with IV immunoglobulin may be very effective in some patients. Cyclophosphamide is also used on a long term basis..
48
Juvenile myoclonic epilepsy
Juvenile myoclonic epilepsy is the commonest of the idiopathic generalised epilepsies.
Seizures types include:
Absences
Myoclonic jerks and
Tonic-clonic seizures which tend to occur within an hour of waking.
Precipitating factors include alcohol, menstruation and sleep deprivation.
49
Lambert Eaton syndrome
This is a paraneoplastic disorder usually associated with small cell lung carcinoma. It is a presynaptic neuromuscular disorder associated with decreased release of acetylcholine from the presynaptic cleft. It is associated with positive voltage-gated calcium channel antibodies.
Clinical features include:
autonomic symptoms
proximal lower limb weakness
reduced reflexes, and
mild ptosis.
50
serotonin syndrome
Serotonin toxicity leads to changes in mental status, systemic changes and motor complications.
Changes in mental status include:
```
confusion
agitation
hypomania
anxiety, and
coma.
Systemic changes include:
```
```
tachycardia
hypertension
nausea
salivation, and
Pyrexia.
Motor complications include:
```
```
myoclonus
hyper-reflexia
seizures
in-coordination, and
tremor.
Muscle rigidity can occur but is more likely in neuroleptic malignant syndrome.
```
Treatment is supportive and involves removing the precipitating drugs, controlling agitation, administering serotonin antagonists (cyproheptadine or methysergide) and controlling hyperthermia.
Dantrolene is a drug used in neuroleptic malignant syndrome for controlling muscle rigidity which is not present in this patient.
51
difference between serotonin syndrome and NMS
NMS develops over days to weeks, whereas serotonin syndrome develops over 24 hours. Serotonin syndrome is characterized by neuromuscular hyperreactivity (tremor, hyperreflexia, myoclonus), while NMS involves sluggish neuromuscular responses (rigidity, bradyreflexia). Hyperreflexia and myoclonus are rare in NMS.
52
PXE
angiod streaks
yellow papules
PXE is a rare heritable connective tissue disorder with autosomal dominant and recessive modes of inheritance. It involves the elastic tissues of the eye, skin and cardiovascular system.
Visual loss can occur by infarction of the visual pathways and is likely to explain the chronic changes of optic disc atrophy in this patient.
Cerebral ischaemia in PXE is caused by small vessel occlusive disease.
Other neurological complications include:
```
Intracranial aneurysms
Subarachnoid and intracerebral haemorrhages
Progressive intellectual deterioration
Mental disturbances, and
Seizures.
```
53
anion gap methanol toxicity
methanol toxicity.
Early signs of toxicity are due to methanol. Later signs are due to its metabolite, formic acid.
Early signs include:
```
Nausea
Vomiting
Headache, and
Confusion.
Formic acid later produces a metabolic acidosis and retinal injury.
```
The laboratory data show a high gap metabolic acidosis.
Anion gap = (Na + K) − (Cl + HCO3); normal range 7-17 mmol/L.
Although elevated, the lactate level does not account for the anion gap. The diagnosis can be made early by measuring the serum methanol and serum formate levels.
Treatment is aimed at:
Eliminating formic acid (alkaline diuresis or haemodialysis).
Correcting acidosis with IV bicarbonate.
Preventing metabolism of methanol to formic acid by administering IV ethanol.
54
Sydenham's chorea.
acute chorea as a result of streptococcal throat infection or Sydenham's chorea.
This tends to occur in children presenting initially with irritability and inattentiveness. This then progresses to generalised chorea with speech involvement occurring in 20% of cases. Those who have had several attacks in childhood may develop chorea in adulthood when exposed to drugs such as the oral contraceptive pill, phenytoin or digoxin, or during pregnancy.
Treatment is for chorea with tetrabenazine or sulpiride and a course of penicillin for acute infection.
55
carotid-cavernous fistula
would cause acute painful, pulsating exophthalmos, in addition to visual loss and diplopia.
56
seizure of unknown aetiology
EEG
MRI/CT head
r/o struvtural cause e.g AVM
57
botulism
clostridium
The clinical presentation of descending weakness with autonomic dysfunction (fixed dilated pupils) is typical of botulism.
It is a neuromuscular junction disorder and therefore nerve conduction studies and EMG are normal.
Repetitive nerve stimulation shows incremental responses, which is diagnostic of botulism.
Cerebrospinal fluid analysis is usually normal
58
selective serotonin reuptake inhibitor (SSRI) discontinuation syndrome
selective serotonin reuptake inhibitor (SSRI) discontinuation syndrome which typically occurs following abrupt withdrawal of SSRIs.
Symptoms can occur within days and can last months following withdrawal and then disappear.
A combination of the following symptoms can develop:
Psychiatric (anxiety, insomnia, mood lability, vivid dreams)
Gastrointestinal (nausea, vomiting), and
Neurological (dizziness, headache, paraesthesia, dystonia, tremor).
It is thought that shorter acting SSRIs, when discontinued or when the dosage is lowered, produce an "anticholinergic rebound," interrupting the production of the acetylcholine.
No specific treatment is given.
59
Korsakoff's syndrome is associated with heavy alcohol use and is characterised by amnesia and confabulation with lack of ability to reason.
recent onset of memory difficulties, mild impairment of attention and concentration and the lack of ability to reason would be more in keeping with Korsakoff's amnesic syndrome
retained features of acute Wernicke's encephalopathy, in particular ataxia and nystagmus, add support to the diagnosis of Korsakoff's amnesic syndrome.
Thiamine is given in established cases to prevent progression of deficits.
60
Cavernous sinus thrombosis
Cavernous sinus thrombosis can present with focal neurological signs, but is frequently accompanied by seizures and a preceding history of headaches, nausea and vomiting; clinical findings include papilloedema, proptosis and cranial nerve signs (III, IV, VI).
61
paradoxical embolus
The clinical findings of wide fixed splitting of the second heart sound and pulmonary flow murmur accompanied by right bundle branch block and right axis deviation might be explained by co-existent pulmonary embolus, though the finding of right ventricular hypertrophy and pulmonary plethora suggests a chronic cause for raised pulmonary pressures.
The most likely explanation is that he has an atrial septal defect with a deep vein thrombosis (DVT) and has suffered a paradoxical embolus.
62
ADEM
ADEM is an acute demyelinating disorder. It is thought to be an autoimmune response to myelin basic protein as a result of a viral illness or vaccination.
Treatment is usually with intravenous steroids, however in some cases where steroids have failed, intravenous immunoglobulin or plasmaphoresis have been used with good effect.
63
Vertebral artery dissection
Vertebral artery dissection is well-recognised cause of stroke in patients under 45 years and is associated with a 10% mortality rate in the acute phase.
Death may occur due to intracranial dissection, brainstem infarction or subarachnoid haemorrhage.
Common causes include:
Structural defects of the arterial wall
Connective tissue disease
Trauma (for example, road traffic accident, sporting injury), and
Chiropractic manipulation of the neck.
The typical clinical presentation is with severe occipital headache followed by focal neurological signs attributable to ischaemia of brainstem or cerebellum.
Other less likely differential diagnoses of stroke in this age group include:
focal seizure
migraine with prolonged aura and migraine variants
multiple sclerosis, and
conversion disorders
64
Treatment of IIH visual loss
Visual loss is the single threatening complication of idiopathic intracranial hypertension (IIH).
Urgent LP shunt is the treatment of choice.
Optic nerve fenestration is an alternative.
65
Aminoglycoside-induced neuromuscular blockad
Aminoglycosides may impair neuromuscular transmission and should not be given to patients with myasthenia gravis; large doses given during surgery have been responsible for a transient myaesthenic syndrome in patients with normal neuromuscular function.
Non-depolarising muscle relaxants (also known as competitive muscle relaxants) compete with acetylcholine for receptor sites at the neuromuscular junction. Non-depolarising muscle relaxants may be divided into the
```
Aminosteroid group (for example, pancuronium, vecuronium), and the
Benzylisoquinolinium group (for example, atracurium).
```
66
Wound botulism
Wound botulism incubation period is four to 14 days. It would present in a similar way to this case with descending, afebrile, symmetric paralysis particularly affecting the cranial nerves. There is also an absence of gastrointestinal symptoms which are common in botulism.v
67
LEMS
proximal weakness, depressed reflexes, and autonomic dysfunction in a smoker patient suggest the diagnosis of Lambert-Eaton myasthenic syndrome (LEMS).
Positive voltage-gated calcium antibodies and electromyogram (EMG) confirm the diagnosis.
autonomic dysfunction
no faitguability
68
PD meds
COMT inhibitors may have a role as an adjuvant treatment in later PD but not in initial pharmacotherapy.
Levodopa, dopamine agonists, and MAO-B inhibitors are the first choice options as there is evidence behind their use in providing symptoms control.
69
Kearns-Sayre syndrome
Kearns-Sayre syndrome produces the classic triad of:
Progressive external ophthalmoplegia
Pigmentary degeneration of the retina, and
Heart block.
Most cases have large mitochondrial DNA mutations.
Ragged-red fibres are seen on muscle biopsy.
70
Listeria meningoencephalitis
Listeria meningoencephalitis affects mainly older patients (more than 50 years old) immunosuppressed (diabetes, excess alcohol, oncology patients). It produces a mixed picture of meningitis and brain stem encephalitis.
The treatment of choice is ampicillin intravenously. Delayed treatment carries a high mortality.
Listeria meningitis should always be considered in patients over 50 years presenting with infective meningitis. In this case the clinical findings of bilateral fifth, sixth and seventh cranial nerve palsies with bulbar dysfunction confirm the brain stem involvement. Brain stem signs in other common bacterial causes of meningitis are rare.
71
mononeuritis multiplex
vasculitis - neutrophilic leukocytosis, high erythrocyte sedimentation rate and primary axonopathy
polyarteritis nodosa and granulomatosis with polyangiitis
72
nterior spinal artery infarct
Sudden onset of anterior cord syndrome causing bilateral paresis is suggestive of an anterior spinal artery lesion
anterior cord syndrome, affecting the legs. There is spastic weakness in association with spinothalamic disruption. sensory level found on examination.
He has several risk factors for arteriosclerosis including:
hypertension
hypercholesterolaemia
diabetes, and
ischaemic heart disease.
73
invertion in intracerebral bleed
low GCS/ cerebellar haemorrhage - neurosurgeons
High bp>170
raised ICP
74
headaches and painful diplopia are:
```
A posterior communicating aneurysm (PCA)
Ophthalmoplegic migraine
Pituitary adenoma
Cavernous sinus thrombosis, or
A medical mononeuritis.
```
cavernous sinus thrombosis are absence of sinusitis or a midface infection, which are common, absence of fever or additional cranial nerve abnormalities.
Although a pituitary adenoma or apoplexy can cause ophthalmoplegia, visual field defects or signs suggestive of pituitary dysfunction would be present.
75
features suggestive of a posterior communicating artery aneurysm
Pupillary dilatation
Ophthalmoplegia, and
Ptosis.
features suggestive of subarachnoid blood (headache, nuchal rigidity and photophobia).
Conventional angiography is the definitive procedure for the detection and characterisation of cerebral aneurysms. Digital subtraction angiography may be helpful in identifying an acutely ruptured aneurysm.
76
cerebellar syndrome
Anti-yo antibodies are found exclusively in female patients with breast or gynaecological malignancies. The role of these antibodies remains elusive although they have an affinity for Purkinje cells.
77
subacute cord degeneration
peripheral neuropathy secondary to vitamin B12 deficiency.
spastic paraparesis with a dorsal column peripheral neuropathy.
Spinothalamic sensory examination is normal
MRI changes in B12 deficiency progress with demyelination involving the dorsal columns. Usually it is in the lower cervical and upper thoracic regions. It may eventually involve the entire dorsal columns.
autoimmune endocrinopathies and pernicious anaemia will be the most likely cause of the vitamin B12 deficiency.
78
carotid artery stenosis management
Carotid endarterectomy is considered if there is significant symptomatic carotid artery stenosis, that is between 70% - 99% although some centres will accept >50%.
One hundred percent carotid artery stenosis usually requires a bypass procedure, as risk of endarterectomy outweighs benefit.
Aspirin is the first choice as antiplatelet treatment and given the elevated cholesterol level a statin should be commenced.
79
uses for botulinum toxin type A
Botulinum toxin type A (or trade name Botox®) acts by decreasing transmission of acetylcholine across the neuromuscular junction in striated muscles, so inducing a temporary flaccid paralysis in the muscle into which it is injected.
Wrist and hand disability due to upper limb spasticity associated with stroke in adults, and use in cases of muscle spasticity, such as cases of cerebral palsy are well recognised indications for the use of Botox®.
Though not licensed, Botox has been used in other medical conditions, including:
Primary axillary hyperhidrosis
Blepharospasm
Strabismus, and
Cervical dystonia.
80
lennox gaustaut
seizures and learning difficulties
81
cerebellar pontine angle tumour.
There is right cerebellar dysfunction in combination with right-sided cranial nerve palsies (VI, VII, and VIII), which emanate from the pons.
82
common peroneal nerve palsy
weakness of ankle dorsiflexion, eversion, diminished sensation of the lateral aspect of leg and dorsum of foot.
83
cluster headaches.
They usually affect men in their 30s and 40s. They occur nocturnally in clusters.
Autonomic symptoms include
```
Nasal congestion
Lacrimation
Conjunctival injection
Ptosis
Eyelid oedema.
The treatment of choice during the acute attacks is either high flow oxygen or subcutaneous sumatriptan.
```
The most commonly used prophylactic agent is verapamil. Other options are lithium and Epilim valproate.
Long term treatment with ergotamine should be avoided due to serious side effects, for example, retroperitoneal fibrosis.
Carbamazepine is the treatment of choice in trigeminal neuralgia and propranolol of migraine
84
CADASIL
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is associated with migraine, recurrent lacunar strokes and dementia.
85
Neuroborreliosis
mononeuritis multiplex involving right IV, V, VI, VII and left VII nerve palsies.
flu-like symptoms and arthralgia.
Investigations reveal a cerebrospinal fluid (CSF) lymphocytic pleocytosis with intrathecal oligoclonal band production.
MRI scan would be in keeping with a demyelinating or inflammatory disorder.
The early phase of the illness is characterised by an expanding rash (erythema migrans) caused by a tick bite, however three to four weeks post bite the rash has faded, and often the patient is unaware of having been bitten.
Diagnosis is by Borrelia burgdorferi serological testing with enzyme-linked immunosorbent assay (ELISA) or immunofluorescence assay (IFA) having good sensitivity (89%) but lower specificity (72%) in detecting patients with Lyme disease.
86
spastic paraparesis in absence of a sensory level, and normal neurology in the upper limb
lesion within the thoracic spine or cerebral cortex
parasagittal meningioma will primarily cause a weakness of the lower limb and cortical sensory loss. Bladder function may be affected and there may be dysfunction of vibration and light touch sense. The CSF findings of raised protein and WCC
87
anti social personality disroder
Anti-social personality disorder presents with a history of violence, impulsive acts and detachment from friends or family.
88
Thoracic outlet syndrome
Thoracic outlet syndrome occurs due to damage of the eighth cervical and first thoracic nerve roots (part of brachial plexus) and/or subclavian vessels either by compression over a cervical or abnormal thoracic rib or an aberrant fibrous band. The patient complains of muscle weakness and atrophy of the hand and wrist. There may be pain, loss of sensation and tingling in the medial forearm and little and first fingers often following carrying heavy items.
Occasionally, cyanosis, Raynaud's phenomenon and swelling of arm can occur.
Syncope is not normally part of this syndrome.
89
subclavian steal syndropme
classic history of subclavian steal syndrome brought on by exercising of his left hand, and associated with a reduction in blood pressure in the left arm.
Subclavian steal syndrome occurs when there is an occlusion proximal to the origin of the left vertebral artery. As a result blood is stolen from the right vertebral artery with resultant basilar insufficiency.
This is manifest by brainstem features such as:
```
Vertigo
Diplopia
Dysarthria, and
Drop attacks.
Risk factors include:
```
Hypertension
Hypercholesterolaemia
Diabetes, and
Connective tissue disorders such as Takayasu's arteritis.
90
korsakoffs syndrome
recent onset of memory difficulties, mild impairment of attention and concentration and the lack of ability to reason would be more in keeping with Korsakoff's amnestic syndrome than
retained features of acute Wernicke's encephalopathy, in particular ataxia and nystagmus, add support to the diagnosis of Korsakoff's amnestic syndrome.
Thiamine is given in established cases to prevent progression of deficits
Korsakoff syndrome is a chronic disorder caused by severe thiamine deficiency. It is characterised by memory problems, confabulation and apathy.
91
antons, claudes, parinauds, fovilles and webers syndrome
Visual anosognosia, or the denial of loss of vision, associated with confabulation in the setting of obvious visual loss and cortical blindness is known as Anton's syndrome.
Claude's syndrome results in ipsilateral third nerve palsy and contralateral cerebellar ataxia and tremor.
Foville's syndrome causes ipsilateral gaze and facial weakness and contralateral hemiparesis of upper and lower limbs.
Parinaud's syndrome causes paralysis of upward gaze and accommodation.
Weber's syndrome results in ipsilateral oculomotor palsy (CN 3 and 4) with contralateral hemiplegia.
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ventrcular dilation
The connection between these sites is via the foramen of Monro, which may be blocked by tumour or can be affected by horizontal shift from an expanding unilateral mass.
Dandy-Walker syndrome is a congenital failure of opening of the foramina of Luschka and Magendie, resulting in dilatation of the entire ventricular system.
The Sylvian aqueduct is the connection between the third and fourth ventricles and if obstructed would cause dilatation of both lateral and third ventricles.
In normal pressure hydrocephalus presenting with gait ataxia, mental dysfunction and sphincteric incontinence, there is dilatation of all the ventricles.
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locked in syndrome
patient has awareness, but is unable to communicate verbally only through eye closure or vertical eye movements
Sudden onset comatose and quadraparesis is suggestive of a pons lesion.
cocaine abuser, and pinpoint pupils together with sudden onset coma and quadriparesis place the lesion within the brainstem (pons).
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miller fisher
Miller-Fisher variant of Guillain-Barré syndrome is a triad of ophthalmoplegia, areflexia and ataxia.
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lebers optic atrophy
Leber's optic atrophy usually affects young men. It causes sequential optic neuropathies in days to weeks. It is typically painless and severe. Visual acuity fails to improve.
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PVS
Permanent vegetative state (PVS) is defined as a state of 'wakefulness without awareness'.
The patient breathes spontaneously without mechanical support, is haemodynamically stable and has cycles of eye closure and opening that resemble a normal sleeping pattern but the patient is inattentive and unaware of his/her surroundings. Patients may have spontaneous movements (moaning, grunting, teeth grinding, roving eye movements) and may also smile, laugh and cry without any apparent reason. Although there may be eye movement, the eyes do not track a moving object. Patients may respond to painful stimuli and may have myoclonus in response to startling stimuli. Primitive reflexes may be present. Posture may become decorticate and plantar responses are commonly extensor.
The condition typically occurs when there is irreversible damage to the cerebral hemispheres but the brain stem remains intact.
Causes include
Head injury
Hypoxic injury (cardiac arrest, carbon monoxide poisoning)
Stroke
Hypoglycaemia
Intracranial infection
End-stage degenerative brain disease (for example, Alzheimer's).
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isolated deep intracerebral haemorrhage in the basal ganglia
commonly - HTN
Others - amyloid abgiopathy, berry aneurysm, tumor, ACM
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Adult onset X linked spinal muscular atrophy,
also known as Kennedy's syndrome, occurs only in males - 50% of daughters are carriers. There may be gynaecomastia. Tongue and facial muscles are particularly affected.
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treatment of spinal cord comp
dex
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treatment of spinal cord comp
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Median nerve
The median nerve innervates the palmar aspect of the lateral three and a half fingers. Sensation to the thenar eminence is supplied via the palmar cutaneous branch of the median nerve, which passes superficially to the flexor retinaculum and is therefore unaffected by carpal tunnel syndrome.
The median nerve also supplies most of the muscles of the thenar eminence, namely the:
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Lateral two lumbricals
Opponens pollicis
Abductor pollicis brevis, and
Flexor pollicis brevis
These can be remembered with the mnemonic LOAF.
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The remaining small muscles of the hand are supplied by the ulnar nerve.
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radial nerve palsy
radial nerve palsy, most likely around the spiral groove. This often occurs as a result of Saturday night palsy, whereby an inebriated patient has caused pressure palsy.
This typically causes weakness of wrist and finger extension and also affects the supinator and brachioradialis (C5/6)
The reflexes are all depressed with sensory loss over the dorsum of the hand, and posterior forearm.
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INO
impaired right eye adduction with nystagmus of the abducting eye would be consistent with right internuclear ophthalmoplegia (INO).
The patient describes binocular horizontal diplopia. The site of lesion is in the medial longitudinal fasciculus in the pons. Because the convergence centre lies in the midbrain convergence is usually normal.
The commonest cause of unilateral INO in a young person in the United Kingdom is multiple sclerosis
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Erb's palsy
is usually the result of traumatic avulsion of the C5 and C6 roots (commonly occurring during delivery at birth) and causes loss of shoulder abduction and elbow flexion with loss of biceps and brachioradialis reflexes.
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Klumpke's palsy,
often the result of a fall that has been stopped by grasping a fixed object with one hand, involves the C8 and T1 roots and causes weakness of small muscles of the hand and of the long finger flexors and extensors, and a sensory disturbance affecting the medial half of the ring finger and little finger.
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benign positional vertigo.
It usually occurs on lying and turning the head to one side.
Hallpike manoeuvre reproduces the symptoms
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Kearns-Sayre syndrome
produces the classic triad of
Progressive external ophthalmoplegia
Pigmentary degeneration of the retina and
Heart block.
Most cases have large mitochondrial DNA mutations.
Ragged red fibres are seen on muscle biopsy.
Although increased cerebrospinal fluid protein concentrations on lumbar puncture and white matter damage on MRI are seen, these changes are not specific to the syndrome.
Cortical blindness is not a complication of the condition.
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neurological manifestations of gluten sensitivity
Coeliac disease can cause neurological symptoms such as peripheral neuropathy and ataxia.
MRI scan of the brain showed cerebellar atrophy and scattered white matter changes.
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limbic encephalitis
paraneoplastic syndrome
Acute to subacute onset of short term memory deficits, with relative preservation of other cognitive functions, is characteristic of limbic encephalitis.2 The memory deficits may be noticed after several weeks of depression, personality change, or irritability.
Seizures can occur and are most often partial complex seizures. Olfactory and gustatory hallucinations are common.
Some patients also develop signs of diencephalic-hypothalamic dysfunction, including drowsiness, hyperthermia, hyperphagia, and, less frequently, pituitary hormonal deficits
indicates the presence of an underlying cancer; the most common underlying malignancy is small cell lung carcinoma (SCLC), followed by testicular cancer, thymoma, and Hodgkin's lymphoma.
anti-Hu
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subacute combine degeneration of spinal cord
Subacute combined cord degeneration is due to vitamin B12 deficiency.
Pernicious anaemia is the commonest cause and is usually associated with other autoimmune diseases (for example, hypothyroidism and diabetes).
Prolonged metformin treatment has also been associated with B12 deficiency (in up to 10% of patients in a large four year clinical trial), therefore B12 levels should be screened every few years in patients taking long term metformin.
The combination of absent reflexes and extensor plantar response is typical and is due to co-existing peripheral neuropathy.
Dorsal column involvement with relative sparing of spinothalamic pathways is also typical.
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SEs Na valproate
Sodium valproate can cause abnormal colour perception and altered visual evoked potentials.
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SEs Vigabatran
Vigabatrin causes visual field loss with patients complaining of blurred vision, oscillopsia, tunnel vision and difficulty in navigation. Visual acuity is preserved.
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SRs catbamazepine
Carbamazepine has been linked with abnormal colour perception and reduced contrast sensitivity, neutropeniam hyponatrenmia
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SEs Levetiracetam
Levetiracetam can cause diplopia.
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SEs topiramate
Topiramate is associated with closed angle glaucoma.
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SEs phenytoin
cerebellar signs
| coarse tremor
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femoral nerve
Femoral neuropathy causes weakness predominantly of the quadriceps.
The femoral nerve is formed by L2-4 roots as part of the lumbar plexus and is predisposed to compression within the psoas muscle. This occurs as a result of haemorrhage into this muscle due to haemophilia, anticoagulation therapy, or trauma.
The main motor component innervates the iliopsoas (a hip flexor) and the quadriceps (a knee extensor). The motor branch to the iliopsoas originates in the pelvis proximal to the inguinal ligament.
The sensory branch of the femoral nerve, the saphenous nerve, innervates skin of the medial thigh and the anterior and medial aspects of the calf.
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obturator nerve
The obturator nerve innervates the adductors of the hip which are intact in this patient. The sciatic nerve innervates the hip extensors and all muscle compartments below the knee which are all intact in this patient.
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clues to a diagnosis of Whipple's disease are:
A background of malabsorption probably misdiagnosed as irritable bowel disease
Characteristic oculomasticatory myorhythmia and myoclonus
Oculoparesis, and
A suspicion of cognitive and hypothalamic involvement.
The diagnosis is made by duodenal biopsy demonstrating PAS positive foamy macrophages throughout the lamina propria and the bacillus Tropheryma whippelii. It is a rare condition and the means of transmission is unknown.
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hemiballism
. The presence of severe flinging movements affecting proximal muscles and following no particular pattern is typical for hemiballism. The site of the lesion is in the contralateral subthalamic nucleus, infarction being the commonest cause.
The patient has several arteriosclerotic risk factors including hypertension, hyperlipidaemia, ischaemic heart disease and diabetes. Usually the flinging movements stop spontaneously in the next 4-8 weeks and tetrabenazine is the treatment of choice. Bilateral ballismus is rare and implicates a metabolic cause usually non-ketotic hyperosmolar coma.
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normal pressure hydrocephalus
Normal pressure hydrocephalus presents with the triad of:
Dementia
Gait disturbance and
Incontinence.
It is associated with episodes of increased pressure related to impaired cerebrospinal fluid (CSF) absorption and therefore may benefit from CSF shunting.
Although the ventricular enlargement described in this case may derive from global atrophy and need not represent hydrocephalus the other features of the case strongly point to the diagnosis.
In equivocal cases therapeutic CSF drainage via lumbar puncture is performed to identify the patients likely to benefit from permanent drainage.
Intracranial pressure monitoring to demonstrate the episodes of increased pressure is a more invasive alternative.
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Features of fat embolism
The history and clinical features of this case suggest fat embolism syndrome. Fat embolism syndrome is a clinical diagnosis secondary to the presence of fat globules in the lung parenchyma and circulation that are typically released following long bone fractures. It requires high index of suspicion and usually presents 12-72 hours after initial injury.
There is a classic triad of:
Respiratory changes - Dyspnoea, tachypnoea and hypoxaemia are early findings. These may progress to respiratory failure and ARDS requiring mechanical ventilation.
Neurological features - Cerebral emboli produce neurological signs in up to 86% cases. They often occur after onset of respiratory symptoms, with a wide spectrum ranging from mild confusion and drowsiness to seizures. Usually there is an acute confusional state with or without focal signs. Most neurological deficits are transient and reversible.
Petechial rash - This is the final component of the triad to develop and occurs in 60% of cases. Rash is seen in the conjunctiva, mucous membranes, skin folds of upper body particularly neck and axilla, appearing within the first 36 hours.
Patients may also develop:
pyrexia
tachycardia
ECG changes (ST segment depression and right heart strain)
fluffy retinal exudates
coagulopathy, and
renal changes (oliguria, lipiduria, proteinuria or haematuria).
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visual loss and cholesterolembolism
Acute monocular painless visual loss is a recognised sign of cholesterol embolism.
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Features of myotonic dystrophy
Features include:
Frontal baldness in men
Atrophy of temporalis, masseters, facial muscle, and
Bilateral ptosis.
Neck muscles, including sternocleidomastoid, are involved early in the course of disease.
Cardiac abnormalities are common and include first degree heart block (shown above) and complete heart block.
Myotonic dystrophy is also associated with:
Intellectual impairment
Gonadal atrophy, and
Insulin resistance.
Diagnosis can be made on electromyogram (EMG) and muscle biopsy.
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Features of NEAD
Clues to NEAD include situational attacks in public and absence of nocturnal events.
There are several features of the attacks which point towards NEAD, including eye closure, red, or normal colouring, prolonged attacks and post-ictal crying. There is a suggestion in the history of previous functional symptoms including appendicectomy with normal histology. Additionally her partner's decision to separate may have led to the onset of her attacks.
Video EEG is the gold standard investigation used to differentiate NEAD from epileptiform events.
Duncan et al.1 write: "Some features traditionally regarded as indicative of epilepsy - such as incontinence of urine and injuries (with the exception of burns) - do not in fact distinguish between epilepsy and Psychogenic Non-Epileptic Seizures."
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symptoms and signs of an anterior cord syndrome, the cause of which is likely to be an acute disc herniation at the level of C5/C6
.
In the lower limb there is upper motor neuron (UMN) weakness and evidence of spinothalamic disruption. In the upper limb there is lower motor neuron (LMN) signs at the level of C5/C6 as evidenced by absent biceps/supinator jerks and weak elbow flexion, and UMN signs below this level (brisk triceps jerk, positive Hoffman's sign, weakness of handgrip, wrist extension, elbow extension).
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Anterior spinal artery infarct
Anterior spinal artery infarct occurs at the 'watershed' T4-T6 and would cause symptoms primarily in the lower limb and a sensory level.
anterior cord syndrome below T4/8
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management of CVT in ET
Essential thrombocytosis (ET) is a chronic myeloproliferative disorder associated with sustained megakaryocyte proliferation, increasing the number of circulating platelets (above 450 ×109/L). It is associated with thrombosis and bleeding.
Thrombotic events may include:
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Stroke
Transient ischaemic attacks (TIAs)
Retinal artery or venous occlusions
Coronary artery ischaemia
Myocardial infarction (MI)
Pulmonary embolism
Hepatic or portal vein thrombosis
Deep vein thrombosis (DVT), and
Digital ischaemia.2
Neurological complications of ET may derive from:1,3,4
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Major artery thrombosis (TIA, stroke, spinal infarct)
Vein thrombosis (CVST and high pressure headache, reduced consciousness, focal neurology)
Microangiopathic complications (amaurosis fugax, reduced visual acuity, erythromelalgia and Raynaud's phenomenon).
Intracranial haemorrhage is a serious bleeding manifestation.5
However, while essential thrombocythaemia may cause primary intracerebral haemorrhage (commonly if platelet counts exceed 1500 ×109/L), secondary haemorrhage is of course a complication of intracranial venous thrombosis.
Clearly this is an important distinction as coming to the wrong diagnostic conclusion might adversely influence treatment - for example, inappropriate withdrawal of antithrombotics in patients with haemorrhagic intracranial venous thrombosis.1
The most appropriate course of action in regard to anticoagulation in this scenario is intravenous unfractionated heparin, with at least a doubled activated partial thromboplastin (a target activated partial thromboplastin time ratio of 1.5-2.5) with later transition to warfarin.6
Low dose aspirin and hydroxycarbamide is part of the treatment for ET in high risk patients (over 60 years, previous thrombotic episode and platelet count >1500 ×109/L)7. This alone will not be sufficient to treat CVST in this scenario.
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negstive auras
negative auras of dark holes and tunnel vision associated with migraine. Dizziness and fatigue are quite common prior to a migraine attack and on some occasions the aura has led to a headache.
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neuro manifestations of ilsons disease
Wilson's disease is an autosomal recessive disorder resulting in copper deposition in organs such as the liver and the basal ganglia. It is
associated with extrapyramidal features (tremor, parkinsonism, dystonia), dysarthria, psychiatric features, liver cirrhosis and Kayser-Fleischer ring.
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lead and mercury poisoning
Lead poisoning can cause symptoms of insomnia, cognitive impairment, delirium, tremor, hallucinations and convulsions.
Mercury poisoning can cause peripheral neuropathy, muscle weakness, problems with coordination and speech impairment.
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HSMN
Mixed motor and sensory symptoms, slowly progressing initially in the lower limbs and then to the upper limbs, together with a family history suggests a diagnosis of hereditary motor sensory neuropathy (HMSN).
Electromyography and nerve conduction studies may distinguish between the demyelinating (type 1) and axonal (type 2) forms.
Genetic testing is available for certain types of HMSN. Genetic testing usually reveals the diagnosis and EMG and NCS help distinguish between the types.
Then the next step is neurophysiology - this will give information about whether this could be a central or root disorder and point to demyelination.
It is less invasive than muscle biopsy (or nerve biopsy) and we do know sufficient information to commence treatment with beta interferon.
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NCS
Nerve conduction studies (NCS) are useful in determining between axonal and demyelinating pathology
Axonal
normal conduction velocity
reduced amplitude
Demyelinating
reduced conduction velocity
normal amplitude
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axonal/de-myelinating
Demyelinating pathology
Guillain-Barre syndrome
chronic inflammatory demyelinating polyneuropathy (CIDP)
amiodarone
hereditary sensorimotor neuropathies (HSMN) type I
paraprotein neuropathy
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Axonal pathology
alcohol
diabetes mellitus*
vasculitis
vitamin B12 deficiency*
hereditary sensorimotor neuropathies (HSMN) type II
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WErnickes encephalopathy
ernicke's encephalopathy. It is characterised by
Confusion
Ataxia
Gaze palsies.
Intravenous thiamine should be given immediately in those situations.
Wernicke's should be considered in the setting of malnutrition or in patients lacking a well-rounded diet.
The commonest cause is chronic alcohol abuse.
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Myotonic dystrophy
Myotonic dystrophy (Steinert's disease) is the commonest adult muscular dystrophy.
Myotonia is often evident at an early age, but the severity is variable and many patients do not present until adulthood.
Distal weakness is often more marked than proximal weakness; there is a typical facial appearance due to atrophy of temporalis, masseter and facial muscles. Men often have frontal balding and some women also have hair loss. Cataracts are common.
Congenital myotonic dystrophy is a more severe form of the disease that occurs in children born to mothers who have established myotonic dystrophy. Babies are hypotonic and frequently require assisted ventilation.
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Klippel-Trenaunay-Weber syndrome
Klippel-Trenaunay-Weber syndrome is a vascular malformation of the spinal cord associated with a cutaneous naevus and can present with spinal segmental or tract lesions.
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Lateral medullary syndrome
Lateral medullary syndrome occurs as a result of posterior inferior cerebellar artery infarct. This is characterised by ipsilateral Horner's syndrome, ipsilateral reduced pain and temperature sensation of the face and other ipsilateral cranial nerve involvement and contralateral loss of pain and temperature sensation of the limbs.
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carotid dissection
Extra-cranial carotid artery dissection presents with haematoma in the neck and possible compression of the sympathetic chain leading to an ipsilateral Horner's syndrome and cranial neuropathies particularly cranial nerves IX, X, XI and XII.
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Lateral medullary syndrome
Lateral medullary syndrome occurs as a result of posterior inferior cerebellar artery infarct. This is characterised by ipsilateral Horner's syndrome, ipsilateral reduced pain and temperature sensation of the face and other ipsilateral cranial nerve involvement and contralateral loss of pain and temperature sensation of the limbs.
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Lateral medullary syndrome
Lateral medullary syndrome occurs as a result of posterior inferior cerebellar artery infarct. This is characterised by ipsilateral Horner's syndrome, ipsilateral reduced pain and temperature sensation of the face and other ipsilateral cranial nerve involvement and contralateral loss of pain and temperature sensation of the limbs.
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B1 defiiency
ataxia, opthalmoplegia and disorientation associated with Wernicke-Korsakoff syndrome. This is as a result of chronic alcohol use and is due to thiamine (vitamin B1) deficiency
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brain lesions
Medial thalamus and mammillary bodies of the hypothalamus -->Wernicke and Korsakoff syndrome
Subthalamic nucleus of the basal ganglia --> Hemiballism
Striatum (caudate nucleus) of the basal ganglia --> Huntington chorea
Substantia nigra of the basal ganglia -->Parkinson's disease
Amygdala --> Kluver-Bucy syndrome (hypersexuality, hyperorality, hyperphagia, visual agnosia
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Neurosyphilis
Neurosyphilis is an infection of the brain and spinal cord in people with untreated syphilis and presents with abnormal gait, incoordination, dementia, seizures, tremors, muscle weakness and incontinence.
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skullbase OM
This is a rare but potentially life threatening condition affecting people with compromised immunity. Typically, Pseudomonas aeruginosa is the causative pathogen. Less common pathogens are Proteus mirabilis, Staphylococcus aureus, Staphylococcus epidermidis.
Usually osteomyelitis of the skull is preceded by a local infection, for example:
Sinusitis extending to the sphenoid sinuses and involving frontal bone may have serious complications such as cavernous sinus thrombosis
Mastoid cell infection and occipital bone osteomyelitis
Necrotising otitis externa, complicated by petrous bone osteomyelitis with cranial nerve involvement (most common site of skull base osteomyelitis).
The clinical scenario depends on the affected part of the skull base in its most common form, that is, petrous bone involvement. Patients suffer from chronic otitis externa with otalgia and otorrhoea, which, if untreated, progress and cause unilateral headache, cranial nerve palsies, most commonly IX, X, XI (jugular foramen content) and include also XII nerve form, Villaret's syndrome.
The usual biochemical picture is raised erythrocyte sedimentation rate (ESR) and normal white cell count (WCC) and C reactive protein (CRP).
The typical imaging finding are signs of bone destruction especially clivus, shown as hypointensity of bone marrow in the clivus and preclival soft tissue infiltration on MRI T1 weighted images.
Diagnosis is confirmed by fine needle aspiration (FNA) of tissue and cultures.
Treatment is with antibiotics.1
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limbic encepalitis
confusion, seizures, agitation, delusions, hallucination, disordered thinking
paraneoplastic syndrome
anti-NDMA positive
causes: ovarian teratoma, malignancy
Rx: steroids, immunoglobulins, cyclophosphomide, plasma exhange
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tubuerous sclerosis
autosomal dominant neurocutaneous disorder
similarities to NF1/2 - cutaneous lesions, AD, ocular harmatomas
cutaneous:
- sunbungal fibromas
- angiofibromas over nose
- ash leaf pathches - hypopigment
- shagreen - rough patches
- cafe au lait spots
neuro
- learning disability
- seizures
- polycystic kidney disease
- retinal harmatomas
- cardia rabdomyomas
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freidreichs ataxia
progessive symptoms in young adults/teens
cns - cerebellar ataxia
pancreas - diabets
heart - cardiomyopathy (HOCM)
genetic test: trinucleotide repeat GAA in FXN gene , autoosmal recessive
optic atrophy
differential: spinocerebellar attaxias e.g ataxia telangiectasia
