Neuro-oncology

Question 1

A 68-year-old man collapsed at home. In the emergency department, he was drowsy and dysphasic, with moderate right-sided weakness (MRC grade 4/5). His left pupil was 5mm, the right was 3mm, and both reacted to light. He had attended 2 weeks earlier because of difficulties with his speech.

What is the neurosurgical DDx

Answer 1

The dysphasia and a right hemiparesis suggest a left cerebral lesion. Unequal pupils may indicate impending transtentorial herniation from mass effect. The speech difficulties two weeks earlier suggest a rapidly expanding mass lesion such as a malignant tumour or a subdural haematoma. The reasons for collapse are unclear from the limited history but he may have suffered a seizure.

Question 2

Answer 2

There is a mass in the left hemisphere (A) surrounded by an extensive area of low density (B) which represents oedema. There is midline shift (C) and compartmental hydrocephalus (demonstrated by the enlarged lateral ventricles on the right (D) due to compression of the ventricular system at the foramen of Monro. There is herniation of the uncus of the left temporal lobe seen on the lower slice (E).

Question 3

What are the three main types of intracerebral oedema

Answer 3

Cytotoxic

Interstitial

Vasogenic

Question 4

Cytotoxic oedema

Answer 4

Mainly in traumatic and ischaemic brain injury

Results from defective sodium ATP-drive transmembrane channels in affected cells.

Leads to Na and subsequently water retention.

It is not responsive to corticosteroids

Question 5

Interstitial oedema

Answer 5

Occurs in hydrocephalus and is due to high CSF pressures in ventricular system resulting in CSF egress into adjacent brain parenchyma

Question 6

Vasogenic oedema

Answer 6

Due to increased capillary permeability from breakdown of BBB, seen principally with tumours and abscesses. Responsive to corticosteroid therapy.

Question 7

A 68-year-old man collapsed at home. In the emergency department, he was drowsy and dysphasic, with moderate right-sided weakness (MRC grade 4/5). His left pupil was 5mm, the right was 3mm, and both reacted to light. He had attended 2 weeks earlier because of difficulties with his speech.

Answer 7

The mass exhibits ring enhancement. The differential diagnosis is between a highgrade glioma, an abscess, and metastasis. In the absence of raised infective markers, a tumour is more likely

Question 8

A 68-year-old man collapsed at home. In the emergency department, he was drowsy and dysphasic, with moderate right-sided weakness (MRC grade 4/5). His left pupil was 5mm, the right was 3mm, and both reacted to light. He had attended 2 weeks earlier because of difficulties with his speech.

CT shows likely tumour

Mx

Answer 8

Risk of rapid deterioration due to raised ICP

Corticosteroids to reduce vasogenic tumour oedema and decompressive surgery.

This gentleman underwent craniotomy and debulking of the tumour.

Question 9

Treatment of glioblastoma after surgical resection

Answer 9

Radiotherapy and temozolomide for patients with good performance status.

Px is 1y even with treatment

Question 10

Fixed pupil

Answer 10

Suggests compression of CN3 to such an extent that neural transmission has been impeded.

Question 11

Use of MRI in differentiation of cerebral tumours from abscesses

Answer 11

DWI MRI can be used to differentiate cystic/necrotic tumour from an abscess.

DWI indicates the degree to which water molecules can diffuse out of cells.

It is typically restricted in abscesses, yielding hyperintense signal on DWI. Tends not to be restricted in tumours.

The pattern on the apperent diffusion coefficient sequence is the opposite.

Abscess ring may appear hypointense on DWI a feature not seen in tumours.

Question 12

Classification of brain tumours

Answer 12

Primary

Secondary

Benign

Malignant

Question 13

Def: Glioma

Answer 13

Brain tumour that arises from the brain parenchyma

Question 14

Grading of gliomas

Answer 14

Now based on molecular factors as per WHO classification.

Previously Low grade WHO I+II

High grade WHO III+IV

Glioblastoma is a grade IV glioma that is the most aggressive

Question 15

Treatment options for glioma

Answer 15

Observation (low grade)

Sx

CTx

RTx

Prognosis variable

Question 16

Typical primary sites for metastasis to brain

Answer 16

Lung

Kidney

Breast

Melanoma

Colorectal

Question 17

Def: Meningioma

Answer 17

Typically but not always benign tumours, that arise from the arachnoid cap cells of the meninges

Question 18

Grading of meningiomas

Answer 18

I benign

II atypical (uncommon)

III malignant (rare)

Question 19

Locations of meningiomas

Answer 19

Parasagittal

Parafalcine

Convexity

Juxtasellar

Olfactory groove

Posterior fossa

Question 20

Aetiology of meningiomas

Answer 20

Sporadic but can be familial.

Radiation exposure

NF2

?COCP, obesity

Question 21

CPA tumours

Answer 21

Vestibular schwannoma

Meningioma

Epidermoid

Ependyoma

Question 22

Def: Vestibular schwanomma

Answer 22

Beign tumours arising from Schwann cells of the vestibulocochlear nerve

Question 23

Bilateral schwannomas are seen in?

Answer 23

NF2

Question 24

Treatment options for schwannomas

Answer 24

Observation

Sx

retrosigmoid

Translabyrinthine approach

RTx

Question 25

Common examples of tumours of the sellar region

Answer 25

Pituitary adenoma Craniopharyngioma Juxtasellar meningioma

Question 26

Classification of pituitary adenomas

Answer 26

Microadenoma \<1cm or macroadenoma \>1cm Secreting or non-secreting

Question 27

A previously well 53-year-old man had developed a sudden onset of severe headache 5 days previously. The headache settled within hours and he did not seek medical advice at the time. He later consulted his GP who sought a neurological referral as he was concerned about an acute vascular migraine. The headache had resolved completely by then, but he had a left-sided homonymous hemianopia. Where is the lesion presumed to be?

Answer 27

To cause a homonymous visual field defect, the lesion must be posterior to the optic chiasm. It may thus be in the right optic tract, thalamus, optic radiation, visual cortex or adjacent structures causing impingement.

Question 28

A previously well 53-year-old man had developed a sudden onset of severe headache 5 days previously. The headache settled within hours and he did not seek medical advice at the time. He later consulted his GP who sought a neurological referral as he was concerned about an acute vascular migraine. The headache had resolved completely by then, but he had a left-sided homonymous hemianopia. DDx

Answer 28

Sudden onset severe headache- ?intracranial bleed. Given presumed location, likely intraparenchymal haemorrhage. Causes include vascular lesions- cavernomas, AVMs Malignant tumours If there is no mass lesion, amyloid angioapthy, HTN and small vessel disease could be precipitating factors

Question 29

A previously well 53-year-old man had developed a sudden onset of severe headache 5 days previously. The headache settled within hours and he did not seek medical advice at the time. He later consulted his GP who sought a neurological referral as he was concerned about an acute vascular migraine. The headache had resolved completely by then, but he had a left-sided homonymous hemianopia. MRI is shown, what is the likely Dx

Answer 29

T2 weighted image shows a lesion of mixed signal intensity adjacent to the primary visual cortex of the right occipital lobe. There is surrounding oedema- high signal. There is no widespread evidence of white matter disease which would be more in keeping with HTN or vasculopathy. Appearances are more in keeping with a tumour. T1 images pre and post contrast show heterogenous enhancement making malignant tumour most likely.

Question 30

What is the most common malignant brain tumour?

Answer 30

Metastatic disease

Question 31

What is the most common primary brain tumour

Answer 31

Glioblastoma

Question 32

Px in glioblastoma

Answer 32

Median survival of 9-15m with treatment

Question 33

Factors influencing Px in glioblastoma

Answer 33

Age at presentation Degree of neurological disability. Treatment factors Younger patients with better performance status survive longer.

Question 34

What are the treatment factors influencing progosis in glioblastoma?

Answer 34

Extent of surgical resection (greater the resection, greater the survival) Provision of adjuvant RTx Adjuvant temozolomide

Question 35

What tumour attributes in glioblastoma may promote survival

Answer 35

MGMT promoter methylation status which if present confers additional benefits of temozolomide treatment

Question 36

What scale can be used to assess performance stauts?

Answer 36

Karnofsky performance status

Question 37

A previously well 53-year-old man had developed a sudden onset of severe headache 5 days previously. The headache settled within hours and he did not seek medical advice at the time. He later consulted his GP who sought a neurological referral as he was concerned about an acute vascular migraine. The headache had resolved completely by then, but he had a left-sided homonymous hemianopia. The patient undergoes a macroscopic resection and glioblastoma is confirmed. He tolerated a full course of radiotherapy with concomitant temozolomide, followed by adjuvant temozolomide for 5 days a week every month. He recovered well but developed headaches a year later. They are progressive and worse in the morning. His visual field improved a little after the surgery but the defect has returned. Repeat MRI below, what does it show

Answer 37

There is tumour recurrence. The T2 sequence (left) shows extensive white matter signal change in the posterior right hemisphere. This ia probably vasogenic oedema, though a similar pattern follows RTx The contrast enehanced T1 image show midline shift and recurrent tumour at the resection site, deeper and more extensive than before.

Question 38

A previously well 53-year-old man had developed a sudden onset of severe headache 5 days previously. The headache settled within hours and he did not seek medical advice at the time. He later consulted his GP who sought a neurological referral as he was concerned about an acute vascular migraine. The headache had resolved completely by then, but he had a left-sided homonymous hemianopia. The patient undergoes a macroscopic resection and glioblastoma is confirmed. He tolerated a full course of radiotherapy with concomitant temozolomide, followed by adjuvant temozolomide for 5 days a week every month. He recovered well but developed headaches a year later. They are progressive and worse in the morning. His visual field improved a little after the surgery but the defect has returned. Repeat MRI shows recurrence. What are his options?

Answer 38

Repeat Sx CTx

Question 39

CTx options if glioblastoma progresses despite temozolomide

Answer 39

PCV CTx or newer agents within clinical trials.

Question 40

PCV CTx

Answer 40

P - procarbazine. C - lomustine (also known as CCNU ®) V - vincristine

Question 41

Repeat surgery in glioblastoma recurrence

Answer 41

Debulking surgery may be considered if patient has a good performance status and the pattern of regrowth suggests surgery could remove a reasonable volume of the tumour with low neurological morbidity. Wound healing following previous Sx and RTx may be problematic. Without further oncological treatment, continued decline may be rapid.

Question 42

A 27-year-old woman presents to the emergency department following a witnessed first grand mal seizure. She was working at her desk, fell to the floor, shook violently for 30 seconds, and was then unconscious. She bit her tongue but was not incontinent. In the emergency department 20 minutes later she is drowsy but responding to voice and obeying commands. Her left arm is weak 1. What factors lower an individual’s seizure threshold?

Answer 42

Various factors Presence of SIRS e.g. sepsis Electrolyte imbalances- Na Certain drugs e.g. antidepressants and tramadol Sleep deprivation Rarely, flashing lights

Question 43

A 27-year-old woman presents to the emergency department following a witnessed first grand mal seizure. She was working at her desk, fell to the floor, shook violently for 30 seconds, and was then unconscious. She bit her tongue but was not incontinent. In the emergency department 20 minutes later she is drowsy but responding to voice and obeying commands. Her left arm is weak What is the significance of the new left arm weakness?

Answer 43

This is a potentially important focal sign which should recover (Todd's paresis) It may relate to a pre-existing mass lesion. The deficit may persist longer if due to an acute lesion e.g. haemorrhage- commonly a cavernoma or AVM rather than aneursym as aneurysms present with ictal headache rather than a fit with focal cortical neurological deficit. Malignant tumours may also present with a haemorrhage

Question 44

A 27-year-old woman presents to the emergency department following a witnessed first grand mal seizure. She was working at her desk, fell to the floor, shook violently for 30 seconds, and was then unconscious. She bit her tongue but was not incontinent. In the emergency department 20 minutes later she is drowsy but responding to voice and obeying commands. Her left arm is weak No obvious precipitating factors identified and CT head performed

Answer 44

The posterior frontal lobe low attenutation (arrow) is probably low grade tumour. Absent contrast enhancement will usually distinguish it from a high-grade (malignant) tumour. With clear edges, a small cortical infarct is another possibility. DWI can help differentiate between infarct and tumour

Question 45

Sagittal T 2 (A) and coronal pre-contrast (B) and post-contrast (C) MRIs are shown in Fig. 31.2 . What is the tumour location and what information does it provide?

Answer 45

The tumour is in the posterior right frontal lobe, close to the motor cortex. Neither CT nor MRI show evidnece of haemorrhage so weakness if likely to be Todds palsy. Coronal sequences show no tumour contrast enhancement so diagnosis is low grade glioma- probably astrocytoma

Question 46

A 27-year-old woman presents to the emergency department following a witnessed first grand mal seizure. She was working at her desk, fell to the floor, shook violently for 30 seconds, and was then unconscious. She bit her tongue but was not incontinent. In the emergency department 20 minutes later she is drowsy but responding to voice and obeying commands. Her left arm is weak Likely astrocytoma on imaging. What are the management options

Answer 46

Anticonvulsants to prevent further seizures. Conservative Surgical

Question 47

Conservative Mx of low-grade glioma

Answer 47

Radioloigcal surveillance. Avoids risks associated with surgery. No histological diagnosis- hence prognosis is uncertain

Question 48

Surgical mx of low-grade glioma

Answer 48

Biopsy- allows for possible further non-surgical treatment e.g. RTx or CTx. May occasionally take a non-representative sample. Resection- near the motor cortex most surgeons would use image guidance or have the patient awake during surgery for intraoperative assessment of the involved brain prior to resection

Question 49

Indications for brain biopsy

Answer 49

Carries appreciable risk and should only be performed after other less invasive diagnostic and management strategies have been considered.

Question 50

What things should factor in making the decision to biopsy the brain.

Answer 50

Potential trajectory and neurological deficit should a complication occur. Biopsy of the meninges and cortex rather than the deeper structures should be performed if diffuse process is being investigated

Question 51

Main complications of brain biopsy

Answer 51

Haemorrhage Oedema

Question 52

Options for brain biopsy

Answer 52

Open Stereotactic

Question 53

Indications for open biopsy

Answer 53

Superficial lesions where there is another indication e.g. relief of mass effect.

Question 54

Features of stereotaxy

Answer 54

Allows the location of an intracranial lesion to be defined in three dimensional coordinates. Either with sterreotactic frame or computer baed image guidance system. Defined site is then accessed via burrhole

Question 55

Risks of stereotactic biopsy

Answer 55

Haemorrhage Post-operative swelling Stroke Seizures Infection Unsuccesful biopsy

Question 56

A 53-year-old woman presented with a 3-week history of dizziness, slurred speech, and numbness of the left side of her face. Describe the MRI scan

Answer 56

Diffuse enlargement of the pons with patchy T2 hyperintesity (A) extending to the middle cerebellar peduncle and left cerebellar hemisphere (B) There is also a small patch of hyperintesnity at the edge of the right internal capsule. There is patchy enhancement with gadolinium She underwent stereotactic biopsy of the internal capsule lesion and histology showed B-cell lymphoma

Question 57

Indications for awake neurosurgery

Answer 57

When intraoperative assessment of neurological function is required e.g. cortical mapping in epilepsy surgery, resection of tumours in eloquent brain areas and DBS for PD or pain. or When patient is not a candidate for GA e.g. elderly patients with chronic SDH or insertion of EVD

Question 58

General approach to awake neurosurgery

Answer 58

Key is effective anaesthetic regimen. Generally LA to scalp incision Analgesia and or anaesthesia.

Question 59

Nociceptors in brain surgery

Answer 59

Brain and skull do not contain nociceptors but meninges and scalp do.

Question 60

A 3-year-old boy has a 6-week history of motor decline. His parents report that previ- ously he was running and playing normally but now is walking slowly and holding on to objects or people to support himself. His GP arranged a non-urgent paediatric out- patient appointment. However, for the past 2 days the child has been irritable and holding his head. This morning he vomited and so came to hospital. What typical diagnosis does the history suggest?

Answer 60

Motor decline can be non-specific but poor balance points to a cerebellar problem. Holding the head, being irritable and vomiting suggests raised ICP. These are typical features of a posterior fossa mass lesion compressing the fourth ventricle and causing hydrocephalus. Although any mass lesion can cause these symptoms, a tumour is the most likely given the age and the progressive nature of his symptoms

Question 61

What are the clincal findings in a patient with a posterior fossa mass lesion?

Answer 61

Cerebellar hemispheric lesion causes loss of coordination ipsilaterally. If the lesion involves the cerebellar vermis, there may be truncal ataxia with a broad based gait. Hydrocephalus in children can cause downward deviation of the eyes- sun setting. Bilateral CN6 nerve palsies (false localising signs). \<18m bulging of anterior fontanelle. Visual acuity may be reduced. Fundoscopy will show papilloedmea

Question 62

Lesion in what portion of the cerebellum may cause truncal ataxia and gait instability

Answer 62

Cerebellar vermis

Question 63

Why do cerebellar lesions cause ipsilateral incoordination

Answer 63

Efferent outputs from the cerebellum to the limbs either double-cross (the dentato-rubro-thalamic tract and the globose-emboliform-rubral tract) or do not cross (fastigial-vestbiular, fastigial-reticular tracts)

Question 64

Which tracts from the cerebellum to the limbs double-cross?

Answer 64

Dentato-rubro-thalamic tract Globose-emboliform-rubral tract

Question 65

Which tracts from the cerebellum to the limbs do not cross the midline?

Answer 65

The fastigial-vestibular and fastigial-reticular tracts.

Question 66

A 3-year-old boy has a 6-week history of motor decline. His parents report that previ- ously he was running and playing normally but now is walking slowly and holding on to objects or people to support himself. His GP arranged a non-urgent paediatric out- patient appointment. However, for the past 2 days the child has been irritable and holding his head. This morning he vomited and so came to hospital. On examination this patient is alert but miserable. Assessment of coordination and fundoscopy is not possible. How should he be investigated?

Answer 66

Urgent brain imaging. MRI is preferable. If there is neurological compromise due to presumed hydrocephalus a CT scan could be perfomed if more readily accesible

Question 67

A 3-year-old boy has a 6-week history of motor decline. His parents report that previ- ously he was running and playing normally but now is walking slowly and holding on to objects or people to support himself. His GP arranged a non-urgent paediatric out- patient appointment. However, for the past 2 days the child has been irritable and holding his head. This morning he vomited and so came to hospital. An MRI is performed What sequence is shown? Describe the abnormalities What are the possible diagnoses?

Answer 67

These are axial T1 post-contrast (L) and FLAIR (right) sequences The T image shows a large mass lesion with a solid component A and large cystic component B arising from the right cerebellar hemisphere and expanding across the midline. The solid component has a strong uniform enhancement pattern with some discrete non enhacning spots in it. Fourth ventricle is not seen and is effaced by the mass. The FLAIR sequence shows lateral ventricular enlargement and the whiter areas around the tips fo the ventricles are indicative of trans-ependymal flow of CSF indicating high pressure. Avidly enhancing solid component with cystic lesion suggests pilocytic astrocyoma. Other childhood tumours include ependyoma and medulloblastoma (primitive neuroectodermal tumour PNET)

Question 68

Location of childhood tumour: Pilocytic astrocytoma

Answer 68

Lateral

Question 69

Location of childhood tumour: Ependymoma

Answer 69

4th ventricle

Question 70

Location of childhood tumour: Medulloblastoma

Answer 70

Usually midline, fourth ventricle

Question 71

Posterior fossa tumour: Solid portion low density on unenhanced CT Solid nodule with large associated cyst

Answer 71

Pilocytic astrocytoma

Question 72

Posterior fossa tumour Often heterogeneous and calcified Grows through fourth ventricle foramina

Answer 72

Ependymoma

Question 73

Posterior fossa tumour, childhood High density on non-contrast CT May have calcification

Answer 73

Medulloblastoma

Question 74

Enhancement of childhood pilocytic astrocytoma vs ependyoma/medulloblastoma

Answer 74

Enhancing nodule with non enhancing cyst vs variable enhancement

Question 75

How does the management of childhood pilocytic astrocytoma differ c.f. medulloblastoma/ependyoma

Answer 75

Pilocytic astrocytoma tend to be benign and are often curable with surgery. The others are malignant and require adjuvant chemotherapy

Question 76

A 3-year-old boy has a 6-week history of motor decline. His parents report that previ- ously he was running and playing normally but now is walking slowly and holding on to objects or people to support himself. His GP arranged a non-urgent paediatric out- patient appointment. However, for the past 2 days the child has been irritable and holding his head. This morning he vomited and so came to hospital. ?Pilocytic astrocytoma. How should this patient be treated in the emergency setting?

Answer 76

Within 24h dexamethasone helps reduce peritumoural oedema. usually allowing CSF flow through to the fourth ventricle to alleviate the hydrocephalus. If the headache does not resolve or the GCS worsens. Treating the hydrocephalus with an EVD or endoscopic third ventriculostomy is required. Even with improvement on steroids, most neurosurgeons would consider an third ventriculostomy a few days after presentation and then plan for resection. Spinal MRI is required to exclude CSF metastasis. This patient does not require drainage but is commenced on dexamethasone and plan for curative excision

Question 77

An 82-year-old woman with 6 weeks of progressive right hemiparesis is referred from the medical team. She denies headaches or seizures and is self-caring and independent, although widowed last year. Her medical history includes type 2 diabetes and hypertension, and she is on aspirin. On examination, she walks well with a stick in her left hand. Her right arm is weak and she is unable to grasp a pen. She has brisk biceps, supinator, and triceps jerks in her right arm. Her left arm and both legs are normal. Damage to which tract typically leads to an inability to grasp a pen or hold small objects?

Answer 77

Impairments of fine motor movements typically arise from lesions of the pyramidal (corticospinal tract)

Question 78

n 82-year-old woman with 6 weeks of progressive right hemiparesis is referred from the medical team. She denies headaches or seizures, and is self-caring and inde- pendent, although widowed last year. Her medical history includes type 2 diabetes and hypertension, and she is on aspirin. On examination she walks well with a stick in her left hand. Her right arm is weak and she is unable grasp a pen. She has brisk biceps, supinator, and triceps jerks in her right arm. Her left arm and both legs are normal. What is the abnormality on her brain MRI (Fig. 33.1)?

Answer 78

An enhancing extra-axial mass over lies the left parietal lobe. It is durally based with a dural tail (arrow), suggesting a meningioma

Question 79

Where is this lesion in relation to the motor cortex

Answer 79

The motor cortex can be identified as follows: Superior frontal sulcus to pre-central sulcus method: superior frontal sulcus (A), joins the precentral sulcus (B) in 85% and hence the central sulcus (C) and the motor cortex (D) can be determined. or Midline sulcus sign: the most prominent sulcus approaching the interhemispheric fissure (E) is the central sulcus in 70% This tumour overlies the sensory areas, behind the post-central gyrus. The motor symptoms are due to mass effect on the pre-central gyrus

Question 80

An 82-year-old woman with 6 weeks of progressive right hemiparesis is referred from the medical team. She denies headaches or seizures and is self-caring and independent, although widowed last year. Her medical history includes type 2 diabetes and hypertension, and she is on aspirin. On examination, she walks well with a stick in her left hand. Her right arm is weak and she is unable to grasp a pen. She has brisk biceps, supinator, and triceps jerks in her right arm. Her left arm and both legs are normal. This tumour overlies sensory areas, behind the post-central gyrus (F). The motor symptoms are due to mass effect on the pre-central gyrus. Does the location suggest any further clinical signs?

Answer 80

Whilst initial sensory examination may be normal, cortical sensory loss may be elicited by asking the patient to carry out tasks of sensory discrimination such as naming a number drawn on her palm or asking to remove objects from a pocket. These tasks require cortical integration of somatosensory stimuli with spatial awareness and decision making.

Question 81

What are the treatment options for meningioma?

Answer 81

Expectant- observation with treatment of seizures if required Surgery Radiosurgery.

Question 82

An 82-year-old woman with 6 weeks of progressive right hemiparesis is referred from the medical team. She denies headaches or seizures and is self-caring and independent, although widowed last year. Her medical history includes type 2 diabetes and hypertension, and she is on aspirin. On examination, she walks well with a stick in her left hand. Her right arm is weak and she is unable to grasp a pen. She has brisk biceps, supinator, and triceps jerks in her right arm. Her left arm and both legs are normal. MRI shows meningioma overlying the sensory area behind the post-central gyrus What treatment option would you prefer for this patient?

Answer 82

This patient has a partial deficit and treatment rather than expectant management is appropriate. Radiosurgery is valuable to control small (\<3cm) tumours that are surgically inaccessible Open surgery has a high cure rate related to the Simpson grade which is highest for the meningiomas of the cranial convexity. Therefore despite her age, surgery was undertaken.

Question 83

Simpson grade

Answer 83

Used for classifciation of meningioma removal

Question 84

Simpson Grade I

Answer 84

Macroscopically complete tumour removal with excision of its dural attachment and of abnormal bone Resection of the sinus where tumour arises from the wall of a dural venous sinus

Question 85

Simpson Grade II

Answer 85

Macroscopically complete tumour removal with endothermy coagulation of its dual attachment

Question 86

Simpson Grade III

Answer 86

Macroscopically complete tumour removal without resection or coagulation of its dural attachment or its extradural extension

Question 87

Simpson Grade IV

Answer 87

Partial tumour removal

Question 88

Simpson Grade V

Answer 88

Biospy only

Question 89

What are the preoperative considerations for meningioma removal?

Answer 89

May bleed considerably during surgery as they derive their blood supply prinicpally from the dura but also recruit vessles from the adjacent brain. Neurovascular embolisation may be performed preoperatively for large tumours. This is rarely necessary for tumours of the convexity as the dural blood supply will be encountered early on in the operation and dealt with readily. Preoperative embolisaiton has a stronger role for skull base tumours or those where the blood supply will be encountered late in the surgery

Question 90

What is the significance of atypical meningioma being found following excision on histology?

Answer 90

Atypical meningiomas are not malignant but have a higher recurrence rate, even after complete excision. Patients should be followed closely with a view to early treatment of recurrence if necessary which may involve further surgery or radiotherapy.

Question 91

A 71-year-old man presents to his GP with a worsening headache which started sud- denly 6 days previously. He became very dizzy and his wife noticed him staggering as if drunk. His balance has been poor since then and he has spent the week in bed. His past medical history includes a stroke 6 years previously from which he recov- ered fully. He is a previous heavy smoker. He takes aspirin and a statin. What is the differential diagnosis?

Answer 91

DDx for sudden onset headache includes any type of intracranial bleed or migraine. Subsequent problems with balance indicate pyramidal motor pathway or cerebellar involvement. A bleed causing hydrocephalus would also lead to unsteadiness.

Question 92

A 71-year-old man presents to his GP with a worsening headache which started suddenly 6 days previously. He became very dizzy and his wife noticed him staggering as if drunk. His balance has been poor since then and he has spent the week in bed. His past medical history includes a stroke 6 years previously from which he recovered fully. He is a previous heavy smoker. He takes aspirin and a statin. The patient’s GP arranges a CT scan and he is then referred to neurosurgery (a) What are the findings on the scans (Fig. 34.1) (left, pre-contrast; right, post-contrast)? (b) What is the likely diagnosis?

Answer 92

a) There is a well defined weakly enhancing mass in the left cerebellar hemisphere crossing the midline. It is heterogeneous on non-contrast CT but high density suggests recent haemorrhage. The fourth ventricle is occluded causing hydrocephalus. There is an old area of right frontotemporal encephalomalacia indicating his previous stroke. b) The most common cause of a posterior fossa tumour in adults is metastasis. Malignant tumours can present with intratumoural haemorrhage. Malignant primary tumours of the cerebellum are most uncommon

Question 93

A 71-year-old man presents to his GP with a worsening headache which started sud- denly 6 days previously. He became very dizzy and his wife noticed him staggering as if drunk. His balance has been poor since then and he has spent the week in bed. His past medical history includes a stroke 6 years previously from which he recov- ered fully. He is a previous heavy smoker. He takes aspirin and a statin. CT shows posterior fossa mass with recent haemorrhage and hydrocephalus. How should this patient be managed acutely

Answer 93

There is hydrocephalus but the patient is alert and does not require immediate surgery. He receives dexamethasone 8mg BD to reduce the vasogenic oedema around the tumour, this will improve the headaches and may improve the hydrocephalus. His aspirin is stoppped in anticipation of a tumour. An urgent effort should made to locate a primary and establish the extent of metastatic dieseae. If prognosis is especially poor, cranial surgery may not be in his best interests. If a histological diagnosis is needed, tissue may be obtained more easily from other sites rathr than a posterior fossa craniotomy.

Question 94

A 71-year-old man presents to his GP with a worsening headache which started sud- denly 6 days previously. He became very dizzy and his wife noticed him staggering as if drunk. His balance has been poor since then and he has spent the week in bed. His past medical history includes a stroke 6 years previously from which he recov- ered fully. He is a previous heavy smoker. He takes aspirin and a statin. CT shows posterior fossa mass with recent haemorrhage and hydrocephalus. How should this patient be managed acutely He undergoes a chest X-ray and a CT of his body (Fig. 34.2). What is seen? How would you manage him now?

Answer 94

There is a right hilar mass posteriorly situated on the CT, consistent with primary lung tumour. Metastatic lung Ca has a poor prognosis, if the patient does not undergo surgery he will probably die of the hydrocephalus in the next few days or weeks. Surgery may extend his life by a few weeks but little more

Question 95

What are the important Ix subsequent to the diagnosis of possible brain metastases?

Answer 95

MRI of the brain to look for small metastases. CT CAP. Tissue should be sampled from wherever is most convenient In practice, when the first presentation of malignancy is with metastatic disease, it is difficult to offer only palliative care without a tissue diagnosis.

Question 96

A 44-year-old nurse is referred by ENT with a 6-month history of right ear tinnitus. Audiography revealed high-frequency hearing loss on this side. She has useful hearing in the right ear and can repeat words whispered to her when the left ear is occluded. There is no other neurological deficit. Three images from the MRI scan are shown in Fig. 35.1. (a) What is the finding?

Answer 96

There is a mass in the right CPA (A) it enhances with contrast and appears to be partly in the internal acoustic meatus (B). There is a little distortion of the brainstem (C) but the fourth ventricle (D) is open and there is no hydrocephalus.

Question 97

A 44-year-old nurse is referred by ENT with a 6-month history of right ear tinnitus. Audiography revealed high-frequency hearing loss on this side. She has useful hearing in the right ear and can repeat words whispered to her when the left ear is occluded. There is no other neurological deficit. Three images from the MRI scan are shown in Fig. 35.1. (a) What is the DDx?

Answer 97

The mass is enhancing and extra-axial. The three most likely possibilities are acoustic neuroma, meningioma and metastasis. The long history and absence of systemic disease symptoms make metastasis unlikely. Acoustic neuroma is more likely of the benign tumours as it is more common in this location and this tumour has an intracanalicular (within the internal acoustic meatus) component which has spilled out into the CP angle- ice cream cone appearance. Meningioma often has a dural tail on imaging

Question 98

A 44-year-old nurse is referred by ENT with a 6-month history of right ear tinnitus. Audiography revealed high-frequency hearing loss on this side. She has useful hearing in the right ear and can repeat words whispered to her when the left ear is occluded. There is no other neurological deficit. MRI shows likely acoustic neuroma. What are the management options?

Answer 98

The three management options are observation, surgery and radiosurgery. Treatment will not improve the patient's symptoms and therefore it should be offered for progression of the tumour and its associated problems. There is evidence to suggest that hearing loss can be minimised with early surgery for small acoustic neuromas but this is not sufficient to justify surgery unless the patient has a very strong preference

Question 99

Observation of acoustic neuromas

Answer 99

Involves MRI scanning at intervals of 6/12 to a year. If tumour growth is demonstrated, treatment will be considered

Question 100

Radiosurgery for acoustic neuroma

Answer 100

Involves image-guided accurate delivery of radiation to small volumes of the brain with the use of collimation to reduce dose to surrounding structures. Complex volumes can be treated usually in a single session

Question 101

Collimation

Answer 101

Radiosurgery using multiple intersecting beams, can be used to reduce the dose to surrounding structures

Question 102

Difference between radiosurgery and radiotherapy for tumours

Answer 102

Radiosurgery gives higher doses per unit volume and causes focal vascular changes that slowly result in intimal thickening and vascular occlusion of a region of interest in contrast to radiotherapy which relies on DNA damage. In the case of tumours benign or malignant, this is intended to control (slow growth or shrink) the lesion. It may also be used for AVMs and other vascular lesions. If it fails surgery may still be undertaken later. High rates of control for small acoustic neuromas (\<3cm diameter) are reported. There are concerns over the rare malignant transformation of benign tumours. Radiosurgery will usually preserve the residual hearing function which may remain useful.

Question 103

Open surgery for acoustic neuromas

Answer 103

Usually curative and this may be psychologically preferable for the patient. The risks relate principally to the lower cranial nerves, particularly CN7 and 8. Facial weakness can be minimsed by the use of intraoperative facial nerve monitoring.

Question 104

Facial nerve injury in surgical resection of acoustic neuroma

Answer 104

Risks of facial nerve injury increase with size of tumour. This has resulted in an interest in subtotal resection of large acoustic nueromas with the aim of preserving facial nerve function. The remaining tumour can then be observed for growth or treated up front with stereotactic radiosurgery.

Question 105

A 44-year-old nurse is referred by ENT with a 6-month history of right ear tinnitus. Audiography revealed high-frequency hearing loss on this side. She has useful hearing in the right ear and can repeat words whispered to her when the left ear is occluded. There is no other neurological deficit. MRI shows an acoustic neuroma. The patient opts for surgical resection. This is performed via a retrosigmoid approach in the prone position. Facial nerve monitoring is used to identify and spare the sev- enth nerve which is seen to be well preserved at the end of the operation which completely removes the tumour. Her facial nerve function is unaffected. The patient returns to the emergency department after 3 days having noticed drooping of the right side of her mouth which has come on gradually over the previous 24 hours. When she blinks, her right eye is slow to close. What is the likely diagnosis and how should it be managed?

Answer 105

The patient has a right LMN facial nerve palsy. The facial nerve was known to be intact at the end of the operation. The diagnosis is delayed swelling of the nerve. She requires imaging to exclude a delayed post-operative haematoma

Question 106

What is an objective tool for the measurement of facial weakness?

Answer 106

House-Brackmann scale

Question 107

House-Brackman I

Answer 107

Normal symmetrical function in all areas

Question 108

House-Brackman 2

Answer 108

Slight weakness noticeable only on close inspection Complete eye closure with minimal effort Slight aysmmetry of smile with maximal effort Synkinesis barely noticeable, contracture or spasm absent

Question 109

House-Brackman 3

Answer 109

Obvious weakness but no disfiguring May not be able to lift eyebrow Complete eye closure and strong but asymmetrical mouth movement with maximal effort Obvious but not disfiguring synkinesis mass movement or spasm

Question 110

House-Brackman 4

Answer 110

Obvious disfiguring weakness Inability to lift brow Incomplete eye closure and asymmetry of mouth with maximal effort. Severe synkinesis, mass movement, spasm

Question 111

House-Brackman 5

Answer 111

Motion barely perceptible Incomplete eye closure, slight movement of corner of mouth Synkinesis, contracture and spasm usually absent

Question 112

House-Brackman 6

Answer 112

No movement, loss of tone, no synkinesis, contracture or spasm

Question 113

A 44-year-old nurse is referred by ENT with a 6-month history of right ear tinnitus. Audiography revealed high-frequency hearing loss on this side. She has useful hearing in the right ear and can repeat words whispered to her when the left ear is occluded. There is no other neurological deficit. MRI shows an acoustic neuroma. The patient opts for surgical resection. This is performed via a retrosigmoid approach in the prone position. Facial nerve monitoring is used to identify and spare the sev- enth nerve which is seen to be well preserved at the end of the operation which completely removes the tumour. Her facial nerve function is unaffected. The patient returns to the emergency department after 3 days having noticed drooping of the right side of her mouth which has come on gradually over the previous 24 hours. When she blinks, her right eye is slow to close. An MRI is performed, waht does it show?

Answer 113

There is a small area of high FLAIR signal in the lateral pons, this is consistent with postoperative oedema

Question 114

What is the managment of postoperative oedema causing facial nerve palsy after surgical resection of acoustic neuroma

Answer 114

The facial nerve palsy should improve with conservative management. A short course of steroids is sometimes given. The ipsilateral eye must also be addressed, if the corneal reflex is absent the patient is at risk of corneal ulceration. If the facial weakness prevents full eye closure, which is the key distinction between House-Brackmann grades 3 and 4, the risk is greater. Eye care involves application of artificial tears or lubricant several times a day. The patient should be shown how to tape the eye shut, particular at night. If eye closure remains a problem it can be suturedshut with a fine nylon stitch or a lateral tarsorrhaphy can be performed or a gold weight can be attached to the upper eyelid to facilitate closure

Question 115

What are the three surgical approaches for acoustic neuroma?

Answer 115

Translabyrthinthine Subtemporal Retrosigmoid

Question 116

Facial nerve monitoring during surgical resection of acoustic neuroma

Answer 116

Considered mandatory for all approaches to minimise the risk of facial nerve injury

Question 117

Translabyrinthine approach

Answer 117

Curved incision behind the ear and drills through the petrous temporal bone and semicircular canal towards the triangle of dura between the sigmoid sinus, petrous apex and jugular bulb inferiorly. Acoustic nerve is idenitfied early where it is distinct from the facial nerve. It is considered preferable for small tumours lateral in the internal acoustic meatus. It results in complete sensorineural hearing loss but good rates of facial nerve preservation

Question 118

Subtemporal (middle fossa) approach

Answer 118

Involves elevating the dura of the middle fossa from anterior to posterior and then drilling the medial part of the petrous apex to expose the internal acoustic meatus It offers the highest rate of hearing preservation for small intracanalicular acoustic neuromas. It poses risks to the posterior temporal lobe and the vein of Labbe with consequent venous infarction

Question 119

Which approach to acoustic neuroma provides the greatest preservation of hearing?

Answer 119

Subtemporal approach

Question 120

What are the risks associated with the subtemporal appraoch

Answer 120

Risks to posterior temporal lobe and vein of Labbe with subsequent venous infarction

Question 121

Retrosigmoid approach

Answer 121

Provides access to the CPA for acoustic neuromas as well as other tumours. Access to the intracanalicular component of the tumours is more difficult. It involves a posterior fossa craniotomy or craniectomy behind the sigmoid sinus with retraction of the cerebellum medially. It allows CSF drainage via fenestration of the cisterna magna to improve brain relaxation and hence operating conditions

Question 122

A 27-year-old hairdresser presents to her local emergency department with progres- sive headaches, vomiting, and blurred vision over 2 weeks. She has been waking up at night and vomiting profusely in the mornings. Over the past 24 hours her headaches have been unremitting. She is alert and orientated. Her power is preserved throughout and coordination is normal. She is not photophobic or meningitic. She has reduced visual acuity at 6/12 in the right eye and 6/18 in the left. On fundoscopy there is gross papilloedema. This patient has features of increasing intracranial pressure without focal neuro- logical signs or symptoms. What is the differential diagnosis?

Answer 122

The DDx is a large SOL in a non-eloquent area such as the right frontal or temporal lobe. The short history makes a malignant tumours more likely than a benign tumour. The second option is hydrocephalus. There are many causes presenting in adulthood, most will also have symptoms of the causative condition such as meningitis, SAH or cerebellar tumour. Presentation with hydrocephalus alone suggests small mass lesions around the third ventricle, cerebral aqueduct and fourth ventricle

Question 123

DDx for small lesions causing hydrocephalus presenting in adults

Answer 123

Colloid cysts of the third ventricle Pineal region tumors Fourth ventricular tumours including choroid plexus tumours and ependymoma Aqueduct stenosis commonly presents in the neonatal period but may also present in delayed manner in older patients

Question 124

A 27-year-old hairdresser presents to her local emergency department with progres- sive headaches, vomiting, and blurred vision over 2 weeks. She has been waking up at night and vomiting profusely in the mornings. Over the past 24 hours her headaches have been unremitting. She is alert and orientated. Her power is preserved throughout and coordination is normal. She is not photophobic or meningitic. She has reduced visual acuity at 6/12 in the right eye and 6/18 in the left. On fundoscopy there is gross papilloedema. She undergoes an MRI, describe the findings

Answer 124

There is a welll defined homogenously enhanving mass in the posterior aspect of the third ventricle with a small cyst superiorly. There is marked associated hydrocephalus. The basal CSF cisterns including the pre-pointine cistern are patent

Question 125

What is the differential diagnosis for a patient with a welll defined homogenously enhancing mass in the posterior aspect of the third ventricle?

Answer 125

This is a pineal region tumours, germinomas and teratomas are the most common. Other pineal region tumours incude pineocytoma or its malignant counterpart pineoblastoma Astrocytomas Meningiomas In an older patient one might suspect a metastasis

Question 126

A 27-year-old hairdresser presents to her local emergency department with progres- sive headaches, vomiting, and blurred vision over 2 weeks. She has been waking up at night and vomiting profusely in the mornings. Over the past 24 hours her headaches have been unremitting. She is alert and orientated. Her power is preserved throughout and coordination is normal. She is not photophobic or meningitic. She has reduced visual acuity at 6/12 in the right eye and 6/18 in the left. On fundoscopy there is gross papilloedema. MRI shows a large lesion in the pineal region with associated hydrocephalus and patent basal CSF cisterns. How should she be managed?

Answer 126

Steroids should be given, though as there is no visible perilesional oedema improvement with steroids may be minimal. The hydrocepalhus should be treated with either an EVD or ETV. ETV allows physiological CSF resorption and avopids external hardware with attendant risk of infection. Depending on the angle of approach, it may also allow tumour biopsy if positioned posteriorly in the third ventircle after creating the ventriculostomy

Question 127

What is the value of tumour markers in pineal region tumours

Answer 127

Tumour markers in the serum and CSF can aid diagnosis although they do not provide a definitive diagnosis. They can also be used to monitor response to treatment

Question 128

Pineal region tumour AFP -ve BHCG positive PALP positive Melatonin negative

Answer 128

Germinoma

Question 129

Pineal region tumour AFP + BHCG - PALP - Melatonin -

Answer 129

Teratoma

Question 130

Pineal region tumour AFP + BHCG - PALP +/- Melatonin -

Answer 130

Yolk sac tumour

Question 131

Pineal region tumour AFP + BHCG + PALP + Melatonin -

Answer 131

Embryonal carcinoma

Question 132

Pineal region tumour AFP - HCG + PALP +/- Melatonin -

Answer 132

Choriocarcinoma

Question 133

Pineal region tumour AFP - BHCG - PALP - Meltaonin +

Answer 133

Pinealocytoma/Pinealoblastoma

Question 134

Surgical management of pineal region tumour

Answer 134

Pineal region is surgically challenging. The most common approach is the supracerebellar infratentorial approach. With the patient in a sitting position, craniotomy is performed over the cerebellum to allow it to fall away from the tentorium. Access to the pineal region is then through the corridor above the cerebellum and below the tentorium.

Question 135

Which anatomical structures are at risk during surgical approach to pineal region tumours

Answer 135

Contains a large number of large venins including the internal cerebral vein that join to form the vein of Galen which feeds into the straight sinus in the tentorium. The approach to craniotomy will be near the transverse sinus which can bleed torrentially if opened and associated risk of intraoperative air embolism

Question 136

Pathophysiology of intraoperative air embolism.

Answer 136

If a patient is sitting upright and the venous sinus is inadvertently opened, there is a risk of air embolism. Cerebral venous pressure in the sitting position is lower then atmospheric pressure and hence open veins may suck air in. The anaesthetist must be prepared. The treatment invovles emergency repositiong of the patient or central venous line in the right atrium to evacuate air with a syringe

Question 137

What investigation should be performed prior to an operation in which there is a risk of air embolism

Answer 137

Bubble echo study looking for ASD to assess for risk of arterial emboli

Question 138

A 48-year-old man was referred to the neurosurgical service by the neurologists with a 3-year history of intermittent headaches. He denies any other symptoms. A CT scan is obtained, what are the abnormalities.

Answer 138

There is a well circumscribed mass within the third ventricle abutting the left foramen of Monro (A). It is hyperdense on this non-contrast CT. This s a colloid cyst of the third ventricle, there is no associated hydrocephalus. There is also an incidental left temporal arachnoid cyst (B) with scalloping of the bone (C). This is a developmental abnormality that manifests as a widened cortical CSF space.

Question 139

A 48-year-old man was referred to the neurosurgical service by the neurologists with a 3-year history of intermittent headaches. He denies any other symptoms. CT shows a colloid cyst An MRI scan is perofmred. What are rthe properties of the cyst and why?

Answer 139

The T2 (left) and T1 (right) scans are sbown. The cyst is hypointense on T2 and hyperintesnse on T1. This is due to presence of protein withint he cyst which also gives it high density on CT

Question 140

Mx of a patient with headaches from a colloid cyst that is not causing hydrocephalus

Answer 140

The concern with colloid cysts is that they can cause rapid deterioration from acute hydroephalus and potentially death. This is usually preceded by a period of headaches which may last for weeks and months or for a few hours only. Treatment is thus conservative unless the patietns has headaches, hydrocephalus or a cyst of diameter \>10mm. Sudden death is only reported in patients with cysts of this size. Surgery to remove the cyst is performed transcallosally or transcortically via the lateral ventricle. Endoscopes may be used. A third option for patients with large cysts that are a higher intesnity on T2 and therefore suspected to be liquid is stereotactic aspiration.

Question 141

34-year-old woman who is otherwise well. One afternoon she devel- oped headaches which prevented her from sleeping after lunch. She started vomiting shortly afterwards and became confused. By the time she arrived at the emergency department she was grunting occasionally, localizing to pain, and not opening her eyes. Her blood pressure was 180/110mmHg and she was intubated. An urgent CT scan is performed. What does it show?

Answer 141

This is a colloid cyst of the third ventricle with acute hydrocephalus. This is the acute presentation of colloid cysts and is life threatening without immediate management

Question 142

34-year-old woman who is otherwise well. One afternoon she devel- oped headaches which prevented her from sleeping after lunch. She started vomiting shortly afterwards and became confused. By the time she arrived at the emergency department she was grunting occasionally, localizing to pain, and not opening her eyes. Her blood pressure was 180/110mmHg and she was intubated. CT shows acute hydrocephalus 2o to third ventricular colloid cyst. What is the correct emergency management for this patient?

Answer 142

She should be transferred immedately to theare for CSF drainage. The priority is to relieve the hydrocephalus rather than to remove the cyst itself. It would be reasonable to administer mannitol. On arrIval in theatre both her pupils were 5mm and unreactive. She underwent immediate bilateral EVD insertion

Question 143

34-year-old woman who is otherwise well. One afternoon she devel- oped headaches which prevented her from sleeping after lunch. She started vomiting shortly afterwards and became confused. By the time she arrived at the emergency department she was grunting occasionally, localizing to pain, and not opening her eyes. Her blood pressure was 180/110mmHg and she was intubated. CT shows acute hydrocephalus secondary to colloid cysts. She is taken immediately to theatre and undergoes bilateral EVD insertion. Following surgery the patient returns to the ITU. Her pupils are smaller and sluggishly reactive. She remains sedated and intubated. Twelve hours later her EVDs stop draining and a repeat CT scan is performed (Fig. 37.5). What has happened and should the drains be changed?

Answer 143

There is widespread supratentorial cerebral infarction and generalised brain swelling as a result of the severe raised ICP. The EVDs are appropriately sited and the ventricles are obliterated. Changing the drains at this stage will be futile as brainstem death will soon ensue

Question 144

Criteria for confirmation of brain death Clinicians

Answer 144

Two clinicians with 5y experience, one of whom is a consultant. Neither members of the transplant service. These two clinicians must perform the tests independently and if the patient satisfies the criteria for both clinician the patient is certified brain dead at the time of the first set of tests.

Question 145

What criteria must be fulfilled before brainstem testing is undertaken?

Answer 145

Aetiology of the event should be known Unconscious state must not be attributable to drugs either pre or intrahospital Hypothermia and metabolic, chemical and respiratory imbalance must be corrected to give the injured brain the optimum conditions to demonstrate some response

Question 146

What are the tests for brainstem death

Answer 146

Pupillary response Corneal reflex VOR Painful stimulus Gag reflex Respiratory response

Question 147

Pupillary response compatible with brainstem death

Answer 147

Pupils fixed and dilated No light reflex

Question 148

Corneal reflex consistent with brainstem death

Answer 148

Absent

Question 149

How to assess VOR in brainstem testing

Answer 149

50mL ice-cold water instilled to external auditory meatus

Question 150

VOR response consistent with brainstem death

Answer 150

No eye movements seen

Question 151

How to assess respiratory response in brainstem death testing

Answer 151

Preventilate with 100% O2 and then disconnect from the ventilator, allowing CO2 to rise without associated hypoxia. Measure blood gas to confirm raised PCO2

Question 152

Respiratory response consistent with brainstem death

Answer 152

No respiratory effort at PaCO2 \>6.65kPa

Question 153

Pitfalls in using painful stimuli to assess brainstem death

Answer 153

Limb and trunk movements may be seen in brainstem dead patients as part of an exaggerated series of spinally mediated reflxes. They may occur in response to light, touch, turning or other minor stimuli. Painful stimuli to diagnose brainstem death should be applied to cranially innervated region, typically the supraobital ridge.

Question 154

A 31-year-old man presents via the ophthalmology department. He has a 2-week his- tory of progressive difficulty with his vision, particularly bumping into things that he did not realize were next to him and having to turn excessively to either side to see people that are talking to him. The events culminated in a car accident when he drove into a parked car on his side of the road. What type of visual problem does he have?

Answer 154

Bumping into objects in the periphery and needing to turn excessively to see people points to a visual field defect affecting the temporal visual fields

Question 155

A 31-year-old man presents via the ophthalmology department. He has a 2-week his- tory of progressive difficulty with his vision, particularly bumping into things that he did not realize were next to him and having to turn excessively to either side to see people that are talking to him. The events culminated in a car accident when he drove into a parked car on his side of the road. What is the likely diagnosis from the history?

Answer 155

A bitemporal field defect is caused by compression of the central part of the optic chaism and is the typical finding in patients with large pituitary tumours.

Question 156

A 31-year-old man presents via the ophthalmology department. He has a 2-week his- tory of progressive difficulty with his vision, particularly bumping into things that he did not realize were next to him and having to turn excessively to either side to see people that are talking to him. The events culminated in a car accident when he drove into a parked car on his side of the road. Given the likely diagnosis of pituitary tumour, what other symptoms might be elicited in the history?

Answer 156

Lethargy Loss of sex drive Impotence Features of pathological hormone secretion. Galactorrhoea, loss of body hair and impotence Most pituitary tumours large enough to present with visual failure will be nonfunctioning

Question 157

A 31-year-old man presents via the ophthalmology department. He has a 2-week his- tory of progressive difficulty with his vision, particularly bumping into things that he did not realize were next to him and having to turn excessively to either side to see people that are talking to him. The events culminated in a car accident when he drove into a parked car on his side of the road. A pre- and post-contrast CT scan is performed (Fig. 38.1). What does it show? What is the differential diagnosis?

Answer 157

There is a well defined mass eroding the right side of the sella turcica (A) from which it arises into the suprasellar region. It enhances uniformly. The most likely diagnosis is a large pituitary tumour, the differential diagnosis is for other causes of tumour in this region which include craniopharyngioma and meningioma. In this case, the bony erosion suggests that the mass has arisen in the pituitary fossa and there is almost certainly a pituitary tumour

Question 158

A 31-year-old man presents via the ophthalmology department. He has a 2-week his- tory of progressive difficulty with his vision, particularly bumping into things that he did not realize were next to him and having to turn excessively to either side to see people that are talking to him. The events culminated in a car accident when he drove into a parked car on his side of the road. CT shows a likely pituitary tumour. What are the next steps in high management?

Answer 158

The patient has visual failure due to this tumour so it requires treatment before it deteriorates. Formal visual field examination with Goldman fields to document extent of visual failure. Definitive imaging in the form of MRI pituitary fossa. This will clarify the diagnosis and show the relationship of the tumour to optic chiasm. A full pituitary hormone screen.

Question 159

What are the components of a pituitary function screen

Answer 159

Early morning cortisol FSH/LH TSH GH Prolactin levels

Question 160

What are the three horones most commonly secreted by pituitary tumours?

Answer 160

ACTH GH Prolactin

Question 161

Why is an urgent prolactin level necessary in someone presenting with a pituitary mass

Answer 161

Initial treatment is then medical with cabergoline or bromocriptine

Question 162

A 31-year-old man presents via the ophthalmology department. He has a 2-week his- tory of progressive difficulty with his vision, particularly bumping into things that he did not realize were next to him and having to turn excessively to either side to see people that are talking to him. The events culminated in a car accident when he drove into a parked car on his side of the road. An MRI is performed

Answer 162

This confirms sellar expansion due to the pituitary tumour. On the T2 image, the optic chiasm can be seen stretched over the tumour accounting for the visual failure.

Question 163

A 31-year-old man presents via the ophthalmology department. He has a 2-week his- tory of progressive difficulty with his vision, particularly bumping into things that he did not realize were next to him and having to turn excessively to either side to see people that are talking to him. The events culminated in a car accident when he drove into a parked car on his side of the road. CT shows a pituitary mass which is confirmed on MRI. He undergoes a pituitary function screen pre-op. The prolactin level in this patient is moderately elevated (93ng/mL, normal range \<20ng/mL). What is the relevance of this result, and how would it affect management?

Answer 163

This is probably due to compression of the pituitary stalk by the tumour resulting in loss of hypothalamic dopamine mediated inhibition of prolactin release from the pituitary gland. The result is a prolactin level elevated a few times that of normal. This level is aslo seen with small prolactin-secreting tumours- microprolactinomas. If a large tumour causing visual failure were secreting prolactin the serum levels would be typically increased a few hundred fold. As the prolactin level is not grossly elevated, the diagnosis is a non-functioning pituitary marcro-adenoma. In the context of sellar expansion and an optic chiasm which is clearly elevated, the surgical approach should be trans-sphenoidal

Question 164

How should hormonal replacement be managed in the perioperative period for a patient undergoing transphenoidal pituitary resection for nonfunctioning adenoma?

Answer 164

Depends on the preoperative status. If he has been shown biochemically or clinically to have impaired pituitary function he will already be on steroid replacement and this should continue post-operatively. The dose should be increased on the day of surgery and for the subsequent days and then retruned to maintenance around day 4. If he has normal preoperative pituitary function, perioperative steroid replacement should still be given. It is reasonable to measure the morning cortisol level prior to giving steroid replacement on days 4 and 5 postoperatively and if normal, steroid replacement is stopped.

Question 165

A patient undergoes trans-sphenodial pituitary resection. He undergoes trans-sphenoidal surgery which is uneventful. Immediately afterwards he feels that his vision has improved. However, the evening after surgery his urine output increases to a steady 300 mL/hour. What is the differential diagnosis for the increased urine output and management of each possibility?

Answer 165

There are two possibilites, firstly he may have received excess perioperative fluid and he is offloading this physiology- this can be managed expectanlty. The second more important diagnosis is diabetes insipidus due to loss of ADH which is a common transient complication of pituitary surgery.

Question 166

Management of cranial DI secondary to pituitary surgery

Answer 166

Supply or water and instruction to drink to thirst. Synthetic ADH may be given subcutaneously. The Na level should be monitored and fluid balance charts maintained until day 3 post-op

Question 167

A 31-year-old man is referred by endocrinologists. His GP had found hypertension. He was referred for investigation of the underlying cause and was found to have coarse facial features, thickening of the skin around the hands, and a large jaw. After direct questioning he admitted that his appearance had changed somewhat over the last few years but he assumed that he was ageing normally Dx?

Answer 167

Acromegaly

Question 168

What are the clinical features of acromegaly?

Answer 168

Coarse facial features Thickening of skin around hands and large jaw. Prominent supraorbital ridge Prognathism Increased interdental spaces. HTN DM Carpal tunnel OSA Visual failure Increased risk of CRC

Question 169

How can the diagnosis of acromegaly be confirmed?

Answer 169

OGTT- oral glucose should suppress GH levels to below 1ng/mL Serum IGF-1 can also be used

Question 170

What are the common causes of excess growth hormone secretion

Answer 170

Most common is pituitary adenoma Ectopic GH secretion can also occur from breast, lung and ovarian tumours as well as from carcinoid and hypothalamic tumours

Question 171

If a patient has biochemical results suggestive of acromegaly, what imaging should they undergo?

Answer 171

MRI pitutiary to look for an adenoma

Question 172

A 31-year-old man is referred by endocrinologists. His GP had found hypertension. He was referred for investigation of the underlying cause and was found to have coarse facial features, thickening of the skin around the hands, and a large jaw. After direct questioning he admitted that his appearance had changed somewhat over the last few years but he assumed that he was ageing normal MRI images are shown (left is pre-contrast, right post-contrast) What are the findings?

Answer 172

The post contrast image shows diffuse enhancement of the pituitary gland and a non-enhancing within it on the right side of the gland (B) which is the adneoma. Pituitary adneomas are opposite to most cranial and spinal tumours as the normal pitutiary enhances whilst tumour does not. The scans shows the relationship of the pituitary gland to the optic chiasm (C), confirming their separation hence visual failure is not imminent.

Question 173

What are the nonsurgical options for the mangement of pituitary adenoma causing acromegaly?

Answer 173

Medical treatment cosnsists of somatostatin analogues to suppress GH production. These require repeated injections every 2-4/52 and although they may be used for long-term control, the usual treatment is surgical. RTx will be an option in the future if medical and surgical treatment fail

Question 174

Approach tor resection of pituitary tumours?

Answer 174

Transphenoidal either open or endoscopic dependent on local preference

Question 175

Risks of surgery for pituitary adenoma causing acromegaly?

Answer 175

Haemorrhage CSF leak Visual deficit Recurrence Risk of injury to the cranial nerves running in the cavernous sinus Patients with acromegaly have swollen tissues making them prone to surgical bleeding.

Question 176

A 65-year-old man presents with sudden-onset headache, nausea, and visual distur- bance. He reports loss of libido for the last 4 years, but otherwise has no previous medical history. He is in quite severe distress due to the headache but is alert and orientated, with no focal neurological deficits. A CT scan is performed, what does it show?

Answer 176

There is a mixed density mass situated in the sella turcica with extension into the cavernous sinuses laterally and the chiasmatic cistern above. The diagnosis is pituitary apoplexy

Question 177

A 65-year-old man presents with sudden-onset headache, nausea, and visual distur- bance. He reports loss of libido for the last 4 years, but otherwise has no previous medical history. He is in quite severe distress due to the headache but is alert and orientated, with no focal neurological deficits. CT shows a mixed-densitry mass with an area of high intensity. He undergoes MRI

Answer 177

The optic chiasm (A) is displaced superioroly. There is a mixed high and low intensity signal within the tumour indicating recent haemorrhage

Question 178

Def: pituitary apoplexy

Answer 178

Clinical syndrome characterised by sudden onset headache, visual distrubance and reduced consciousness caused by haemorrhage or infarction of the pituitary gland. Compression of the optic chiasm above may lead to reduced visual acuity and fields. Involvement of the cavernous sinsus laterally may lead to ocular palsies

Question 179

Mx of pituitary apoplexy

Answer 179

Carefully document CN function including acuity, fields, eye movements. Serum electrolytes and PFT Secondary adrenal insufficiecny is the major soruce of mortality associated with pituitary apoplexy and haemodynamic function must be closely monitored. Corticosteroids are recommended if the presentation is severe. Some studies have shown that early surgery leads to a greater improvement in visual function but this has been contradicted by others which show no difference between conservatively and surgically managed cases. In general, patients with severe or deteriotating neuro-ophthalmic symptoms are surgical candidates whereas others are managed conservatively.

Question 180

A 12-year-old boy was referred by his paediatrician following 5 weeks of visual dete- rioration, particularly in the right eye. He kept bumping into things at home. He had experienced no headaches or vomiting. He had a visual acuity of 6/36 on the left and 6/12 on the right, and a bitemporal visual field loss to confrontation. The optic fundi were normal. Where is the lesion. What is the ddx What investigations need to be organised?

Answer 180

The bitemporal hemianopia means that the optic chiasm is affected. In theory a lesion compressing from above the chiasm would cause lower bitemporal hemianopia first. The most likely diagnosis in this age is craniopharyngioma, followed by pituitary tumour (rare in children). In adults a suprasellar aneurysm and meningioma are also possibilities. Imaging with CT or MRI would be appropriate as well as endocrine pituitary investigation. Formal visual field assessment should be undertaken

Question 181

A 12-year-old boy was referred by his paediatrician following 5 weeks of visual dete- rioration, particularly in the right eye. He kept bumping into things at home. He had experienced no headaches or vomiting. He had a visual acuity of 6/36 on the left and 6/12 on the right, and a bitemporal visual field loss to confrontation. The optic fundi were normal. He undergoes an MRI which is shown Comment on the findings

Answer 181

T1 axial slices are shown. There is a partly cystic (A), partly solid (B) loculated suprasellar lesion in close proximity to the optic nerves and chiasm although the exact position is not determined. The temporal horns of both lateral ventricles are visualised (C) and do not appear enlarged. This is consistent with the patients lack of headache. There are areas of hypointensity within the cyst which represent calcification (D) and are typical of craniopharyngiomas. The carotid arteries are partly encapsulated by the cyst and displaced laterally (E)

Question 182

What operative options exist for treating craniopharyngiomas

Answer 182

General approach is to debulk solid elements and drain the cystic areas taking care not to disturb the superior aspect which can be stuck to the hypothalamus. Macroscopic excision has fallen out of favour because of the risk of hypothalamic damage and the consequences in children. Postoperative radiotherapy is usually effective in preventing regrowth and long-term control is generally achivable. Hydrocephalus may need VP shunt and it is also possible to place a subcutaneous reservoir into a cyst cavity to allow repeated aspiration of recurrent cystic areas

Question 183

What are the possible consequences of hypothalamic damage in children?

Answer 183

Short stature, hyperphagia, morbid obesity, polydipsia, sleep disturbance, cognitive problems or in more severe cases persistent coma

Question 184

What are the possible operative approaches to craniopharyngioma

Answer 184

Laterally via pterional craniotomy and splitting the sylvian fissure Subfrontal or lateral subfrontal approach underneath the lobe Anterior interhemispheric or transcallosal approach if the tumour extends significantly into the third ventricle. Transphenoidal if there is sellar extension

Question 185

What specific care must be taken when removing tumours around the optic nerves or chiasm?

Answer 185

Care has to be taken not to completely strip the vascular supply to the optic apparatus from above or below the chiasm as this can result in blindness

Question 186

What are the immediate concerns in the post-operative period after resection of craniopharyngioma?

Answer 186

Neurological obs Visual fields need to be closely monitored in case of deterioration from postoperative haematoma or swelling of optic nerves DI is usually transient but can last up to weeks Steroid replacement with hydrocortisone may be required to avoid Addisonian crisis.

Question 187

A 47-year-old accountant is referred by his GP with 3 months of ascending numbness that began in his toes and spread upwards towards his umbilicus. He was initially investigated for diabetes and presumed peripheral neuropathy, but fasting blood glu- cose was normal (4.7 mmol/l). He has had stiffness in his legs which is troublesome first thing in the morning. His bladder and bowel function are normal and he reports no symptoms in his upper limbs. On examination he is thin but denies recent weight loss. Tone is increased in the legs and power is reduced (4/5). His knee and ankle jerks are very brisk and there is ankle clonus on both sides. One plantar reflex is upgoing and the other is equivocal. He has subjective numbness throughout his legs up to the level of the umbilicus but preserved pinprick sensation. He has no spinal tenderness. DDx

Answer 187

The patient clinically has a spastic paraparesis. Sparing of the upper limb suggets the pathology is below the cervical region. Two most common causes of spinal cord compression are bony mets and degenerative disease. Spinal mets usually cause pain. A progressively enlarging thoracic disc prolapse may cause symptoms in the absence of back pain. Benign tumour such as neurofibroma and meningioma are most common

Question 188

Explain how ascending numnbess arises from extrinsic cord compression?

Answer 188

Sensory and motor tracts are somatotopically arranged, In the lateral spinothalamic tract, sacral fibres are most lateral followed by lumbar, thoracic and cervical fibres. Therefore sensory disturbance due to extrinsic compression is typically percevied to begin in the lower limbs and progress upwards

Question 189

A 47-year-old accountant is referred by his GP with 3 months of ascending numbness that began in his toes and spread upwards towards his umbilicus. He was initially investigated for diabetes and presumed peripheral neuropathy, but fasting blood glu- cose was normal (4.7 mmol/l). He has had stiffness in his legs which is troublesome first thing in the morning. His bladder and bowel function are normal and he reports no symptoms in his upper limbs. On examination he is thin but denies recent weight loss. Tone is increased in the legs and power is reduced (4/5). His knee and ankle jerks are very brisk and there is ankle clonus on both sides. One plantar reflex is upgoing and the other is equivocal. He has subjective numbness throughout his legs up to the level of the umbilicus but preserved pinprick sensation. He has no spinal tenderness. The GP has arranged an MRI. What is the radiological diagnosis?

Answer 189

There is a large mass with mixed signal intensity (A) extending from the left side of the spinal canal in the midthoracic region, through the neural foramen B and into the chest cavity. C The body and posterior elements of the T7 vertebra are eroded by the mass.D The SC is displaced. These appearances are diagnostic of neurofibroma

Question 190

Slow growing, benign tumour that grows along a nerve root and through a neural foramen, typically expanding either side of the foramen, hence the term "dumb-bell"

Answer 190

Neurofibroma

Question 191

The patient has numbness up to the umbilicus which corresponds to the T10 dermatome. Why is there a discrepancy between the level of the lesion (in this case T7) and the sensory level (at T10)?

Answer 191

It is common for a clinical sensory level to be noted several segments below the anatomical level of the pathology as the spinal cord is shorter than the spinal canal and in lower thoracic spinal canal this equates to two to three spinal levels

Question 192

A 47-year-old accountant is referred by his GP with 3 months of ascending numbness that began in his toes and spread upwards towards his umbilicus. He was initially investigated for diabetes and presumed peripheral neuropathy, but fasting blood glu- cose was normal (4.7 mmol/l). He has had stiffness in his legs which is troublesome first thing in the morning. His bladder and bowel function are normal and he reports no symptoms in his upper limbs. On examination he is thin but denies recent weight loss. Tone is increased in the legs and power is reduced (4/5). His knee and ankle jerks are very brisk and there is ankle clonus on both sides. One plantar reflex is upgoing and the other is equivocal. He has subjective numbness throughout his legs up to the level of the umbilicus but preserved pinprick sensation. He has no spinal tenderness. MRI shows a neurofirboma How should the patient be counselled, if surgery is required how should it be done?

Answer 192

The patient has progressive spinal cord compression and will become paralysed if untreated. Surgery carries the rare risk of exacerbation of his neurological deficit. If he chooses to go ahead with surgery he should be given dexamethasone for a few days beforehand to reduce vasogenic odema. Surgery should aim to find the tissue plane deep to the tumour so that it can be removed completely from the SC. There is the risk that surgery will result in instability which may require supplementation with pedicle screws.

Question 193

Def: False localising sign

Answer 193

Neurological signs that are produced by lesions situated at locations other than those that would be expected for such a sign

Question 194

Pathophysiology of CN5 and 7 also acting as false localising signs

Answer 194

Presumed mechanism is by stretching of the cranial nerves due to downward displacement of the brainstem by a supratentorial mass lesion

Question 195

Kernohan-Woltman notch phenomenon

Answer 195

Occurs when a supratentorial lesion caues ipsilateral uncal herniation and ipsilateral pupillary dilatation in conjunction with an ipsilateral hemiparesis due to compression of the contralateral midbrain from displacement of the free edge of the tentorium. This falsely loalising as one would expect a supratentorial lesion to cause contralateral rather than ipsilateral paralysis

Question 196

Patient is usually elderly with a relatively minor flexion-extension injury to the cervical spine on a background of degenerative disease. Lower motor neurone weakness affecting the territory of the injury.

Answer 196

Central cord syndrome

Question 197

Pathophysiology of central cord syndrome

Answer 197

Acute compressive lesion to the cervical cord which on MRI shows expanded swollen cord and signal change often with evidence of fresh haemorrhage. Grey matter is typically more vulnerable than white so is affected more at the level of the injury

Question 198

A 12-year-old girl is admitted by the paediatricians. She was previously healthy but recently she was noticed to be running less and tending to walk more slowly or limp at times. She began to complain of neck pain and has started to use her hands more slowly and deliberately. Her GP was concerned about Guillain-Barré syndrome and sent her to the local emergency department. What features of the history are important and what is the differential diagnosis?

Answer 198

The combination of neck pain with motor deficits in the upper and lower limbs that have evolved over a period of time points to an insiduous pathological process affeting the cervical spinal cord. Neck pain is important as it is clasically associated with extradural pathology. Degenerative/metastastic disease or trauma are unlikely An abscess should be considered including TB depending on social circumstances. Primary extradural tumours are also a possibility. Intradural pathology can cause neck pain, particularly if there is stretching of the dura. Acute inflammatory processes such as GBS and transverse myelitis are a possibility

Question 199

A 12-year-old girl is admitted by the paediatricians. She was previously healthy but recently she was noticed to be running less and tending to walk more slowly or limp at times. She began to complain of neck pain and has started to use her hands more slowly and deliberately. Her GP was concerned about Guillain-Barré syndrome and sent her to the local emergency department. She undergoes an MRI. Describe the abnroamlity.

Answer 199

The sagittal T2 image shows a hyperintense circumscribed mass in the spinal cord from D3 to C5. The axial view dmeonstrates that lesion, appearing hyperintense with normal cord around it which appears as a rim of hypointensity. This is an intramedullary tumour expaninding the cord and there is little dilatation of the central canal of the cord below it

Question 200

What types of intramedullary tumour are there?

Answer 200

The most common types are gliomas. Grade III gliomas- anaplastic astrocytomas rather than glioblastoma are seen in the SC. The other relatively common tumour in children is ependymoma. Other rare CNS tumours can also occur as well as intramedullary metastases

Question 201

A 12-year-old girl is admitted by the paediatricians. She was previously healthy but recently she was noticed to be running less and tending to walk more slowly or limp at times. She began to complain of neck pain and has started to use her hands more slowly and deliberately. Her GP was concerned about Guillain-Barré syndrome and sent her to the local emergency department. MRI shows an intramedullary tumour What is the initial management for this patient?

Answer 201

Dexamethasone to reduce any associated oedema and full neuro-axis MRI for staging

Question 202

What are the aims of treatment for primary intramedullary tumours?

Answer 202

To biopsy- where there is diangostic uncertainty To debulk or excise the tumour where there is a realistic chance of doing so without unacceptable neurological deficit and with good prognosis. To manage assoicated problems e.g. syrinomyelia or drain tumour cysts

Question 203

What factors can help to decide which patients with intramedullary tumours are suitable for debulking surgery vs conservative management

Answer 203

If the tumour is well circumscribed, surgery can be potenitally curative and more aggressive strategies are justified. Similarly for malignant tumours where neurolgoical deficit is profound and progressive, aggressive surgery including cordectomy for patients with complete or near-complete paraplegia is well described with the occasional long term survivor. Patients with partial slowly progressive deficits presenting earlier in the coures of their illness merit a more cautious appraoch

Question 204

What technique can be used during debulking of intramedullary tumour to minimise intraoperative damage

Answer 204

Continuous electrophysiological monitoring, typically motor and somatosensory evoked potentials to alert surgeon to potential intraoperative damage

Question 205

Classification of spinal tumours

Answer 205

Extradural intradural-extramedullary Intradural-intramedullary

Question 206

Features of extradural spinal tumours

Answer 206

Typically cause pain and signs of progressive external cord compression. Posteriorly sited tumours will affect the dorsal columns first whereas laterally sited tumours will cause pain and affect the spinothalamic and corticospinal tracts. Sphincter disturbancs usually occur at a later stage

Question 207

Symtpoms of intradural-extramedullary tumours

Answer 207

Also present with pain at the spinal level but also a radicular pain, progressing to signs of cord compression

Question 208

Features of intradural intramedullary tumours

Answer 208

Do not cause pain as often and when it does occur is poorly localised. It has the propensity to affect the SC tracts from inside out. Sphincter disturbance may be an early feature and dissociated sensory deficits can occur.

Question 209

A 67-year-old woman presents to the emergency department with increasing back pain for 2 months, preventing her from sleeping and driving. Her legs have been feel- ing slightly numb for 4 days, and for the past 2 days she has been confined to the ground floor of her house. This morning she fell and was unable to get up. She under- went a lobectomy and radiotherapy for small-cell lung cancer last year. She last saw her oncologist 3 months ago and is under clinical surveillance What features of the back pain point to a more sinister cause?

Answer 209

There are anumber of red flag symptoms that can point to a sinister cause of back pain: Neurolgoical deficit Pain at rest or night Localised spinal tenderness History of malignancy History of trauma Weight loss Fever Systemic illness Steroid use

Question 210

A 67-year-old woman presents to the emergency department with increasing back pain for 2 months, preventing her from sleeping and driving. Her legs have been feel- ing slightly numb for 4 days, and for the past 2 days she has been confined to the ground floor of her house. This morning she fell and was unable to get up. She under- went a lobectomy and radiotherapy for small-cell lung cancer last year. She last saw her oncologist 3 months ago and is under clinical surveillance. The patient has reduced power at grade 3/5 in both lower limbs. Her lower limb reflexes are slightly brisk with plantars downgoing. There is a sensory level below the umbilicus. Anal tone is slightly reduced and a bladder scan shows a residual volume of 100mL. Her arms are normal. (a) How should the above neurological findings be interpreted?

Answer 210

The picture is mixed, she has features consistent with spinal cord compression but the reflexes and anal tone findigns are ambiguous and could be normal. Downgoing planatars are not typically associated with cord compression. If the neurological findings are ambiguous but the features consistent with cord compression, the patient should be treated as such

Question 211

A 67-year-old woman presents to the emergency department with increasing back pain for 2 months, preventing her from sleeping and driving. Her legs have been feel- ing slightly numb for 4 days, and for the past 2 days she has been confined to the ground floor of her house. This morning she fell and was unable to get up. She under- went a lobectomy and radiotherapy for small-cell lung cancer last year. She last saw her oncologist 3 months ago and is under clinical surveillance. Are there any investigations that may be of value in the emergency department?

Answer 211

If plain XR of the spine show bony destruction, an urgent MRI is required. Even if the XR are normal but there is clinical suspicion of cord ocmpression, an MRI will still be indicated. The XR are not indicated for back pain per se but beacuse of the red flag history. Other immediate investigations that are appropriate include CXR and blood including calcium

Question 212

A 67-year-old woman presents to the emergency department with increasing back pain for 2 months, preventing her from sleeping and driving. Her legs have been feel- ing slightly numb for 4 days, and for the past 2 days she has been confined to the ground floor of her house. This morning she fell and was unable to get up. She under- went a lobectomy and radiotherapy for small-cell lung cancer last year. She last saw her oncologist 3 months ago and is under clinical surveillance. She underoges XR, what do they show?

Answer 212

There is collapse and kyphotic angulation of a lower thoracic vertebrae. The AP XR show a paraspinal mass (dotted arrow) Ad the adjacent pedicle of T9 is not clearly seen. A classic finding in metastatic bony disese. An MRI shows tumour destroying the T9 body, extending along the T9 pedicles and causing severe spinal cord compression

Question 213

A 67-year-old woman presents to the emergency department with increasing back pain for 2 months, preventing her from sleeping and driving. Her legs have been feel- ing slightly numb for 4 days, and for the past 2 days she has been confined to the ground floor of her house. This morning she fell and was unable to get up. She under- went a lobectomy and radiotherapy for small-cell lung cancer last year. She last saw her oncologist 3 months ago and is under clinical surveillance. MRI shows destructive metastatic lesion in T9 vertebra causing severe spinal cord compression. What are the management options in this case?

Answer 213

The patient needs urgent treamtent to prevent a complete cord lesion. The management options are RTx or Sx. She should be started on dexamethasone.

Question 214

What are the risks of radiotherapy for malignant SC compression?

Answer 214

Avoids surgery with its attendent risks of haemorrhage, infection and worsening the deficit but takes several days to have an effect, with the potential for the deficit to worsen over this time period.

Question 215

What are the benefits and risks of surgery for malignant spinal cord compression

Answer 215

Haemorrhage, infection, worsening deficit. It has the benefit of providing immediate decompression. Patients may also require spinal stabilisation if decompressive surgery leaves the bony structure unstable

Question 216

What are the additional considerations before potential treatment of malignant spinal cord compression?

Answer 216

Stage cancer with CT. Discuss prognosis with oncologist.

Question 217

A 32-year-old man consults his GP with a history of headaches for 3 months, with no vomiting or nausea. They are intermittent and not associated with diurnal variation. His friends have asked him to speak more clearly on occasion, and he has felt that his speech is less fluent. However, he has no imbalance and he has been playing tennis. What is the DDx?

Answer 217

Headaches and speech coordination changes raise the possibility of a cerebellar mass with hydrocephalus. It would be unsual for a supratentorial mass to produce raised intracranial pressure with headaches and focal deficit in relation to mild speech distrubance which sounds like dysarthria rather than dysphasia. The slow and minimally progressive nature of the symptoms susggest a benign lesion

Question 218

A 32-year-old man consults his GP with a history of headaches for 3 months, with no vomiting or nausea. They are intermittent and not associated with diurnal variation. His friends have asked him to speak more clearly on occasion, and he has felt that his speech is less fluent. However, he has no imbalance and he has been playing tennis. A CT scan is done. Comment on the findings

Answer 218

There is an obvious enhancing mass which has an isodense capsule and hypodense contents to the right of the midline in the cerebellum. There is mild surrounding oedema. The third ventricle is oval rather than slit like and the temporal horns of the lateral ventricles are clearly visible, indicating hydrocephalus The most common enhancing tumour type in the cerebellum is metastatic disease, however the patient's age and duration make this unlikely

Question 219

What are the primary posterior fossa tumours in adults?

Answer 219

Haemangioblastoma Ependymoma Pilocytic astrocytomas

Question 220

A 32-year-old man consults his GP with a history of headaches for 3 months, with no vomiting or nausea. They are intermittent and not associated with diurnal variation. His friends have asked him to speak more clearly on occasion, and he has felt that his speech is less fluent. However, he has no imbalance and he has been playing tennis. MRI is performed. What does it show?

Answer 220

There is a well circumscribed avidly enhancing mass. The T2 image through the upper part of the mass (axial) shows a number of surrounding black flow voids indicating blood vessels. Enlarged surrounding vessels are a typical features of cerebellar haemangioblastoma and the diagnosis should be questioned if not seen around a lesion oft his size

Question 221

A 32-year-old man consults his GP with a history of headaches for 3 months, with no vomiting or nausea. They are intermittent and not associated with diurnal variation. His friends have asked him to speak more clearly on occasion, and he has felt that his speech is less fluent. However, he has no imbalance and he has been playing tennis. MRI shows likely haemangioblastoma What treatment should be offered?

Answer 221

There is no merit in conservative management for this patient as his symptoms will inevitably progress. Haemangioblastomas are benign (WHO I) so can be completely cured by surgical excision

Question 222

What are the features of cerebellar heamangioblastomas

Answer 222

WHO I Although usually sporadic they do occur as part of vHL. Commonly occur as a cystic mass with a non-enhancing wall and a solid enhancing nodule. Removal of the nodule aloneis the surgical treatment. Soli haemangiomas are less common and enhance throguhout. The tumour is highly vascular and an effort to remove it en block should be made

Question 223

What may be possible pre-operatively?

Answer 223

Catheter angiography to elucidate vascular supply +/- embolisation of feeding vessels