A young man with depressed conscious state and seizures

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Problem 39 A young man with depressed conscious state and seizures

Michelle Kiley

A young man is brought into the emergency department early one morning. He was found ‘unconscious’ in the street and an ambulance called. There were no witnesses. Immediate assessment shows that his airway is intact and patent, he has normal colour, respiratory rate and pulse oximetry. There is no circulatory compromise. There are no obvious injuries.
He is stuporose and responds with sluggish but purposeful limb movements to painful stimuli. He groans, but does not make any comprehensible sounds. His Glasgow coma score is 6 (E1, V2, M3). He has a temperature of 37°C and cardiorespiratory examination is normal. The pupils are mid-position, equal and reactive and the optic fundi are normal. The plantars are downgoing. He has no neck stiffness and Kernig’s sign is negative. No focal neurological abnormality can be elicited. The rest of the physical examination is normal.

Q.1

What test should immediately be performed on arrival in the emergency department and what else should be sought on examination?

The test does not help elucidate the cause of the patient’s collapse. The man is reasonably well kempt but there is a faint smell of alcohol on his breath. On closer inspection there is a superficial laceration over the occiput and bruising of the right lateral aspect of the tongue. The patient’s underpants are found to be damp with urine.

Before any further assessment is performed the patient suddenly lets out a grunt, all four limbs extend and his spine arches. He stops breathing and rapidly becomes cyanosed. After 30 seconds, violent rhythmic contractions of the limbs begin and persist for at least 5 minutes

Q.2

What is the diagnosis, and what should be done for the patient?

The seizure is aborted after 10 mg intravenous diazepam. At this stage a brother, who had been contacted by the nursing staff, arrives and provides further information. The patient is 24, has had epilepsy since his mid teens, and has been on regular anticonvulsant medication, although the brother is unsure as to the medication’s name.

Q.3

What are the causes of this condition? What are the likely causes in this patient?

The brother states that the patient has seizures every few months and his local doctor has told him that due to his poorly controlled epilepsy he is currently ineligible to drive and that it is the patient’s responsibility to inform the local licensing authorities. Over the last few days the patient has had several late nights, despite having an upper respiratory tract infection, including the previous night in which he had been out with his brother drinking until the early hours of the morning. He seemed reasonably well when he left the bar, saying that he would walk home.

The patient slowly regains consciousness over the next hour. His last recollection was leaving the bar with his brother the night before. He confirms his brother’s account and admits he has not taken his anticonvulsant medication (carbamazepine) for 2 days. In addition to occasional generalized tonic seizures the patient reports isolated myoclonic jerks of the arms and trunk, which usually occur first thing in the morning or later at night, particularly when tired.

Q.4

How will you manage this patient in the short term?

An electroencephalogram (EEG) is performed (Figure 39.1), as one had not been done since diagnosis many years ago. This shows 4–5 Hz paroxysmal generalized polyspike and wave discharges consistent with the diagnosis of primary generalized epilepsy.

image

Figure 39.1

Q.5

How are you going to manage his medications?

You counsel the patient about the medications you feel most appropriate for his needs, and spend some time talking to him about his illness.

Q.6

What other advice will you offer him?

Q.7

The patient asks you when he is likely to be able to stop taking anticonvulsant medication. What is your reply?

Answers

A.1 Hypoglycaemia should be promptly excluded by blood glucose estimation.

Further examination should be undertaken for evidence of:

• Drug abuse (needle tracks) and/or drugs or drug paraphernalia on his person.
• Seizure (tongue biting or incontinence).
• Medication usage (medical alert bracelet, personal details in pockets, any information about medications such as sedatives, antidepressants or illicit drugs).
• Signs of chronic liver disease.
• Head injury.
• Assess if the patient is kempt or unkempt and asssess the breath for the smell of alcohol.
• Look for a form of identification or any other personal items that will enable you to contact relatives or friends. Most importantly, these people may be able to give a history.

A.2 The initial clinical picture is consistent with a post-ictal state. The subsequent event was a generalized tonic–clonic seizure. He has not regained consciousness between seizures and the second seizure persisted for at least 5 minutes. He fits the operational definition of convulsive status epilepticus. This is defined as continuous or recurrent generalized seizure activity of greater than 5–10 minutes. There is some evidence that a convulsive seizure of at least 5 minutes duration is unlikely to self-abort. Repeated and or prolonged tonic–clonic seizures are life-threatening and require prompt treatment to avoid refractory status epilepticus which carries a 30% mortality.

Management consists of:

• Clear and maintain the airway and call for support.
• Monitor cardiac and respiratory status.
• Give high-flow oxygen by face mask and connect a pulse oximeter.
• Establish intravenous access × 2 with large-bore cannulae.
• Give an intravenous benzodiazepine – ideally lorazepam 4 mg (0.7 mg/kg), but if not available then diazepam (5–10 mg at a rate not exceeding 5 mg/min) OR midazolam (1–5 mg at a rate not exceeding 2 mg/min).

If immediate IV access can not be gained then give midazolam 5 mg intramuscularly and consider insertion of a central line.

• Check for respiratory depression.
• Collect blood for glucose, electrolytes, complete blood picture, toxicology screen and if applicable anti-epileptic drug levels. Also obtain arterial blood gas and pH estimation.
• If lorazapam* is not the benzodiazepine given then load with intravenous phenytoin via separate IV access (15–20 mg/kg given at a rate of 50 mg/min, unless elderly or known history of cardiac or hepatic impairment in which case the rate should be reduced to 25 mg/min).
• If seizures persist despite the above, prepare to intubate and ventilate the patient.

A general anaesthetic agent (thiopental/propofol/midazolam) can then be used to control refractory seizures. There is no evidence that any one of these agents is superior to any other – they all have both advantages and disadvantages.

If facilities are not available to intubate/ventilate, but seizures persist despite loading with phenytoin, consider IV phenobarbital (10 mg/kg given at a rate of no greater than 50 mg/min).

Once a patient with status epilepticus is intubated and ventilated it is imperative that EEG/consultant neurologist advice is sought to assist in guiding ongoing treatment.

Only when control of the seizure is obtained can the underlying cause be sought and treated.

A.3 Common causes of status epilepticus in adults include:

• Epilepsy exacerbated by non-compliance with medications, intercurrent illness, other lifestyle factors such as alcohol abuse, use of other recreational drugs or sleep deprivation. The lifetime incidence of convulsive status epilepticus in adults with epilepsy is 5% and the most common cause is low anti-epileptic drug levels.
• Head trauma.
• Space-occupying lesion, e.g. brain tumour or metastases.
• Stroke including both cerebral infarction and haemorrhage.
• Central nervous system infection.
• Metabolic disorders.
• Medication effects including drug withdrawal.

Any of these causes are possible in this patient. Although there are no signs of meningism or focal neurological signs, meningitis, encephalitis or a subarachnoid haemorrhage are still possibilities. Withdrawal from alcohol or sedative medications should also be considered. Further history would be the most helpful in determining the cause and will determine if further investigations, such as a CT scan of the brain, are necessary. If the patient is not a known epileptic then this investigation should definitely be undertaken. If recovery is slow or there are focal neurological signs in the setting of known epilepsy then imaging should be strongly considered.

A.4 The additional information from the patient’s brother and the patient’s rapid recovery diminishes the need for further investigations. As above, in the absence of a history of epilepsy and/or focal signs/slow recovery, the next investigation would be a CT scan. A negative CT would be followed by a lumbar puncture.

In this instance the underlying cause of the status epilepticus was longstanding primary generalized epilepsy exacerbated by:

• Non-compliance with medication.
• Intercurrent viral illness.
• Alcohol abuse.
• Sleep deprivation.

An EEG may be useful, not to determine whether a seizure has occurred – this will be apparent from the history – but to provide information about the type of epilepsy, i.e. partial/focal or generalized, and thus guide further investigation (particularly if a focal discharge is found) and the choice of anticonvulsant (as in this case).

A.5 This patient has generalized tonic–clonic and myoclonic seizures and an EEG pattern of idiopathic generalized epilepsy (IGE) with 4–5 Hz polyspike and wave discharges, rather than focal epilepsy with secondary generalization. The history and EEG findings are consistent with juvenile myoclonic epilepsy (JME), the most common form of IGE. This diagnosis influences the optimal choice of anticonvulsant. Single-drug therapy with sodium valproate would be more appropriate than carbamazepine in this instance (carbamazepine is not advised for JME as it may exacerbate myoclonus). The effectiveness of drug therapy, once the patient is compliant, is best assessed by the clinical reduction in seizure frequency. Serum blood levels can be used to monitor compliance if that is in doubt, but serum levels for sodium valproate are very variable and are not advised routinely. Serum levels for carbamazepine are of greater consistency and thus may help guide dosing. If seizures persist, alternative and/or adjunctive therapy should be considered. The more highly effective agents, in addition to sodium valproate, for IGE include lamotrigine, levetiracetam and topiramate. Carbamazepine is the drug of first choice for partial/focal seizure disorders and serum carbamazepine levels are consistent and helpful in determining therapeutic dosage. At this stage referral to a neurologist is advisable.

A.6 The patient has had a life-threatening exacerbation of his epilepsy. This is a good time to discuss the importance of better illness management with him. You should emphasize that the combination of non-compliance with medications, sleep deprivation and alcohol, or in fact any of the above in isolation, will significantly lower seizure threshold. Therefore, he must:

• Be compliant with medication.
• Use alcohol in moderation, e.g. 1–2 drinks daily.
• Avoid alcohol excess, particularly binges.
• Avoid sleep deprivation and fatigue.
• Give up driving until his epilepsy is controlled. The length of time he is not able to drive will be determined by local/national guidelines and the advice of a consultant neurologist.
• Take precautions at work and with recreation (particular care with fire/heights/water).
• Wear a medical alert bracelet and carry information concerning his diagnosis and medication on his person at all times.

A.7 Up to 90% of patients with idiopathic generalized epilepsy will achieve freedom from seizures with anticonvulsant therapy. Lifestyle factors (especially avoidance of sleep deprivation) are also extremely important in maintaining seizure control. The chance of seizure freedom with partial seizure disorders is not quite as good, about 65% with monotherapy and up to 75–80% with polytherapy. In considering when to stop anticonvulsant medication, the chance of continued remission after anticonvulsant withdrawal is greatest in those who have been seizure free for more than 2 years on anticonvulsants, have a single seizure type, a normal neurological examination, no underlying structural abnormality of the brain and a normal EEG (of greater relevance in IGE, persisting focal changes in partial seizure disorders are not helpful in guiding the withdrawal of medication). Conversely, those with a structural brain abnormality, an abnormal EEG, more than one seizure type and poor seizure control requiring more than one anticonvulsant medication, have a high risk of seizure recurrence if medication is stopped. The risk of having further seizures and the impact of seizures on daily activities (for example driving) should be taken into account before recommending withdrawal of anticonvulsants.

* Lorazepam has a much longer half-life than diazepam or midazolam and thus phenytoin is only required if lorazepam fails to stop the seizure. Due to the higher risk of recurrent seizure activity with shorter-acting benzodiazepines, phenytoin should be given prophylatically. One of the leading causes of refractory status epilepticus is early under-management.

Revision Points

Management of the First Seizure

Emergency Care

Check ABC: i.e. airway, breathing and circulation. Stop seizure of greater than 5 minutes’ duration with intravenous benzodiazepine, e.g. diazepam.

Aetiology

The likely cause depends on the patient’s age. Idiopathic generalized epilepsy is unlikely to present for the first time in the elderly but is a common cause in young adults, as is drug and alcohol abuse. Space-occupying lesions, stroke and metabolic disturbance are all more common causes in the elderly, but all causes can occur at any age.

History

From the patient retrospectively, plus an eyewitness account is invaluable:

• How did the seizure start?
• Any aura?
• Any focal or lateralizing features at seizure onset?
• Seizure duration?
• Time taken to recover?
• Past history of head trauma, neurological illness, other diseases (e.g. cardiovascular or cancer), drug and alcohol consumption.
• Family history.
• Associated symptoms, e.g. recent headaches, fever.

Investigations

Always search for an underlying cause:

• Neurological disorder:

– meningitis/encephalitis
– space-occupying lesion (tumour)
– stroke/subarachnoid haemorrhage

• Metabolic disturbance:

– hypoglycaemia
– hyponatraemia
– hypercalcaemia.

• Drug and alcohol abuse.
• Head injury.

Investigations depend on suspected underlying cause but all patients should have complete blood picture, glucose, electrolytes and CT scan. Further investigations may include LP, MRI, EEG, toxicology.

Management

Depends on underlying cause. All patients should be given strict advice about driving according to national regulations.

Issues to Consider

• What advice would you give about driving to a young man presenting with a witnessed first fit?
• What non-pharmacological treatments are available for the treatment of debilitating epilepsy?
• What are the detrimental effects of long-term phenytoin therapy?

Further Information

, http://www.epilepsysociety.org.uk/Forprofessionals. A superb resource for professionals interested in epilepsy from the National Society for Epilepsy. Hundreds of articles, references, literature reviews

, www.epilepsy.org.uk. A good website from the British Epilepsy Association with lots of information for patients, carers, professionals. Lots of links

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