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SUMMARY Lennox–Gastaut syndrome is a clinically defined epileptic encephalopathy of childhood characterized by multiple seizure types generic kamagra super 160mg visa, which remain refractory to medical and surgical intervention buy 160mg kamagra super mastercard, suggestive electroencephalogram patterns safe kamagra super 160 mg, and significant mental retardation cheap kamagra super 160 mg without a prescription. The long term use of felbamate in children with severe refractory epilepsy. Vagus nerve stimulation in children with refractory seizures associated with Lennox–Gastaut Syndrome. Topiramate in Lennox–Gastaut syndrome: open label treatment of patients completing a randomized controlled trial. Non convulsive status epilepticus— a possible cause of mental retardation in patients with Lennox Gastaut syndrome. Topiramate: efficacy and tolerability in children according to epilepsy syndromes. Lamotrigine for generalized seizures asso- ciated with the Lennox Gastaut syndrome. A double-blind, randomized trial of topiramate in Lennox–Gastaut Syndrome. The efficacy of felbamate as add-on therapy to valproic acid in the Lennox Gastaut syndrome. INTRODUCTION In 1957, Landau and Kleffner reported a group of children with a syndrome of acquired epileptic aphasia (Landau–Kleffner syndrome, LKS) and in 1971, Patry et al. These syndromes can be relatively distinct and the Inter- national League Against Epilepsy has recognized them as separate syndromes. An overlap of symptoms of these conditions has led to a developing view that these disorders may be related to each other with the common feature of electrical status epilepticus in sleep (ESES). Furthermore, they may be related to the less severe condition of benign childhood epilepsy with central–temporal spikes. CLINICAL FEATURES In the relatively pure form of LKS, children usually do not have antecedent devel- opmental or neurological abnormalities. Seizures, typically partial or generalized tonic–clonic, occur in 70–80% of individuals and may precede or develop around the same time as the language deterioration. The language impairment usually appears as a receptive aphasia or verbal auditory agnosia (VAA) with intact hearing. Reading, writing, and use of visual cues often are preserved initially, but may deteriorate over time. Associated features include hyperactivity, inattention, irritability, and mild motor apraxia. Routine imaging studies are generally normal, although tumors, neurocysticercosis, congenital hemi- paresis, a history of encephalitis and other conditions have been reported in associa- tion with LKS. The seizures are usually easily controlled, but the language impairment is often more refractory to treatment. Adverse factors for language recovery 85 86 Trescher include: younger age on onset, longer duration of ESES, and spread of the spikes bilaterally. Most children have seizures, which may be partial, generalized tonic–clonic, as well as myoclonic, atonic, or atypical absence. Seizures, when they occur, develop as early as the first year of life, often preceding the onset of ESES by 1–2 years. Global cognitive deterioration, behavioral dysfunction, and motor impairments are more severe than with LKS. Approximately one-third of children have a history of antecedent neuro- logical problems or abnormalities on imaging studies. Precise diagnosis of these conditions may be difficult because many cases described in the literature and possibly a greater number of children presenting to clinicians do not manifest classic symptoms, but rather have intermediate forms of these disorders. Further complicating diagnosis, approximately one-third of children with autistic spectrum disorder (ASD) experience language regression, albeit at a much earlier age than the loss of language associated with LKS. While the incidence of epilepsy among children with autistic ASD is no different between those with and without regression, there is some evidence to suggest that the incidence of epilepti- form discharges on EEG is greater among those with language regression compared to those without regression. The term Landau–Kleffner variant often is used to char- acterize those children with infantile autism with language regression and coincident epileptiform discharges, but this designation is not universally accepted. DIAGNOSIS The key to diagnosis is recognition of the clinical syndrome of a loss of language function with or without a deterioration of cognitive abilities and behavior. Routine daytime EEGs that include at least 20–30 min of sleep may capture ESES, but caution is advised in the event of a negative daytime study in the setting of cognitive, behavioral, or language deterioration.

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To achieve success with a small group you must also have a clear under- standing of how a group operates and how it develops generic kamagra super 160mg with amex. You have particular responsibilities as the initial leader of the group but your role will vary considerably both within a session and from session to session purchase kamagra super 160mg on-line. For instance generic 160mg kamagra super otc, if you adopt an autocratic or authoritarian style of leadership (not an uncommon one among medical teachers) you may well have a lot of purposeful activity but there will be a limited amount of spontaneous participation kamagra super 160 mg free shipping. You should pre- ferably adopt a more co-operative role where you demonstrate an expectation that the students will take responsibility for initiating discussion, providing informa- tion, asking questions, challenging statements, asking for clarification and so on. A successful group is one that can proceed purposefully without the need for constant intervention by the teacher. This is hard for most teachers to accept but is very rewarding if one recognises that this independence is one of the key goals of small group teaching and is more important than satisfying one’s own need to be deferred to as teacher and content expert. These are factors relating to the task of the group and factors relating to the maintenance of the group. In addition there must be a concern for the needs of each student within the group. The tasks of the group: clear definition of tasks is something that must be high on the agenda of the first meeting. The reason for the small group sessions and their purpose in the course must be explained. In addition, you should initiate a discussion about how you wish the group to operate, what degree of preparation you expect between group meetings, what role you intend to adopt, what roles you expect the students to assume and so on. Because such details may be quickly forgotten it is desirable to provide the students with a handout. Ideally it is one that is open, trustful and supportive rather than closed, suspicious, defensive and competitive. It is important to establish that the responsibility for group maintenance rests with the students as well as with the teacher. The firm but pleasant handling of the loquacious or dominating students early in the session or the encouragement of the quiet student are obvious examples of what can be achieved to produce the required environment for effective group discussion. The successfully managed group will meet the criteria shown in Figure 3. A structured approach to tasks and to the allocation of the time available is a useful tool for you to consider. An example of such a structured discussion session is illustrated in Figure 3. Note that the structure lays out what is to be discussed and how much time is budgeted. Such a scheme is not intended to encourageundue rigidity or inflexibility, but to clarify purposes and tasks. This may seem to be a trivial matter, but it is one which creates considerable uncertainty for students. You need to be alert to how time is being spent and whether time for one part of the plan can be transferred to an unexpected and important issue that arises during discussion. Another structure, not commonly used in medical educa- tion, is illustrated in Figure 3. From an individual task, the student progresses through a series of small groups of steadily increasing size. There are special advantages in using this structure which are worth noting: it does not depend on prior student preparation for its success; the initial individual work brings all students to approximately the same level before 45 discussion begins; and it ensures that everyone partici- pates, at least in the preliminary stages. INTRODUCING STIMULUS MATERIALS A very useful means of getting discussion going in groups is to use what is generally known as ‘stimulus material’. We have seen how this was done in the snowballing group structure described previously. It is limited only by your imagination and the objectives of your course. Here are a few examples: A short multiple-choice test (ambiguous items work well in small groups). X-rays, photographs, slides, specimens, real objects, charts, diagrams, statistical data). A journal article or other written material (an interesting example is provided by Moore where he used extracts from literary works to help students understand the broader cultural, philosophical, ethical and personal issues of being a doctor.

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Care should be taken to avoid generating air currents with the hand movement as this may stimulate the corneal reflex which may simulate the visuopalpebral reflex kamagra super 160mg with visa. It is probable that this reflex requires cortical processing: it is lost in persistent vegetative states order kamagra super 160mg with visa. Loss of this reflex may occur in Balint’s syndrome generic kamagra super 160mg with amex, ascribed to inability to recognize the nearness of the threatening object quality 160 mg kamagra super. The final common (efferent) pathway for these responses is the facial nerve nucleus and facial (VII) nerve, the afferent limbs being the trigeminal (V), optic (II), and auditory (VIII) nerves respectively. Electrophysiological study of the blink reflex may demonstrate peripheral or central lesions of the trigeminal (V) nerve or facial (VII) nerve (afferent and efferent pathways, respectively). It has been reported that in the evaluation of sensory neuronopathy the finding of an abnormal blink reflex favors a nonparaneoplastic etiology, since the blink reflex is normal in paraneoplastic sensory neuronopathies. Journal of Clinical Neuro-ophthalmology 1992; 12: 47-56 Cross References Balint’s syndrome; Blinking; Corneal reflex; Glabellar tap reflex Body Part as Object In this phenomenon, apraxic patients use a body part when asked to pan- tomime certain actions, such as using the palm when asked to demon- strate the use of a hair brush or comb, or fingers when asked to demonstrate use of scissors or a toothbrush. Ideomotor apraxia in patients with Alzheimer’s disease: why do they use their body parts as objects? Neuro- psychiatry Neuropsychology and Behavioral Neurology 2001; 14: 45-52 Cross References Apraxia “Bon-Bon Sign” Involuntary pushing of the tongue against the inside of the cheek, the “bon-bon sign,” is said to be typical of the stereotypic orolingual move- ments of tardive dyskinesia, along with chewing and smacking of the mouth and lips, and rolling of the tongue in the mouth. These signs may help to distinguish tardive dyskinesia from chorea, although periodic pro- trusion of the tongue (flycatcher, trombone tongue) is common to both. Cross References Chorea, Choreoathetosis; Trombone tongue Bouche de Tapir Patients with facioscapulohumeral (FSH) dystrophy have a peculiar and characteristic facies, with puckering of the lips when attempting to whistle. The pouting quality of the mouth, unlike that seen with other types of bilateral (neurogenic) facial weakness, has been likened to the face of the tapir (Tapirus sp. Cross References Facial paresis Bovine Cough A bovine cough lacks the explosive character of a normal voluntary cough. It may result from injury to the distal part of the vagus nerve, particularly the recurrent laryngeal branches which innervate all the muscles of the larynx (with the exception of cricothyroid) with result- ant vocal cord paresis. Because of its longer intrathoracic course, the left recurrent laryngeal nerve is more often involved. A bovine cough may be heard in patients with tumors of the upper lobes of the lung (Pancoast tumor) due to recurrent laryngeal nerve palsy. Bovine cough may also result from any cause of bulbar weakness, such as motor neu- rone disease, Guillain-Barré syndrome, and bulbar myopathies. New England Journal of Medicine 1997; 337: 1370-1376 Cross References Bulbar palsy; Diplophonia; Signe de rideau Bradykinesia Bradykinesia is a slowness in the initiation and performance of voluntary movements, one of the typical signs of parkinsonian - 58 - Broca’s Aphasia B syndromes, in which situation it is often accompanied by difficulty in the initiation of movement (akinesia, hypokinesia) and reduced ampli- tude of movement (hypometria) which may increase with rapid repet- itive movements (fatigue). It may be overcome by reflexive movements or in moments of intense emotion (kinesis paradoxica). Bradykinesia in parkinsonian syndromes reflects dopamine depletion in the basal ganglia. It may be improved by levodopa and dopaminergic agonists, less so by anticholinergic agents. Slowness of voluntary movement may also be seen with psy- chomotor retardation, frontal lobe lesions producing abulia, and in the condition of obsessive slowness. Cross References Abulia; Akinesia; Fatigue; Hypokinesia; Hypometria; Kinesis paradoxica; Parkinsonism; Psychomotor retardation Bradylalia Bradylalia is slowness of speech, typically seen in the frontal-subcorti- cal types of cognitive impairment, with or without extrapyramidal fea- tures, or in depression. Cross References Palilalia; Tachylalia Bradyphrenia Bradyphrenia is a slowness of thought, typically seen in the frontal- subcortical types of cognitive impairment, e. Such patients typically answer questions correctly but with long response times. Cross References Abulia; Dementia Bragard’s Test - see LASÈGUE’S SIGN Broca’s Aphasia Broca’s aphasia is the classic “expressive aphasia,” in distinction to the “receptive aphasia”of Wernicke; however, there are problems with this simple classification, since Broca’s aphasics may show compre- hension problems with complex material, particularly in relation to syntax. Considering each of the features suggested for the clinical classi- fication of aphasias (see Aphasia), Broca’s aphasia is characterized by: ● Fluency: slow, labored, effortful speech (nonfluent) with phonemic paraphasias, agrammatism, and aprosody; the patient knows what s/he wants to say and usually recognizes the paraphasic errors (i. Silent reading may also be impaired (deep dyslexia) as reflected by poor text comprehension. Aphemia was the name originally given by Broca to the language disorder subsequently named “Broca’s aphasia. Broca’s aphasia is sometimes associated with a right hemiparesis, especially affecting the arm and face; there may also be bucco-lingual-facial dyspraxia.

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