Emergency presentation of neurosurgical conditions Many neurosurgical conditions present in an acute fashion, and optimal outcome often depends on prompt recognition and referral by general practitioners and emergency room personnel. The approach to neurosurgical emergencies is discussed in this article.

P Semple, MB ChB, FCS (Neurosurg) (SA), MMed, PhD Division of , University of Cape Town Patrick Semple is Associate Professor, Division of Neurosurgery, University of Cape Town, Principal Specialist and Head of Neurocritical Care, Groote Schuur Hospital, Cape Town. His interests are neuroendoscopy, pituitary surgery and neurocritical care.

B J Malan, MB ChB Division of Neurosurgery, University of Cape Town Barend Malan is a final-year registrar in the Division of Neurosurgery, University of Cape Town. His interests are neurovascular surgery and neuro- oncology.

Correspondence to: B J Malan ([email protected])

Approach to a neurosurgical emergency management is instituted at the same time respirations (Cushing’s ) and is a sign begins with an appropriate history of as problems are identified. of impending tonsillar herniation. surrounding events followed by general examination and focused neurological Temperature examination.[1] This should be performed Elevated may Fever may indicate underlying CNS while applying general supportive care. After be a reactive phenomenon or infection. outlining a general approach to the patient reflect the underlying cause. presenting with a neurosurgical emergency, General examination the more common clinical scenarios will be Evidence of trauma – scalp bruising or discussed. Vital signs laceration, periorbital (raccoon eyes) or Airway and retro-auricular bruising (Battle sign) and History Ensure adequate airway and oxygen CSF leak from the nose or ear. A detailed history of presenting events and saturation above 95%. Cheyne Stokes general medical history should be obtained is the most common abnormal from the patient if possible, family members breathing pattern, characterised by periods Level of consciousness or emergency service personnel and should of hyperventilation alternating with periods Level of consciousness is assessed using the include the following: of apnoea. Consider intubation if Glasgow GCS (Table 1).[2] The GCS is made up of the Presenting events: coma scale (GCS) <8. sum of the best eye opening, best verbal • rapidity of symptom onset or neurological and best motor responses, with a total score deterioration Circulation ranging from 3 to 15 (normal). • seizures Elevated blood pressure may be a reactive • nausea or vomiting phenomenon or reflect the underlying Fundoscopy and pupillary response • recent or previous trauma cause. With increasing Fundoscopic examination for papilloedema • recent febrile illness. an acute rise in blood pressure may be is an essential clinical skill. Pupils are accompanied by and decreased checked for both size and response to Previous medical history: • and compliance with Table 1. Glasgow Coma Scale treatment • diabetes mellitus Best motor response 6 Obeys commands 5 Localises painful stimulus • epilepsy 4 Abnormal flexion • known history of previous/underlying 3 Decorticate posturing malignancy/pituitary tumour 2 Decerebrate posturing • HIV and antiretroviral treatment 1 No response • history of anticoagulant use. Best verbal response 5 Orientated 4 Confused speech Surgical history: 3 Words 2 Incomprehensible sounds • previous neurosurgery, e.g. ventriculo- 1 No response peritoneal shunt.[1] Best eye opening response 4 Opens eyes spontaneously 3 Opens eyes to speech Examination 2 Opens eyes to painful stimulus Clinical examination focuses on the 1 No response principles of ABCD, and supportive

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light. A unilateral dilated pupil suggests Focal motor deficits All patients presenting with unusually the presence of a structural lesion with Focal motor deficits include hemiparesis/ sudden or severe headaches should be uncal herniation. Pontine lesions result in plegia and monoparesis and should be investigated for SAH.[5] pinpoint pupils, while midbrain lesions are documented during assessment of the motor associated with midposition fixed pupils. component of the Glasgow coma score. Examination Findings include decreased level of Eye movements Common presentations consciousness, neck stiffness and focal Evaluate spontaneous eye movements and • Acute loss of consciousness. Loss of neurological deficits. Focal deficits may the ocular – oculocephalic (doll’s consciousness is a presentation common be due to mass effect from the aneurysm eye) and oculovestibular. Hemispheric to a variety of medical conditions and is (e.g. third nerve palsy in posterior lesions involving the frontal eye fields result best assessed using the GCS, as described communicating aneurysms) or in deviation of the eyes away from the side of above. The causes of coma are divided intracerebral haematomas. the associated hemiplegia. lesions into 2 major groups and common causes involving the reticular formation in the of each are shown in Table 3. Metabolic Investigation result in conjugate deviation of the eyes causes of acute loss of consciousness Imaging toward the side of the associated hemiplegia. are more likely to produce symmetrical Uncontrasted CT scan of the is the Nystagmus may indicate cerebellar lesions.[1] neurological deficits, compared with focal investigation of choice in suspected SAH deficits in structural causes.[3] and may reveal blood in the cisterns at Cranial nerve examination • Sudden-onset severe headache. the base of the brain, intraventricular Third and sixth nerve palsies are common • Subarachnoid haemorrhage (SAH) or intraparenchymal blood (Fig. 1). indicators of raised intracranial pressure. occurs when blood enters the Hydrocephalus may also be present. If Other cranial nerve deficits may also be subarachnoid space, in the majority of uncontrasted CT of the brain is negative, present depending on the location of cases due to rupture of an intracranial a lumbar puncture should be performed to pathology.[1] saccular aneurysm. It occurs most establish the presence of xanthochromia, commonly in the 5th - 6th decades.[4] unless a contraindication to lumbar puncture Meningism is present.[6] Remember that neck stiffness has a number History of causes and does not always mean Sudden-onset severe headache, described Laboratory meningitis – performing a lumbar puncture as ‘the worst headache of my life’, occurs in Full blood count (FBC), clotting profile, in a patient with tonsillar herniation will 80% of patients. Headaches are commonly urea, electrolytes and creatinine (UEC). have disastrous consequences (Table 2). retro-orbital, radiate to the neck and often Initial management occur during periods of intense activity or • Monitor arterial and straining. Vomiting is also common.[4] Twenty blood pressure Table 2. Causes of neck stiffness per cent of patients have a history of • Supplemental oxygen preceding symptoms, including headache, • Intravenous access and fluid Meningitis nausea, vomiting and dizziness, thought administration, correct hyponatraemia Subarachnoid haemorrhage to arise from small haemorrhages from • Stool softeners to prevent straining Tonsillar herniation (raised intracranial the aneurysm or acute expansion of the • Anticonvulsants, especially if seizures pressure) aneurysm sac. Within seconds or minutes occurred during the initial event.[6] Cervical spine trauma following headache, patients may lose consciousness, develop seizures or die.

Table 3. Causes of acute loss of consciousness Toxic/metabolic Structural Electrolyte imbalance Vascular Hypo/hypernatraemia Bilateral cortical/subcortical infarcts Endocrine Bilateral carotid artery stenosis Hypoglycaemia Bilateral di-encephalic infarcts Diabetic ketoacidosis Infectious Addison crisis Abscess Toxins Empyema Ethanol Trauma Drug overdose Neoplastic Organ failure Primary Uraemia Secondary Hypoxaemia Increased intracranial pressure Fig. 1. Uncontrasted CT brain revealing Liver failure Herniation from mass effect Epilepsy Acute lateral shift of the brain extensive subarachnoid blood in the basal cisterns (arrow).

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Pituitary apoplexy structures (oral cavity, sinuses, middle ear), (Fig. 2) or enhancing subdural collection Pituitary apoplexy results from haemor-rhage either directly or by retrograde venous in the presence of subdural empyema. or of a pituitary adenoma, resulting thrombosis, or haematogenous spread Opacification or air-fluid levels in the sinuses in acute tumour expansion with compression in patients with valvular heart disease may indicate sinusitis. Chest X-ray can reveal of surrounding structures in the sellar region, (infective endocarditis), cyanotic congenital evidence of pneumonia or bronchiectasis.[10] notably the optic nerves and hypothalamus.[7] heart disease and chronic suppurative lung disease (bronchiectasis).[9] Laboratory History FBC, UEC, septic markers (CRP/ESR) The patient may be known to have a pituitary History and blood culture.[10] Lumbar puncture is tumour, but in the majority pituitary apoplexy Patients may have a history of recent febrile contraindicated in the presence of decreased is the first presentation. Symptoms include illness, including dental infections, otitis level of consciousness or focal neurological acute-onset headache, acute deterioration media, mastoiditis or sinusitis. Symptoms deficit. in visual acuity or visual fields. Vomiting are usually present for 1 - 2 weeks before often accompanies pituitary apoplexy. There acute deterioration. Common symptoms Initial management may be a preceding history of symptoms are headache (often progressive and severe, • ABCs suggestive of hypopituitarism (tiredness, localised to the side of the abscess), mental • Supplemental oxygen, intubation as weight gain, decreased libido, menstrual status changes, high- or low-grade fever, required (GCS<8) irregularities). Features of hormone excess seizures, nausea and vomiting.[10] • IV access and broad-spectrum antibiotics may also be present, e.g. Cushing’s disease, acromegaly or hyperthyroidism.[8] Examination • Anti-seizure prophylaxis.[10] Findings on physical examination include Examination fever, neck stiffness and signs of raised Focal neurological deficits/ Hypotension and hypothermia may indicate intracranial pressure due to mass effect seizures acute adrenal insufficiency secondary from the lesion or associated cerebral Acute-onset intracerebral to low cortisol. Common findings are oedema. Note that some patients with brain haematoma (ICH) ophthalmoplegia, decreased visual acuity abscess do not have a fever. Focal deficits are History and visual field deficits – most commonly common and determined by the size and Spontaneous ICH usually occurs in patients bitemporal hemianopia.[8] location of the pus collection. Cerebellar older than 55 years and is slightly more lesions result in cerebellar signs, while common in men. Pre-existing hypertension supratentorial lesions lead to hemiparesis, is commonly present and concurrent use of Investigation mental status changes and speech difficulties. oral anticoagulants should be determined. Imaging A thorough ENT, oral, cardiovascular and In younger patients, structural lesions occur A CT scan of the brain with and without chest examination should be performed to more frequently and a history of illicit drug use contrast may reveal a sellar or suprasellar identify a possible source of infection.[9] (e.g. cocaine) should be excluded. Common mass with evidence of haemorrhage, with or symptoms include headache, vomiting, acute- without subarachnoid haemorrhage. MRI Investigation onset focal weakness and seizures.[11] is better for diagnosis and should be done Imaging in all cases of suspected pituitary apoplexy CT brain, with and without contrast, reveals Examination following an initial CT scan.[8] a ring-enhancing lesion (location often Elevated blood pressure may be an depends on the underlying cause) with indication of the underlying cause in Laboratory surrounding oedema in the case of an abscess patients with chronic hypertension or a FBC, UEC. Baseline endocrine bloods (T4, reactive phenomenon secondary to raised TSH, FSH, LH, prolactin, GH and cortisol).[7] intracranial pressure. Excessive bruising or bleeding suggest anticoagulant toxicity. Initial management Focal neurological deficits are dependent • ABC on haematoma location and include • IV access and immediate administration hemiparesis, gaze palsy or other cranial nerve of hydrocortisone 100 mg palsy.[11] • Urinary catheter and monitoring of urine output to monitor for diabetes Investigation insipidus.[8] Imaging Uncontrasted CT scan of the brain shows Meningism with fever hyperdense areas of acute haemorrhage. Intracranial infections Hypertensive bleeds often occur in Intracranial infections include brain abscess deep locations (basal ganglia, thalamus, and subdural empyema. These conditions Fig. 2. Contrasted CT brain revealing ring cerebellum). Younger patients with more usually occur in the first 4 decades of life enhancing cerebral abscess in the right superficial haematomas or in atypical and result from spread from contiguous parieto-occipital area (arrow). locations are more likely to have an

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underlying vascular lesion, and further without hydrocephalus. Further imaging is neurological deficits and signs of herniation, investigation is warranted, e.g. CT directed at identifying a possible underlying as discussed elsewhere in this issue. angiography.[11] cause (CXR, ultrasound, etc.).[12] Imaging Laboratory Initial management • Extradural haematoma – convex, FBC, UEC, clotting profile. • General supportive measures hyperdense extra-axial lesion with or • Correction of coagulopathies should be without midline shift (Fig. 4a). An Gradual progression performed immediately.[11] overlying fracture is often identified. Progressive headache or neurological • Anti-seizure prophylaxis • Subdural haematoma – crescent- deficits are suggestive of an expanding • Steroids can be administered in the shaped hyperdense lesion following the mass lesion, most commonly intracranial presence of oedema surrounding a contour of the cerebral hemisphere (Fig. 4b). , which can be either primary or suspected tumour.[12] Parenchymal contusions are also common. secondary. History of trauma History Extradural haematoma A history of previous or current underlying Extradural haematomas are collections cancer may be present, as well as systemic of blood outside the dura and are most symptoms such as weight loss. Gradual commonly caused by skull fractures with an progression of pre-existing weakness, associated vascular tear, following a direct progressive longstanding headache or blow to the head. Clinically there may or seizures are suggestive of a progressively may not be a period of loss of consciousness, enlarging lesion or increasing cerebral often severe headache, vomiting and a oedema around the tumour. Acute history of seizures. About 20% of patients deterioration may also occur due to haemor- have the classic ‘lucid interval’ between rhage into the tumour. the initial trauma and neurological Fig. 4a. Acute extradural haematoma: deterioration.[13] biconvex hyperdense extra-axial lesion with Examination mass effect on the underlying hemisphere. Altered level of consciousness, signs of raised Subdural haematoma intracranial pressure and focal neurological Subdural haematomas are the most common deficits are dependent on the location of the intracranial haematomas following trauma lesion. Systemic examination should also be and usually follow a high-speed impact performed to identify a possible underlying to the skull, which causes brain tissue to primary tumour.[12] decelerate relative to fixed dural structures, leading to tearing of blood vessels. Brain Investigation is often more severe and the patient is Imaging more likely to present with a decreased level CT brain, with and without contrast, may of consciousness.[13] reveal intrinsic or extrinsic mass lesions (Fig. 3) with surrounding oedema, with or Chronic subdural haematoma In chronic subdural haematomas there is Fig. 4b. Subdural haematoma: hyperdense usually a history of minor head trauma extra-axial lesion following the contour of the some weeks prior to presentation. hemisphere. Patients complain of severe, progressive headache, seizures and decreased level of consciousness.[13]

History • Mechanism of injury – blunt or penetrating assault, MVA • Loss of consciousness or amnesia • Seizures.

Examination There is usually external evidence of trauma Fig. 3. Contrasted CT brain showing a – lacerations, bruising, palpable fractures and homogenously enhancing extra-axial lesion CSF leak from the nose or ears suggestive of a Fig. 4c. Chronic subdural haematoma. in the interhemispheric fissure (parasaggital base of skull fracture. Clinical findings include Hypodense extra-axial lesion with mass meningioma). those of raised intracranial pressure, focal effect.

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• Chronic subdural haematoma – iso- to hypodense collection over a hemisphere, Table 4. Causes of spinal cord compression often with a significant midline shift Degenerative Cervical/thoracic stenosis (Fig. 4c).[13] Acute disc prolapse Trauma Fractures Initial management Fracture-dislocations • Resuscitation according to ATLS Infectious Spinal epidural abscess/empyema principles, ensuring adequate blood Vascular Spinal epidural haematoma pressure and oxygenation to prevent secondary injury Neoplastic Extradural: vertebral tumours (primary or metastases) Intradural: extramedullary (e.g. meningioma or neurofibroma) • Anti-seizure prophylaxis • Urgent neurosurgical referral for surgical evacuation. swelling. Shunt series X-ray studies (SXR, haematoma and spinal epidural abscess,[16] lateral neck, AP chest, AP abdomen) may resulting in dysfunction of multiple sacral Previous neurosurgery reveal disconnection or fracture along the nerve roots within the lumbar spinal canal. Ventriculoperitoneal shunt shunt tract.[14] dysfunction Spontaneous ICH usually History Laboratory Progressive headache, vomiting or FBC, UEC and baseline septic markers for occurs in patients older drowsiness are common complaints. possible shunt infection. than 55 and is slightly more Patients who have experienced shunt common in men. dysfunction before can often recognise Initial management a specific pattern when their shunt is not Provide initial supportive care and urgent functioning, and very careful attention neurosurgical consult. Clinical presentation should be paid to this history. Endoscopic CES most commonly presents in an acute third ventriculostomy (ETV) has Spinal compression fashion. Clinical findings include lower recently been accepted as a treatment for Various lesions may compress either back pain and sciatica (often bilateral), urine hydrocephalus. These patients do not have the spinal cord or the roots of the cauda incontinence, decreased anal sphincter a shunt but the ETV can also block, leading equina. This is one of the most commonly tone, saddle anaesthesia, motor weakness to rapid clinical deterioration. missed neurosurgical emergencies and an involving more than one motor nerve root increasingly important cause of litigation. and bilaterally decreased or absent Achilles Various lesions may tendon reflexes.[16] compress either the spinal Spinal cord compression The most common causes of spinal cord Investigation cord or the roots of the compression are listed in Table 4.[15] All patients require an urgent MRI scan and cauda equina. This is one of prompt referral to a neurosurgeon. the most commonly missed Clinical presentation • Paraplegia/paraparesis Conclusion neurosurgical emergencies • Quadriplegia/quadriparesis Neurosurgical conditions commonly and an increasingly • Urinary retention present as emergencies. and a high index important cause of litigation. • Impaired sensation below the level of of suspicion should always be maintained compression when assessing these patients in order to • Hyperactive reflexes ensure rapid diagnosis and prompt referral Examination • Plantar reflexes may be upgoing to a neurosurgeon. Assess level of consciousness, presence • Clinical findings may develop over hours of papilloedema and other signs of raised or days.[15] References available at www.cmej.org.za intracranial pressure. Document any signs of inflammation or swelling around the scalp Investigation In a nutshell or abdominal incisions suggestive of shunt • Plain film X-rays • Neurosurgical conditions frequently infection or obstruction. The presence of • All patients need to be referred for urgent present as emergencies. neck stiffness is a sign of possible meningitis MRI to determine the level and cause of • A good history, general examination and or shunt infection. Palpate the shunt tract compression.[15] focused neurological examination pro- vide vital diagnostic clues. for possible disconnection or fractures.[14] • Urgent imaging with CT or MRI is in- Cauda equina compression dicated in all neurosurgical emergencies. Investigation (CES) • Basic supportive care should be provided Imaging Aetiology to stabilise the patient. CT reveals ventriculomegaly with or without Possible causes include a massively herniated • Urgent neurosurgical consult is required in all cases. periventricular lucency or generalised brain lumbar disc, tumour, trauma, spinal epidural

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