Neurology Flashcards

Question

What are the idiosyncratic SEs of Sodium Valproate?

Answer 1

* Weight gain * Alopecia * **Pancreatitis** * Behaviour disturbance * **Fulminant hepatitis** (rare) MUST NOT BE USED IN PREGNANCY!!

Answer 2

* Behaviour disturbance * Increased bronchial and salivary secretions

Answer 3

* Kidney stones * Weight loss * Speech disturbance * Oligohydrosis/hyperthermia

Answer 4

* Behaviour disturbance

Answer 5

* Rash * Hyponatremia

Answer 6

* Peripheral vision impairment * Behaviour disturbance * Weight gain

Answer 7

* Rash * Behaviour disturbance

Answer 8

* Acute cerebellar ataxia occurs primarily in children 1-3 years of age, and is a diagnosis of exclusion. * It often follows a viral illness such as varicella, coxsackievirus, or echovirus by 2-3 weeks, and is thought to be due to an autoimmune response to the viral agent affecting the cerebellum. * Post-infectious cerebellitis is sudden, and the truncal ataxia can be so severe that the child can’t stand or sit. * Vomiting may occur initially, horizontal nystagmus is present in ~50% of cases, and dysarthria can be present. Resolution is usually complete by a few weeks to 2 months.

Answer 9

unilateral weakness with upper motor neuron signs, sensory abnormalities, visual complaints (with sudden loss of vision secondary to optic neuritis) or ataxia. The diagnosis may be suspected after the first episode, but may not be confirmed until a second clinical episode occurs after a month **or a new white lesion appears on MRI after 3 months**. •The pathophysiology involves demyelination and plaque formation. •Short of biopsy or autopsy findings, the diagnosis can be confirmed with MRI showing the presence of small plaques in the brain stem and spinal cord. The CSF often also contains oligoclonal bands. •Treatment with high dose methylprednisolone can quicken recovery, but does not appear to alter the long-term course of the disease. •Optic neuritis may be the first presentation of MS and aggressive treatment of optic neuritis with prednisone does appear to decrease the long-term risk of MS. •Interferon therapies can have a disease modifying effect. •The prognosis of childhood MS is similar to that of adult disease. The recovery is often complete with slow progression of disease and long periods of remission in most cases.

Answer 10

Pompe Disease

Answer 11

Carbamazepine, it increases frequency of both.

Answer 12

Brain abscess

Answer 13

Cerebellitis

Answer 14

Craniopharyngeoma

Answer 15

Disseminated encephalomyelitis

Answer 16

Pontine Glioma

Answer 17

Triad of: 1. **Developmental delay** 2. Multiple **seizure** types that include atypical absences, and myoclonic, astatic, and tonic seizures. 3. **EEG findings**: 1-2 Hz spike–and-slow waves, polyspike bursts in sleep, and a slow background in wakefulness. * Typically starts between the age of 2 and 10 yr * The tonic seizures occur either in wakefulness (causing falls and injuries) or also, typically, in sleep. * Most pts are left with long-term mental retardation and intractable seizures despite multiple therapies. Myoclonic astatic epilepsy is a syndrome similar to but milder than Lennox-Gastaut syndrome that usually does not have tonic seizures or polyspike bursts in sleep. The prognosis is more favorable than that for Lennox Gastaut syndrome.

Answer 18

Epileptic encephalopathy which is most common epileptic syndrome in infancy. Requires urgent diagnosis, investigation and treatment. 1. Epileptic spasms 2. Hypsarrhythmia 3. Developmental delay _Epileptic spasms_ * Brief tonic seizures that typically occur in a series over a minute or more, usually many times a day. * Onset usually 3-8 months of age. * Males \> females * Salaam (flexor) spasms most common - drawing up of legs, hunching forward of neck and shoulders and flinging out of arms. * Opisthotonic or extensor spasms less common. _Hypsarrhythmia_ * EEG shows diffusely disorganised pattern with high-voltage, multifocal epileptic activity (see image). _Developmental Delay_ * May be delayed prior to onset of seizures * May be loss of visual attention and arrest or regression of development at seizure onset. **_Treatment_** Corticosteroids (PO prednisolone or IM ACTH) best for rapid cessation of spasms. Also better neurological outcome in patients with no identified predisposing condition. Vigabatrin second line (first line in tuberous sclerosis).

Answer 19

Usually present after age 4 years. **_Two main syndromes:_** 1. Chilhood absence epilepsy - begins before age 10 yrs, tonic-clonic rare, good prognosis for seizure remission. 2. Juvenile absence epilepsy - later onset, sometimes teen years. Sometimes associated tonic-clonic seizure and prognosis poorer for seizure remission. **_Seizures_** * Sudden cessation of activity with staring, usually lasting only 5-15 seconds. * Blinking, upward deviation of eyes, slight mouthing and fidgeting hand movements (automatisms) may occur. * Unresponsive, does not fall, rarely incontinent, return promptly to normal activity at offset of absence with no memory of seizure. **_EEG_** * **Childhood** absence - Runs of generalised 3-Hz spike-wave activity * **Juvenile** absence - faster and more irregular spike-wave activity. **_Treatment_** * Sodium valproate (epilim), ethosuximide (zarontin) and lamotrigine (lamictal) most commonly used Rx. * Treatment for 2 years in CAE with expectation of seizure remission. * Treatment through puberty into teen years in JAE. * Rare refractory cases may respond to ketogenic diet.

Answer 20

Common epileptic syndrome in children. Occurs in otherwise normal primary school-aged children. Manifest with infrequent sleep-related focal seizures. Seizure and EEG focus in low in the central sulcus. Cause is unknown with no underlying structural brain lesions and no evidence indicating genetic association. **_Seizures_** * Onset between 5-10 years * Male predominance * Focal seizures feature tingling or twitching of the mouth, preserved consciousness. * Often associated with drooling, choking noises and inability to speak. * May progress to jerking of one side of body, with our without impaired consciousness. * Typically occur from sleep. **_EEG_** * Frequent focal epileptiform activity over the centrotemporal regions on one or both sides. **_Treatment_** * Treatment with antiepileptic medication is not always necessary as seizures are infrequent and many children only have one or two in total, and occurance is usually nocturnal. * Treatment with sodium valproate (epilim) or carbamazepine (tegretol) for 1-2 years. Excellent prognosis, without cognitive and behavioural problems and remission of seizures by teenage years.

Answer 21

Common epileptic sydrome in children occuring mostly in preschool age. Seizure and EEG focus in occipital region. **_Clinical Features_** * Presentation usually before 6 years * Female predominance * Focal seizure characteristically from sleep, beginning with staring, vomiting, head rotation, eye deviation and may lead to hemiclonic or bilateral jerking. * Daytime attacks may occur with episodic visual distortions or hallucinations and migraine-like headaches. **_EEG_** * Focal epileptiform activity over the occipital regions **_Treatment_** * Treatment with antiepileptic medication is not always necessary as seizures are infrequent and many children only have one or two in total, and occurance is usually nocturnal. * Treatment with sodium valproate (epilim) or carbamazepine (tegretol) for 1-2 years. Excellent prognosis, without cognitive and behavioural problems and remission of seizures by teenage years.

Answer 22

**_Seizures_** * Motionless staring, fearful or bewildered facial expression, unresponsiveness, hand and mouth movements that resemble voluntary actions (automatisms) and postictal amnesia and confusion. * In some patients, may be head turning or stiffening or jerking of the limbs on one side during the seizure. * Autonomic disturbances - facial flushing or pallor, salivation and vomiting - may occur. * Apnoea may be predominant manifestation in infancy. * Aura often present. Young or developmentally delayed children may describe as fear, unusual smells or tastes, abdominal discomfort and dizzy or dreamy states. * Seizures last 30-60 seconds (longer than absence seizures) and are followed by postictal confusion and sleepiness. * Occur in clusters over several days, alternating with seizure free periods. **_Treatment_** * Carbamazepine (tegretol) first drug of choice for epilepsies with focal seizures.

Answer 23

* Usually start in 1st or 2nd year of life * Up to 4% of children * Attacks are precipitated by physical or emotional trauma * Usually commence with crying, but may be brief or absent * Apnoea and bradycardia occur, suddenly or gradually, with cyanosis or pallor following. * Attack may terminate without LOC * Attack may progress with LOC, limp and sometimes brief stiffening and jerking in response to cerebral ischaemia. * Attacks usually cease by 3rd year of life. * Iron-deficiency anaemia is an exacerbating factor in some children.

Answer 24

1. Sudden or gradual development of aphasia 2. Seizures in 70% 3. Sleep EEG shows epileptiform activity +++ (ESES - electrographic status epilepticus of sleep) Age of onset usually 3-7 years

Answer 25

Benign epilepsy usually presenting in teenage years. Begins with myoclonic jerks, worse in the morning or shortly before falling asleep. Arms moreso than legs. Clusters of myoclonus can lead to absence seizures, and clusters of absence seizures can lead to GTC.

Answer 26

The clonus will continues if held or position change, but will cease when woken.

Answer 27

``` Parasomnia Parasomnias are defined as episodic nocturnal behaviors that often involve cognitive disorientation and autonomic and skeletal muscle disturbance. Parasomnias may be further characterized as occurring primarily during NREM sleep (partial arousal parasomnias) or in association with REM sleep, including nightmares, hypnogogic hallucinations, and sleep paralysis; other common parasomnias include sleep-talking. Partial arousal parasomnias, which include sleepwalking, sleep terrors, and confusional arousals are more common in preschool and school-aged children because of the relatively higher percentage of SWS in younger children. Any factor that is associated with an increase in the relative percentage of SWS (certain medications, previous sleep deprivation) may increase the frequency of events in a predisposed child. There appears to be a genetic predisposition for both sleepwalking and night terrors. In contrast, nightmares, which are much more common than the partial arousal parasomnias but are often confused with them, are concentrated in the last third of the night, when REM sleep is most prominent. Partial arousal parasomnias may also be difficult to distinguish from nocturnal seizures. Many children (15-40%) sleepwalk on at least one occasion; the prevalence of children who regularly sleepwalk is approximately 17%, and 3-4% have frequent episodes. Sleepwalking may persist into adulthood, with the prevalence in adults of about 4%. The prevalence is approximately 10 times greater in children with a family history of sleepwalking. Approximately 1-6% of children experience sleep terrors, primarily during the preschool and elementary school years, and the age of onset is usually between 4 and 12 yr. Because of the common genetic predisposition, the prevalence of sleep terrors in children who sleepwalk is about 10%. Although sleep terrors can occur at any age from infancy through adulthood, most individuals outgrow sleep terrors by adolescence. Confusional arousals commonly co-occur with sleepwalking and sleep terrors; prevalence rates have been estimated to be upwards of 15% in children ages 3-13 yr. The partial arousal parasomnias have several features in common. Because they typically occur at the transition out of “deep” or SWS, partial arousal parasomnias have clinical features of both the awake (ambulation, vocalizations) and the sleeping (high arousal threshold, unresponsiveness to the environment) states; there is usually amnesia for the events. The typical timing of partial arousal parasomnias during the first few hours of sleep is related to the predominance of SWS in the first third of the night; the duration is typically a few minutes (sleep terrors) to an hour (confusional arousals). Sleep terrors are sudden in onset and characteristically involve a high degree of autonomic arousal (i.e., tachycardia, dilated pupils), while confusional arousals typically arise more gradually from sleep, may involve thrashing around but usually not displacement from bed, and are often accompanied by slow mentation on arousal from sleep (“sleep inertia”). Sleepwalking may be associated with safety concerns (e.g., falling out of windows, wandering outside). Avoidance of, or increased agitation with, comforting by parents or attempts at awakening are also common features of all partial arousal parasomnias. ```

Neurology Flashcards

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