Neuro

Frage	Antworten
What is the definition of a seizure?	A clinical event in which there is a sudden disturbance of neurological function caused by an abnormal or excessive neuronal discharge. Seizures may be epileptic or non-epileptic.
List some causes of 'funny turns' in children.	Breath-holding attacks, reflex anoxic seizures, syncope, migraine, benign paroxysmal vertigo, others (e.g. prolonged QT, tics etc).
What is a breath-holding attack?	Occur in some toddlers when they are upset. The child cries, holds his breath and goes blue. Sometimes children will briefly lose consciousness but rapidly recover fully. Drug therapy is unhelpful. Attacks resolve spontaneously, but behaviour modification therapy, with distraction, may help.
What is a reflex anoxic seizure?	Occurs in infants or toddlers. Many have a 1st degree relative with a history of faints. Commonest triggers are pain or discomfort, particularly from minor head trauma, cold food (ice cream etc), fright or fever. Some children with febrile seizures may have experienced this phenomenon. After the triggering event, the child becomes very pale and falls to the floor. The hypoxia may induce a generalised tonic-clonic seizure. The episodes are due to cardiac asystole from vagal inhibition. The seizure is brief and the child rapidly recovers. Ocular compression under controlled conditions often leads to asystole and paroxysmal slow-wave discharge on the the EEG.
What is benign paroxysmal vertigo?	This is characterised by recurrent episodes of vertigo, lasting from one to several minutes, associated with nystagmus, unsteadiness or even falling. It is a primary headache disorder of childhood occasionally due to a viral labyrinthitis.
What is epilepsy and how is it now classified?	A chronic neurological disorder characterised by recurrent unprovoked seizures, consisting of transient signs and/or symptoms associated with abnormal, excessive or synchronous neuronal activity in the brain. Most is idiopathic. Seizures are broadly classified as either: GENERALISED - discharge arises from both hemispheres. May be absence, myoclonic, tonic, tonic-clonic and atonic. FOCAL - where seizures arise from one or part of one hemisphere.
How would a frontal seizure manifest?	Involve the motor or pre-motor cortex. May lead to clonic movements, which may travel proximally (Jacksonian march). Asymmetrical tonic seizures can be seen.
How would a temporal seizure manifest?	Most common of all the epilepsies. May result in strange warning feelings or aura with smell and taste abnormalities and distortions of sound and shape. Lip-smacking, plucking at one's clothing and walking in a non-purposeful manner (automatisms) may be seen, following spread to the pre-motor cortex. Deja-vu and jamais-vu are described. Consciousness can be impaired and the length of event is longer than a typical absence.
How is epilepsy diagnosed?	Primarily from a a detailed history from the child and eyewitnesses, substantiated by video if available. Particular attention is focused on any triggers and if the child has any impairments, as there may be educational, psychological or social problems. Clinical examination should include checking for skin markers for a neurocutaneous syndrome or neurological abnormalities. Although epilepsy is usually idopathic, it may be the presentation or a complication of an underlying neurological disorder.
What is an EEG used for in the investigation of epilepsy?	EEG is indicated whenever epilepsy is suspected. It is interpreted to identify a background that is abnormal for the child's age; asymmetry or slowing that may suggest underlying structural abnormalities; or evidence of any neuronal hyperexcitability such as sharp waves or spike-wave complexes. Many children with epilepsy have a normal inital EEG; and many children who will never have epilepsy will have EEG abnormalities. Unless a seizure is actually captured, an EEG does no more than add supportive evidence for or against the diagnosis. If the standard EEG is normal, a sleep or sleep-deprived record can be helpful. Additional techniques are 24h ambulatory EEG, or, ideally, video-telemetry.
What is the role of imaging in the investigation of epilepsy?	MRI and CT are not required routinely for childhood generalised epilepsies. They are indicated if there are neurological signs between seizures, of if seizures are focal, in order to identify a tumour, vascular lesion, or area of sclerosis which could be treatable. PET and SPECT scans are functional scans which may be able to detect areas of abnormal metabolism suggestive of seizure foci. Both can be used between seizures to detect areas of hypometabolism in epileptogenic lesions.
What are the treatment options for epilepsy?	A specialist epilepsy nurse may assist families by providing education and continuing advice on lifestyle issues. The decision whether to treat or not is related to the level of inconvenience seizures are bringing into the young person's life. It is common practice not to institute treatment after a single unprovoked seizure. Not all seizures require AED therapy. This decision should be based on the seizure type, frequency and the social and educational consequences of seizures against the possible SEs of the drugs. Ketogenic diet helps some children. Vagal nerve stimulation may be useful. If well-localised, a temporal lobotomy (or hemispherectomy/hemispherotomy or other focal resections) may be performed.
What are the principles of anti-epileptic drug therapy (AED)?	Choose appropriate drug for seizure - i.e. carbamazepine can make absence and myoclonic seizures worse. Monotherapy at the minimum dosage is the desired goal, though in practice more than one drug may be required. All AEDs have potential unwanted effects - discuss with child and parent. Drug levels not measured routinely, but may be useful to check for adherence to advice or with some drugs with erratic pharmacokinetics, e.g. phenytoin. Children with prolonged seizures are given rescue therapy to keep with them. This is usually a benzodiazepine, e.g. rectal diazepam or buccal midazolam. AED therapy can usually be discontinued after 2 years free of seizures.
What is the first and second line drug for generalised seizures?	Valproate 1st line. Lamotrigine 2nd line.
What is the first and second line drug for focal seizures?	Carbamazepine/valproate are 1st line. Topiramate/levetiracetam 2nd line.
What are the side effects of Valproate?	Weight gain, hair loss. Rare idiosyncratic liver failure.
What are the side effects of Carbamazepine/oxcarbazepine?	Rash, neutropenia, hyponatraemia, ataxia. Liver enzyme induction, can intefere with other medication.
What are the side effects of Vigabatrin?	Restriction of visual fields, which has limited its use. Sedation.
What are the side effects of Lamotrigine?	Rash.
What are the side effects of Benzodiazepines (clabazam, clonazepam, diazepam, nitrazepam)?	Sedation, tolerance, increased secretions.
What restrictions might you suggest for a child with newly diagnosed epilepsy?	Inform school as unrecognised absences could damage learning. Avoid deep baths (showers preferable) and swimming alone in deep water. Those with photosensitivity should sit at a distance from televisions, can cover one eye, and check that TVs and VDUs in use are acceptable (Epilepsy Action consider most moderns ones to be fine and can give advice). For adolescents, discuss driving (only after 1 year free of seizures), contraception and pregnancy.
What is SUDEP?	Sudden Unexpected Death in Epilepsy. Risks of this occurring are low and can be reduced even further by ensuring good control of seizures. Occurs in people who have had many seizures - very rare in those who are seizure-free or newly diagnosed.
What is the definition of status epilepticus?	A seizure lasting 30 min or repeated seizures for 30 min without recovery of consciousness.
What is the definition of cerebral palsy?	CP is an abnormalit of movement and posture, causing activity limitation attributed to non-progressive disturbances that occurred in the developing fetal or infant brain . The motor disorders of CP are often accompanied by disturbances of cognition, communication, perception sensation, behaviour and seizures/secondary MSK problems. It is the most common cause of motor impairment in children, affecting 2 in 1000 live births. The term is usually used for brain injuries occurring in the first 2 years of life. After this age, it is more appropriate to use acquired brain injury as the diagnosis. Althought the underlying cause is static, the resulting motor disorder may evolve, giving the impression of deterioration. The diagnosis for each child should formulate: the distribution of the motor disorder, the movement type, the cause and any associated impairment.
What is the cause of CP?	About 80% is antenatal in origin due to vascular occlusion, cortical migration disorders or structural maldevelopment of the brain during gestation. Some of these problems are linked to gene deletions. Other antenatal causes are genetic syndromes and antenatal infections. Only about 10% of cases are thought to be due to hypoxic-ischaemic injury during delivery and this proportion has remained relatively constant over the last decade. About 10% are postnatal in origin. Preterm infants are particularly vulnerable to brain damage from periventricular leucomalacia (PVL) secondary to ischaemia and/or severe intraventricular haemorrhage. Postnatal causes are meningitis/encephalitis/encephalopathy, head trauma from accidental or NAI, symptomatic hypoglycaemia, hydrocephalus and hyperbilirubinaemia.
What would be the early presenting features of CP?	Abnormal limb/trunk posture and tone in infancy with delayed motor milestones; may be accompanied by slowing of head growth. Feeding difficulties, with oromotor incoordination, slow feeding, gagging and vomiting. Abnormal gait once walking in achieved. Asymmetric hand function <12 months. Primitive reflexes may persist and become obligatory.
How is a diagnosis of CP made?	The diagnosis is made by clinical examination, with particular attention to assessment of posture and the pattern of tone in the limbs and trunk, hand function and gait. There are three main clinical subtypes: spastic (90%), dyskinetic (6%) and ataxic (4%). A mixed pattern may occur. Functional ability id described using the Gross Motor Function Classification System.
Describe the characteristic features of Spastic CP.	In this type there is damage to the upper motor neurone (pyramidal or corticospinal tract) pathway. Limb tone is persistently increased with associated brisk deep tendon reflexes and extensor plantar responses. The tone in spasticity is velocity dependent, so the faster the muscle is stretched, the greater resistance it will have. Limb involvement is increasingly described as unilateral or bilateral to acknowledge asymmetrical signs. Spasticity tends to present early and may even be seen in the neonatal period. Sometimes there is initial hypotonia, particularly of head or trunk. Three main types of spastic CP: Hemiplegia (unilateral involvement of arm and leg, face spared), Quadriplegia (all four limbs affected, often severely), Diplegia (all four limbs, but the legs are affected to a much greater degree than the arms, so hand function may appear relatively normal).
Describe the characteristic features of Dyskinetic CP.	May be described as Chorea (irregular, sudden and brief non-repetitive movements), Anthetosis (slow writhing movements occurring more distally such as fanning of the fingers), and Dystonia (simultaneous contraction of agonist and antagonistic muscles of the trunk and proximal muscles often giving a twisting appearance). Intellect may be relatively unimpaired. Affected children often present with floppiness, poor trunk control and delayed motor development in infancy. Abnormal movements may only appear towards the end of the first year of life. Signs are due to damage or dysfunction in the basal ganglia or their associated pathways (extrapyramidal). In the past the commonest cause was hyperbilirubinaemia (kernicterus) due to rhesus disease of the newborn but it is now hypoxic-ischaemic encephalopathy at term.
Describe the characteristic features of Ataxic (hypotonic) CP.	Most are genetically determined. When due to acquired brain injury (cerebellum or its connections), the signs occur on the same side as the lesion but are usually relatively symmetrical. There is early trunk and limb hypotonia, poor balance and delayed motor development. Incoordinate movements, intention tremor and an ataxic gait may be evident later.
What is a baclofen pump?	An intrathecal delivery system for baclofen, which is said to reduce spasticity.
What is Friedreich ataxia?	Autosomal recessive condition. The gene mutation (Frataxin) is a trinucleotide repeat disorder. It presents with worsening ataxia, distal wasting in the legs, absent lower limb reflexes but extensor plantar responses because of the pyramidal involvement, pes cavus and dysarthria. This is similar to the hereditary motor sensory neuropathies, but in Friedreich ataxia there is impairment of joint position and vibration sense, extensor plantars and there is often optic atrophy. The cerebellar component becomes more apparent with age. Evolving kyphoscoliosis and cardiomyopathy can cause cardiorespiratory com[romise and death at 40-50 years.
What is Ataxia telangiectasia?	A disorder of DNA repair. Autosomal recessive. The gene (ATM) has been identified. There may be a mild delay in motor development in infancy and oculomotor problems with incoordination and delay in ocular pursuit of objects (oculomotor dyspraxia), with difficulty with balance and coordination becoming evident at school age. There is subsequent deterioration, with a mixture of dystonia and cerebellar signs Many children require a wheelchair for mobility in early adolescence. Telangectasia develops in the conjunctiva, neck and shoulders from about 4 years of age. These children: Have an increased susceptibility to infection, pricipally from an IgA surface antibody defect. Develop malignant disorders, principally ALL (10%). Have a raised serum alpha-fetoprotein. Have an increased white cell sensitivity to irradiation, white can be used diagnostically, but the ATM gene test is now mostly used.
List some conditions which commonly cause developmental regression.	Batten disease (usually becomes apparent age 5-10, usually fatal in late teens/early twenties - loss of sight and motor skills, seizures), Rett syndrome, Leukodystrophies (a group of genetic disorders that are characterised by the imperfect development or poor maintenance of the myelin sheath), Wilson disease (accumulation of copper causes changes in behaviour and involuntary movements or a mixture of neuro and hepatic symptoms), Subacute sclerosing panencephalitis (SSPE - a delayed response in adolescence to prev measles infection causing neuro regression with characteristic EEG, but has become rare since measles vaccination).
Describe the different types of hydrocephalus.	There is obstruction to the flow of CSF, leading to dilatation of the ventricular system proximal to the site of obstruction. The blockage may be within the ventricular system or aqueduct (non-communicating or obstructive hydrocephalus), or at the arachnoid villi, the site of absorption of CSF (communicating hydrocephalus).
What are the signs and symptoms of hydrocephalus?	In infants, as their skull sutures haven't fused, the head circumference may be disproportionately large or show an excessive rate of growth. The skull sutures separate, the anterior fontanelle bulges and the scalp veins become distended. An advanced sign is fixed downwards gaze or 'sun setting' of the eyes. Older children will develop signs and symptoms of raised ICP.
What may cause a non-communicating hydrocephalus (obstruction in ventricular system)?	Congenital malformation - aqueduct stenosis, atresia of the outflow foramina of the 4th ventricle (Dandy-Walker malformation), Chiari malformation. Posterior fossa neoplasm or vascular malformation. Intraventricular haemorrhage in preterm infant.
What may cause a communicating hydrocephalus (failure to reabsorb CSF)?	Subarachnoid haemorrhage. Meningitis - pneumococcal, tuberculous.
What is the definition of Microcephaly and what are the potential causes?	A head circumference below the 2nd centile. May be: Familial - when it is present from birth and development is normal. An autosomal recessive condition - when it is associated with developmental delay. Caused by a congenital infection. Acquired after an insult to the developing brain e.g. perinatal hypoxia, hypoglycaemia or meningitis, when it is often accompanied by cerebral palsy and seizures.
What is the definition of Macrocephaly and what are the potential causes?	Head circumference above the 98th centile. Most are normal children and often the parents have large heads. Other causes: Tall stature, raised ICP, hydrocephalus, chronic subdural haematoma, cerebral tumour, neurofibromatosis, cerebral gigantism (Sotos syndrome), CNS storage disorders e.g. mucopolysaccharidosis (Hurler syndrome). A rapidly increased head circumference, even if still below the 98th centile, suggests raised ICP and shold be investigated promptly by intracranial USS if the anterior fontanelle is still open, otherwise by CT/MRI.
What are the four types of primary headaches?	Comprises migraine, tension-type headache, cluster headache (and other trigeminal autonomic cephalalgias) and other primary headaches (such as cough or exertional headaches). They are thought to be due to a primary malfunction of neurones.
Describe a tension-type headache.	A symmetrical headache of gradual onset, often described as tightness, a band or pressure. There are usually no other symptoms.
Describe migraine without aura.	This accounts for 90% of migraine. In children, episodes may last 1-72 hours; the headache is commonly bilateral but may be unilateral. Characteristically pulsatile, over temporal or frontal area, it is often accompanied by unpleasant gastrointestinal disturbance such as nausea, vomiting and abdo pain and photo/phonophobia. Aggravated by physical activity.
Describe migraine with aura.	10% of migraine. Headache is preceded by an aura (visual, sensory or motor), although the aura may occur without a headache. Features are the absence of problems between episodes and the frequent presence of premonitory symptoms (tiredness, difficulty concentrating, autonomic features etc). The most common aura comprises visual disturbance, which may include: negative phenomena, such as hemianopia or scotoma (small areas of visual loss). Positive phenomena such as fortification spectra (seeing zigzag lines). Rarely there are unilateral sensory or motor symptoms. Episodes normally last for a few hours, during which time children often prefere to lie down in a quiet, dark place. Sleep often relieves the bout. Symptoms of a tension-type and a migraine often overlap, and they may be part of the same pathophysiological continuum, with evidence that both result from primary neuronal dysfunction, including channelopathies, with vascular phenomena as secondary events. There is a genetic predisposition with 1st and 2nd degree relatives often also affected.
What kind of things may trigger a migraine?	Bouts are often triggered by a disturbance of inherent biorhythms, such as late nights or early rises, stress, or winding down after stress at home or school. Certain foods, e.g. cheese, chocolate and caffeine are only rarely a reliable trigger. In girls, headaches can be related to menstruation and the oral contraceptive pill.
What is familial migraine?	This is dominantly inherited and linked to a calcium channel defect.

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