05_Semple_Chap05.indd 144 12/7/2012 3:28:34 PM VASCULAR DEMENTIA 145 syphilis serology are additional tests in unusual cases (e.g. \200\230young strokes \200 \231). ECG, CXR, CT, and MRI are essential. Other investigations may include: echocardiogra phy (for cardiac/ valvular defects or disease); carotid artery Doppler ultrasound. \200 Management \200 \200 Establish causative factors. Medical or surgical diseases that are contributory need to be treated early. Daily aspirin may delay course of disease. General health interventions incl ude changing diet, stopping smoking, managing hypertension, optimizing diabetic control, an d increasing exercise. \200 1 Rossor M, Brown J (1998) Vascular and other dementias. In: R Butler and B Pitt, eds, Semi nars in old age psychiatry. London, Gaskell. 2 Gelder M, Gath D, Mayou R, et al. (1996) Oxf ord textbook of psychiatry, 3rd edn. Oxford: Oxford University Press. 05_Semple_Chap05.indd 145 12/7/2012 3:28:34 PM CHAPTER 5 Organic illness 146 Prion diseases Prion diseases are rapid, aggressive, dementing illnesses caused by depos- i ts of prion proteins throughout the brain. They are rare and are best considered as slow vi rus infections. Prions spread throughout the brain by causing irreversible change in neighb oring tissue. The typical pathological \201 nding is a spongy encephalopathy, and in term s of the nosology of the dementias, prion disease is considered a multifocal dementia. Whil e prion diseases tend to respect the species barrier (e.g. \200\230scrapie \200\231 is a p rion disease limited to sheep), there is emerging evidence that this is not always the case (e.g. vCJD). Three diseases are recognized in humans. Creutzfeldt \200\223Jakob disease (CJD) A rare disease (approximately 50 cases/year in UK) of 50 \200\22370yrs-olds with equal sex distribution. 85% cases are spontaneous or sporadic ; 10% result from genetic mutation; 5% result from iatrogenic transmission during transplan t surgery of dura, corneal grafts, and pituitary growth hormone. The clinical picture is on e of rapidly deteriorating dementia, cerebellar and extra-pyramidal signs, myoclonus, and d eath within a year. EEG shows periodic complexes. CT atrophy of cortex and cerebellum. New variant CJD (vCJD): BSE The rise of vCJD followed an epidemic of bovine spongiform ence phalopa- thy (BSE) in cattle. BSE is a prion disease of cows that is thought to have been s pread by cattle feeds that contained CNS material from infected cows. The disease in humans affects mainly young people in their 20s and is characterized by early anxiety and depress ive symptoms, followed by personality changes, and \201 nally a progressive dementia. Ata xia and myo- clonus are prominent and the typical course is 1 \200\2232yrs until death. EEG and CT changes are similar to CJD. Kuru This was a rare disease of New Guinea cannibals who ate the brains of their deceased r elatives. The incubation period was prolonged \200\224 up to 40yrs before disease onset, th en progression was rapid and fatal (Box 5.5). 05_Semple_Chap05.indd 146 12/7/2012 3:28:34 PM PRION DISEASES 147 Box 5.5 Recent research reveals \200\230cannibalism genotype \200\231 Researchers at University College London have recently suggested that cannibalism was commo n and widespread in human ancestors. They analyzed DNA from 30 elderly Fore women from Papu a New Guinea who had participated in many cannibalistic feasts before they were banned by t he Australian government in the 1950s. It was the practice of the Fore for women and childr en to consume the brains of dead kin in the belief that this act would \200\230recycle \200\231 the spirit of the dead within the living. At the peak of the epidemic (1920 \200 \2231950) kuru killed 1% of the population annually. Most of the women survivors tested by researchers had a particular genotype that was much less common in the younger population, indicating that it conferred substantial protection against the disease. Interestingly, non e of the patients who have to date contracted new variant CJD in Britain carry the protecti ve genotype. This suggests that this genotype is protective against prion diseases in human s. The researchers then examined DNA from various ethnic groups around the world and found that all, except the Japanese, carried the protective genotype to a similar degree. Genetic tests showed that this gene could not be there by chance, but was a result of natural sele ction. This implies that ancestral human populations were exposed to some form of prion dis ease. Researchers conclude that frequent epidemics of prion disease caused by cannibalism i n human ancestors would explain the worldwide existence of the protective genotype in moder n humans. From Mead S, Stumpf MP, Whit \201 eld J, et al. (2003) Balancing selection at the prion pr otein gene consistent with prehistoric kurulike epidemics. Science 300: 640 \200\2233. 05_Semple_Chap05.indd 147 12/7/2012 3:28:34 PM CHAPTER 5 Organic illness 148 Amnestic disorders The amnestic disorders are syndromes characterized by memory impair- men t (anterograde and/or retrograde amnesia), which are caused by a gen- eral medical conditio n or substance use, and where delirium and dementia have been excluded as causative of the amnesia. Amnestic disorders may be transient or chronic (< or >1mth). Amnestic conditions u sually involve some or all of the following neuroanatomical structures: frontal cortex; hip pocampus and amygdala; dorsomedial thalamus; mamillary bodies; and the periaqueductal gray matter (PAG). In terms of neurochemistry, gluta- mate transmission at the NMDA receptor is often implicated in amnesia, mainly due to its role in memory storage in the limbic system: long-term potentiation (LTP). A number of amnestic disorders are recognized: Wernicke \200 \231s encephalopathy (b p. 572) An acute syndrome, with a classic tetrad of symptoms (ataxi a, ophthalmo- plegia, nystagmus, and acute confusional state), caused by thiamine deple- ti on, usually related to alcohol abuse, and associated with pathological lesions in the mamil lary bodies, PAG, thalamic nuclei, and the walls of the third ventricle. Korsakoff psychosi s (b p. 572) Amnesia and confabulation associated with atrophy of the mamillary bod- ies, u sually following Wernicke \200\231s encephalopathy (rarer causes include: head injury; hypo xia brain injury; basal/temporal lobe encephalitis; and vascular insult). Vascular disease Insults to the hippocampus (especially involving the posterior cerebral ar tery or basilar artery) may result in an amnestic disorder. Other regions implicated includ e: parietal \200\223occipital junction; bilateral medio-dorsal thala- mus; basal forebrain nuclei (e.g. aneurysm of the anterior communicating artery). Head injury An open or closed head injury involving acceleration or deceleration forces may result in injury to the anterior temporal poles (as this structure collides with the tempo ral bone). Anterograde or post-traumatic amnesia (PTA) is prominent with retrograde amnesia relatively absent. Prognosis is related to length of PTA \200\224better prognosis associat ed with PTA of less than 1wk. HSV encephalitis Affects medial temporal lobes and results in de \201 cits in short-term m emory (STM) storage. Temporal lobe surgery Bilateral damage or surgery to the medial temporal lobes results in a n ina- bility to store new short-term memories (e.g. \200\230patient HM \200\231) (see Box 5.6). 05_Semple_Chap05.indd 148 12/7/2012 3:28:34 PM AMNESTIC DISORDERS 149 Hypoxic brain damage Hypoxia following asphyxia from CO poisoning, near drowning, etc., may damage sensitive CA1 and CA3 neurons in the hippocampus. This results in problems with STM storage. MS 40% of patients have some amnesia due to plaques in the temporal lobes and diencephalon resulting in dif \201 culty with recall. Alcohol blackouts ( \200\230palimpsest \200\231) Signi \201 cant alcohol intoxication may lead to amnesia for the period of intoxication. This usually only occurs in the context of chronic alcohol misuse. ECT There may be a period of mild anterograde and/or retrograde amnesia for several hours f ollowing administration of ECT. More rarely there may be ongoing patchy memory loss for up to 6 \200\2239mths. Transient global amnesia (TGA) This is a syndrome of amnesia lasting 6 \200\22324hrs caused by transient ischae- mia of the temporal lobes and/or diencephalon. It is more common over 50yrs and may occur in the context of hypertension or migraine. Differential diagnosis inc ludes dissociative disorders and malingering, and diagnosis is often unclear. Other causes of amnesia Substances (BDZs, anticholinergics); space-occupying tumors); hypog lycaemia. lesions (e.g. Box 5.6 Patient HM On 23 August 1953, patient HM underwent a bilateral medial temporal lobe resection in an at tempt to control his epileptic seizures. This resulted in a severe anterograde memory impai rment that has made HM one of the most studied patients in the history of cognitive psychol ogy. HM \200\231s syndrome is surprisingly isolated, with impairment mostly lim- ited to hi s inability to register new facts into long-term memory, despite immediate memory being pre served for both verbal and non-verbal tasks. Although his operation was performed when he w as 27, his mem- ories are intact until age 16, with an 11-year retrograde amnesia. His IQ is above average, with almost normal language production and comprehension \200\224h e can understand and produce complex verbal material (but is impaired on tests of semantic and symbolic verbal \202 u- ency). His perceptual abilities are normal except for his sen se of smell (2 to damage of the olfactory tracts). Despite the fact that some of his spa- tial abilities are compromised, he does not have any attentional de \201 cit. 05_Semple_Chap05.indd 149 12/7/2012 3:28:34 PM CHAPTER 5 Organic illness 150 Normal pressure hydrocephalus (NPH) Essence A syndrome where there is dilatation of cerebra l ventricles (especially third ventricle) and normal cerebrospinal \202 uid (CSF) pressur e at lumbar puncture. It typically presents with the triad of: dementia, gait ataxia, and u rinary incontinence. Importantly, the dementia is potentially reversible if NPH is treated promptly. Etiology 50% cases are idiopathic. 50% are secondary to mechanical obstruction of CSF \202 ow across the meninges (e.g. meningitis; subarach- noid hemorrhage; trauma; rad iotherapy). Clinical features There is progressive slowing of cognitive and motor functioni ng consistent with a pattern of subcortical dementia. Ataxia is due to pyramidal upper moto r neuron paraparesis. Urinary incontinence is a late symptom. Investigations CT scan shows isize of the lateral ventricles and thin- ning of the cortex. 24-hr intracranial pressure m onitoring shows typical \200\230B \200\231 pattern. Treatment Those cases where NPH is sec ondary to an identi \201 ed cause are the best candidates for ventriculo-peritoneal shunt. 05_Semple_Chap05.indd 150 12/7/2012 3:28:34 PM CHRONIC SUBDURAL HAEMATOMA 151 Chronic subdural haematoma (SDH) Essence An insidious and \202 uctuating organic syndrome may result from an undetected chronic subdural haematoma. An SDH results from rupture of t he bridging veins between the dura and arachnoid mater and tends to occur over the frontal and/or parietal cortices. In 30% of cases there is bilateral SDH. SDH should be suspected w here there is a changing pattern in cognitive function, especially if risk factors for SDH exist: post trauma; elderly after a fall; infancy; cerebral atrophy (e.g. chronic alcoholis m); clot- ting disorders. Clinical features An SDH may only manifest with symptoms months a fter it develops, therefore there may be no history of recent trauma. Headache, altered lev el of consciousness, and amnesia may all occur, often with \202 uctuations in severity. T ypically, the mental state may be variable on different occasions and there may be periods of unusual drowsiness as well as both cognitive and physical slowness and sluggishness. Min or focal signs are sometimes detected. The general trend is towards a dementia picture, whi ch is characteristically of a subcortical nature. Investigations CT scan during the \201 rst 3wks may not show the SDH as it is isodense during the early phase. Therefore, contrast should be used. Later on, as the SDH lique \201 es, a low-density convexity may be detect ed over the fronto-parietal cortex. Treatment Surgical drainage of SDH via burr holes. Ster oids for con- servative treatment. 05_Semple_Chap05.indd 151 12/7/2012 3:28:35 PM CHAPTER 5 Organic illness 152 Psychiatric sequelae of CVA A range of psychiatric problems may occur following stroke. The se include: Cognitive disorders \200 \200 \200 b p. 144). Vascular dementia (see Subcortical dementia (see Amnestic disorder (see b p. 133). b p. 148). Personality changes These tend to involve a constriction in the range of interests and a lo ss of intellectual \202 exibility. Irritability is common and \200\230catastrophic react ions \200\231 may occur in response to stress or change in routine. Emotional \202 ex- ib ility may be reduced and affective responses often become shallow and stereotyped. Pathological emotionalism May occur 4 \200\2236wks post-CVA, especially following left fron tal infarcts. Presentation involves outbursts of unprovoked and uncontrollable emo- tion an d distress, with disinhibition a major feature. Post-stroke depression Depressive illness after stroke is extremely common, occurring in up to 60% of cases. Its onset is usually between 3 and 24mths following the stroke. It was a neglected phenomenon until recently. Many cases are mis- or under-diagnosed with symptoms b eing attributed to the cognitive insult. Etiology Biological factors \200\224direct physiological effects of the brain injury, with location (e.g. left anterior frontal), large size, increased age, and female gender being r isk factors. Psychological factors \200\224sudden depend- ency; disability; premorbid perso nality traits (especially neurotic or highly independent individuals). Social risk factors \200\224being alone; lack of social sup- port; \201 nancial worries. Treatment Antidepressants: SSRIs and mianserin are considered safe following stroke but som e evidence suggests that TCAs are more ef \201 cacious. Psychoses Manic, hypomanic, and paranoid psychoses may result from a CVA, espe- cially righ t hemisphere infarcts. Peduncular hallucinosis is an uncommon psychosis characterized by vi sual and auditory hallucinations and is associ- ated with infarcts involving the pons and m idbrain. Korsakoff psychosis (b p. 572) A rare chronic complication of subarachnoid hemorrh age. 05_Semple_Chap05.indd 152 12/7/2012 3:28:35 PM This page intentionally left blank CHAPTER 5 Organic illness 154 Psychiatric aspects of head injury1 Head injuries are unfortunately common in a world where there is mass use of motor vehicles and widely misused alcohol. The peak incidence of head injury is in individuals between the ages of 15 and 24yrs and improved medical care has re sulted in larger numbers of individuals surviving with neuropsychiatric consequences. Most head injury survivors who present to psychiatric services have emotional symptoms and perso nality changes ranging from subtle to severe. A smaller number manifest serious and last- i ng cognitive sequelae, such as apathy, disinhibition, and amnesia. There are also important acute psychiatric effects. Acute psychological effects of head injury Most signi \201 cant head injuries are closed a nd involve a period of loss of consciousness (which may extend from brief concussion to pro longed coma). Often amnesia follows recovery of consciousness: \200 Post-traumatic amnesia (PTA) extends from the time of the injury until normal memory resume s. Apparently normal behavior often occurs during this period. PTA may end abruptly. Retrog rade amnesia (RA) recalled memory prior to the injury and the injury itself. It is usually a dense amnesia that is brief, lasting seconds or minutes, and shrinks with time. includes the period between the last clearly Acute post-traumatic delirium (PTD) A state that may follow severe head injury and occurs a s the individual begins to regain consciousness. This is sometimes also called \200\230pos t-trau- matic psychosis \200\231 and is characterized by prolonged and variable confusion, with or without behavioral symptoms, anxiety, affective lability, paranoia, delusional misi nterpretation, and hallucinations. Factors associated with increased psychiatric morbidity following head injury \200 \200 \200 \200 \200 \200 \200 Increased duration of loss of consciousness Increased duration of PTA Increased duration of PTD Increased age, arteriosclerosis, and alcoholism Increased area of damage Increased neu rological sequelae (focal de \201 cits, epilepsy, etc.) Dominant or bilateral hemisphere i nvolvement Chronic psychiatric syndromes following head injury A number of chronic syndromes are recog nized following head injury: \200 Cognitive impairment lasting >24hr. There may be focal cognitive de \201 cits such as amne sia, or diffuse problems including slowing, apathy, affective blunting, decreased concentra tion, executive dif \201 culties, amnesia, and affective lability. Catastrophic reactions and emotional incontinence may occur. In its severest form the cognitive impairment may pre sent as a dementia (post-traumatic dementia). If symptoms are severe, it is particularly im portant to exclude NPH, SDH, or co-existing DAT. Treatment: antipsychotics; stimulants. Especially after closed head injuries with PTA 05_Semple_Chap05.indd 154 12/7/2012 3:28:35 PM PSYCHIATRIC ASPECTS OF HEAD INJURY 155 Personality changes are most likely Personality/behavioral changes after head injury to the orbito-frontal lobe or anterior temporal lobe. \200\230Frontal lobe syndrome \200\231 is characterized by disinhibition, impulsivity, irritability, and aggressive outbursts. Treatm ent: may include B-blockers (e.g. atenolol), carbamazepine. Psychoses is associated with le ft temporal injury, while affective psychoses (esp. mania in 9% patients) are associated wi th right temporal or orbito- frontal injury. There is also an increased prevalence of schiz ophrenia post-head injury (2.5% develop the disorder). Treatment: cautious use of antipsych otics (risk of seizure), anticonvulsants. Neurotic disorders Depressive illness is most com mon but anxiety states (including PTSD) are common sequelae. Persistent depression and anxi ety occur in roughly 1 \201\2044 of head injury survivors. Suicide risk is also higher post head injury. Treatment: SSRIs, ECT. \200\230Punch-drunk syndrome \200\231 Boxers may deve lop diffuse injury to the cortex, basal ganglia, and cerebellum, giving rise to extra-pyram idal symptoms or a subcortical dementia. Pathology shows cerebral atrophy and neuro \201 b rillary tangles. \200\230Post-traumatic syndrome \200\231 Also called \200\230post-concus sional syndrome \200\231. This is a common phenomenon after head injury. The main symptoms are: headache; dizziness; insomnia; irritability; emotional lability; increased sensitivity to noise, light, etc.; fatigue; poor concentration; anxiety; and depression. Although this syndrome was previously thought to be a purely psychological phenomenon (since in many cas es the injury was minor), it is now recognized that it probably involves a complex interpla y of both organic and non-organic factors. A schizophrenia-like psychosis with prominent paranoia Factors in \202 uencing psychiatric disability and prognosis \200 Mental constitution \200\224i.e. vulnerability due to genetics, temperament (premorbid pers onality: increased risk in histrionic, hypochondriacal and dependent personalities), IQ (ce rebral reserve), age. Emotional impact of injury \200\224i.e. extent of psychological traum a. Setting, circumstances, and repercussions of injury. Iatrogenic factors. Home and social environment (including secondary gain issues). Compensation and litigation issues (includi ng secondary gain issues). Post-traumatic epilepsy (PTE) \200\224occurs in 5% closed and 30 % open head injuries (usually during \201 rst year) and worsens prognosis. Size and locat ion of brain damage: frontal, temporal, dominant side worse. \200 \200 \200 \200 \200 Sequelae in children Less psychopathology after head injury due to increased brain plastici ty. Recovery may continue for up to 5yrs after injury (as opposed to 2yrs in adults). Prob lems are generally behavioral in nature and include aggres- sion, delinquency and attention de \201 cit hyperactivity disorder (ADHD)-like syndromes. 1 Rao V, Lyketsos C (2000) Neuropsychiatric sequelae of traumatic brain injury. Psychosomat ics 41: 95 \200\223103. 05_Semple_Chap05.indd 155 12/7/2012 3:28:35 PM CHAPTER 5 Organic illness 156 Psychiatric aspects of epilepsy 1 The lifetime prevalence of experiencing a \200\230seizur e \200\231 is approximately 5%, while the prevalence of epilepsy (recurrent seizures) is 0. 5 \200\2231%. Most (760%) cases of epilepsy have unknown etiology. Seizures may be gen- era lized or focal. Generalized seizures involve the whole cortex and lead to loss of conscious ness (LOC). Focal seizures begin in one area of the cortex and may become secondarily gener alized. Focal seizures are sub- classi \201 ed as simple partial (i.e. localized motor/sen sory features without LOC or memory loss); complex partial (i.e. with or without aura/autom a- tism, and associated changes in conscious level). Between 10% and 50% of patients with e pilepsy have psychiatric symptoms. Psychiatric aspects of epilepsy may be related to: \200 Psychosocial consequences of diagnosis (e.g. unemployment, stigma and ostracism, restricted activities, dependency). Psychiatric syndromes directly attributed to epilepsy. Neuropsych iatric effects of medication. \200 Psychiatric syndromes attributed to epilepsy are best considered by their temporal relation ship to seizures (pre-ictal, ictal, post-ictal, inter-ictal). Pre-ictal \200 Patients may experience a variety of vague symptoms during the days and hours leading up to the seizure. These are termed prodromal symptoms and include feelings of tension, dysphori a, and insomnia. An aura may occur immediately prior to seizure onset. This is most common in complex partial seizures (CPSs) \200\224temporal lobe epilepsy or extra-temporal epileps y (e.g. frontal CPSs). Auras are typically stereotyped, e.g. autonomic or visceral aura (ep igastric sensation); derealization and depersonalization experiences; cognitive symptoms (d ysphasia, \200\230forced thinking \200\231, ideomotor aura, d j vu, jamais vu, fugue an d twilight states); affective symptoms (anxiety, euphoria); perceptual experiences (auditor y, visual, sensory, and olfactory hallucinations or illusions). Ictal \200 Automatisms origin for the seizure such as medial temporal lobe. There is LOC during the ic tus and amnesia for the automatism, which usually lasts <5mins. There are simple or complex stereotyped movements that tend to be disorganized and purposeless (although complex actio ns may be carried out). At this time the individual seems \200\230out of touch \200\231. A utomatisms may be the basis of twilight and fugue states (EEG may aid differential diagnosi s). Epilepsy partialis continuans (EPC) lasting hours \200\223days (may be confused with de lirium or psychosis) (e.g. temporal, frontal, or cingulate seizures). There are variable be havioral, cognitive, and perceptual symptoms and periods of amnesia. Bizarre aggressive beh avior. May occur during the seizure and suggest a focal A condition of prolonged CPSs, 05_Semple_Chap05.indd 156 12/7/2012 3:28:35 PM PSYCHIATRIC ASPECTS OF EPILEPSY 1 157 Post-ictal \200 Post-ictal delirium following a seizure with disorientation, inattention, variable levels o f consciousness, and sometimes paranoia. Tends to last hours to days and shows a trend towa rds improvement and normal consciousness. If prolonged, suspect EPC. Post-ictal psychosis i n the frequency of seizures; may follow withdrawal of anticonvulsant therapy. Thought to re sult from sub-threshold kindling activity. It usually only occurs in individuals with epile psy for >10yrs (particularly associated with a left temporal lobe focus). Clinically, there is an initial non-psychotic interval (lasting hours to weeks) following a seizure. Thereaf ter, the individual develops a brief psychotic episode with variable psychotic and affectiv e symptoms. The episode resolves after a period of days to 1mth. It may recur 2 or 3 times in a year. EEG shows marked changes during the psychotic episode. A very common (10%) confusional state 1 Usually follows a cluster of seizures or an increase Inter-ictal \200 Brief inter-ictal psychosis is good control of epilepsy. In this way seizures are antagonis tic to the psychosis in that the EEG normalizes during the psychosis. This is called forced normalization. A seizure may end the psychotic episode. This form of psychosis has been te rmed alternating psychosis (i.e. there is an inverse relationship between severity of epile psy and severity of psychosis). There may be premonitory symptoms such as anxiety and insom nia while the psychosis is characterized by hallucinations and paranoia. Notably the antago nistic relationship between seizures and psychosis is demonstrated where anticonvulsants ma y aggravate psychosis, and where antipsychotics may reduce the seizure threshold. Chronic i nter-ictal \200\230schizophrenia-like \200\231 psychosis schizophrenia-like psychotic illn ess is 6 \200\22312 times more common in epileptics than in the general population. It is p articularly associated with left temporal lobe epilepsy and is more common in early onset s evere epilepsy and in women with epilepsy. There is often a period of 10 \200\22315yrs that elapses between diagnosis of epilepsy and onset of the psychotic illness. Clinically the i llness is very similar to idiopathic schizophrenia, although there tends to be a prominent affective component. The chronic course is likewise similar. There is typically no family h istory of schizophrenia and an absence of premorbid schizotypal traits. Pathologically, it may represent the cumulative effects of chronic kindling due to a temporal lobe focus, e.g. in temporal lobe epilepsy (TLE). occurs unrelated to a seizure, when there A chronic 1 Sachdev P (1998) Schizophrenia-like psychosis and epilepsy: the status of the association . Am J Psychol 155: 325 \200\22336. 05_Semple_Chap05.indd 157 12/7/2012 3:28:35 PM CHAPTER 5 Organic illness 158 Psychiatric aspects of epilepsy 2 Other presentations Cognitive deterioration is a common o utcome of chronic epilepsy and is caused by a number of factors, including repeated seizure s with cer- ebral hypoxia as well as the neurological effects of chronic anticonvulsant the rapy. Neuroses Epilepsy is associated with a 50% risk of a major depressive episode. Suicide risk is also increased \22725 over non-epileptics. There is an increased prevalence of convers ion disorder in epileptics, including an increased risk of \200\230pseudoseizures \200\231 . Mania Flor-Henry \201 rst described the association between right-side TLE and manic illn ess. Epileptic personality syndrome Also named the Waxman \200\223Geshwind syndrome, this is a c ontroversial phenomenon traditionally associated with chronic TLE. The classic traits inclu de: religiosity; hyposexuality; hypergraphia; and \200\230viscosity of personality \200 \231. Violence Also a controversial issue. There does seem to be an increased risk of violence an d aggression in people with TLE or frontal lobe epilepsy. \200\230Episodic dyscontrol \200 \231 is believed to be the result of sub-threshold kindling in these regions of the brain a nd anticonvulsants are often effective in reducing aggressive outbursts. The ecstatic seizures of Prince Myshkin He was thinking, incidentally, that there was a moment or two in his epileptic condition al most before the \201 t itself (if it occurred in waking hours) when suddenly amid the sad ness, spiritual darkness and depres- sion, his brain seemed to catch \201 re at brief mom ents His sensation of being alive and his awareness increased tenfold at those moment s which \202 ashed by like lightning. His mind and heart were \202 ooded by a dazzling light. All his agitation, doubts and worries, seemed composed in a twinkling, culminating i n a great calm, full of understanding but these moments, these glimmerings were still but a premonition of that \201 nal second (never more than a second) with which the seiz ure itself began. That second was, of course, unbearable. Dostoevsky: The Idiot 05_Semple_Chap05.indd 158 12/7/2012 3:28:35 PM This page intentionally left blank CHAPTER 5 Organic illness 160 HIV/AIDS and psychiatry 1 The HIV/AIDS epidemic means that, increasingly, psychiatrists are encoun- tering individuals with psychological and neuropsychiatric complications of HIV in fection. In some developing countries, rates of infection are as high as 20 \200\22330% (wi th rates in hospitals as high as 70 \200\22380%). Both diagnosis with the disease and the m ortality associated with it have major consequences for the psychological and social functi oning of individuals and their com- munities. In areas where large numbers of young adults are dying from the disease, there are even larger numbers of children becoming orphans, wit h all the developmental, emotional, and social problems associated with the loss of parenta l care and support. In other communities people with HIV/ AIDS are subject to stresses rela ted to their lifestyle choices (e.g. preju- dice related to sexual orientation or directed at those with addictions). Stigma also contributes to the psychological burden of infected individuals and their families. Improved outcome for patients on anti-retroviral therapy brings addi- tional stresses relat ed to living with uncertainty about the future. The responsibility of carers working with p atients with HIV/AIDS goes far beyond treating immediate physical problems. Holistic practi ce requires the health care professional to adopt a true biopsychosocial approach with appr eciation of the emotional state of the patient as well as the host of social, economic, spi ritual, and ethical challenges accompanying diagno- sis with the disease. Contexts in which psychiatric problems may arise There are a number of contexts in which ps ychiatric problems may arise in relation to HIV/AIDS: \200 The \200\230worried well \200\231 (i.e. HIV -ve people may be concerned about being infect ed due to contact with HIV +ve sources/individuals). Pre-test anxiety. Post-test stress may precipitate a psychiatric illness such as adjustment disorder, major depressive episode, a nd suicidality. Living with HIV/AIDS often results in stressful life events (e.g. losing a job, becoming economically disadvantaged, experiencing social alienation). In some cases, i ndividuals with psychiatric needs (e.g. victims of abuse; LD patients) may be more vulnerab le to becoming infected with the virus. HIV directly infects neurons in the brain causing n europsychiatric symptoms. HIV +ve individuals are susceptible to 2 and/or tumors of the CNS , which may manifest as neuropsychiatric symptoms. Anti-retroviral medications may cause ps ychiatric symptoms (e.g. zidovudine [AZT (azidothymidine)] may precipitate a major depressi ve episode, while isoniazid prophylaxis has been known to [AET (azidothymidine)] precipitat e a psychotic illness). \200 opportunistic infections 05_Semple_Chap05.indd 160 12/7/2012 3:28:35 PM HIV/AIDS AND PSYCHIATRY 1 161 Counseling HIV/AIDS patients \200 Pre-test counseling Consider: meaning of a +ve result; what actions the individual will tak e; con \201 dentiality issues; fears of individual; high- risk behaviors; reactions to str ess; social and other implications of +ve result. Post-test counseling to tell; discuss pre vention of transmission; offer support to individual and family. Clarify distortions; assess emotions; decide who Ethical issues \200 HIV testing test result will signi \201 cantly alter clinical manage ment. : encourage individual to tell sexual partner and other Con \201 dentiality medical personnel; if individual refuses, one may be obliged to inform without consent. Resource al location : issues of informed consent; only test without consent if a : e.g. availability of anti-retroviral drugs. 05_Semple_Chap05.indd 161 12/7/2012 3:28:36 PM CHAPTER 5 Organic illness 162 HIV/AIDS and psychiatry 2: clinical presentations Depression At least 30 \200\22350% of ind ividuals suffer a major depressive episode at some time following diagnosis. Clearly depres sion in this context has multiple causes and the challenge is to identify all the contribut ory fac- tors. Depressive illness should be differentiated from physical effects of HIV (we ight loss, loss of energy, etc.) as well as from HIV-associated dementia. Treatment: SSRIs, but problems of weight loss and headache; newer com- pounds such as nefaz odone and venlafaxine may be indicated. Suicide There is a 30x increased risk of suicide in individuals who are HIV +ve. High-risk times include: at diagnosis; at the death of an HIV +ve friend; as the individual experienc es deterioration in physical health. Mania Manic symptoms may develop in the context of HIV psychosis or as a result of treatmen t with anti-retroviral agents such as zidovudine (AZT). Treatment: Lithium is preferable (b eware risk of toxicity) since there is some evidence suggesting that sodium valproate may i ncrease viral repli- cation and reduce white cell count (WCC). Anxiety Infection with the virus is associated with increased risk of generalized anxiety d isorder (GAD), panic disorder, PTSD, and OCD. Chronic pain Up to 80% of patients experience chronic pain at some point, in particular chr onic headache. This may lead some individuals to self-medicate, putting them at risk of sub stance dependence. Delirium Delirium occurs in up to 30% of infected patients and is sometimes irre- versible. It is caused either by direct infection of the brain by the virus or by 2 infections and /or tumors. Delirium may be the initial manifestation of HIV-associated dementia. Psychosis A psychotic illness characterized by \202 uctuating symptoms that may alter ove r hours to days may occur in the context of HIV infection. Atypical bizarre psychotic sympt oms may give way to prominent mixed affective symptoms, which in turn may change to a withd rawn apathetic state. Aetiological factors include the effects of stress, medications, and 2 infections/tumors superimposed on the effects of direct infection of the brain by the virus. Thus psychosis i s a common early manifestation of 05_Semple_Chap05.indd 162 12/7/2012 3:28:36 PM HIV/AIDS AND PSYCHIATRY 2: CLINICAL PRESENTATIONS 163 subsequent HIV-associated dementia and it is likely that mild cognitive de \201 cits co-ex ist with the psychotic illness. Preferred treatment includes low-dose haloperidol or an atypical antipsychotic (e.g. olanza pine, quetiapine) \200\224due to increased sensitivity to extra-pyramidal side-effects. Ant i-retroviral agents such as zidovudine (AZT) may also reduce psychotic symptoms. HIV-associated dementia (HAD) Previously termed AIDS dementia, HAD is a relatively common o utcome in full-blown AIDS. Epidemiology 90% of AIDS patients have CNS changes post-mortem. 70 \200\22380% develop a co gnitive disorder. 30% develop HAD. Mean survival after diagnosis with HAD is 6 months. Pathology (a) Direct CNS infection: HIV is neurotropic, entering the brain through endothel ial gaps; the virus attaches to group 120 on CD4 +ve sites of microglial cells; a cascade o pens calcium channels leading to glutamate and nitrous oxide excitotoxicity; this results i n neuronal death and increased apoptosis. Sites include basal ganglia and subcortical and l imbic white matter. (b) Opportunistic infections/tumors: toxoplasmosis, papovavirus, cytome ga- lovirus (CMV), HSV, non-Hodgkin \200\231s lymphoma, and Kaposi sarcoma give rise to var iable neuropathology including encephalitis and focal necrosis. Clinical presentation \200 Early \200\230minor \200\231 cognitive disorder have very early CNS infection that is ofte n discounted as stress. Symptoms include cognitive slowing and memory de \201 cits as well as motor slowing and subtle incoordination. : with worsening of symptoms, HIV-associated d ementia (HAD) the clinical picture constitutes a dementia syndrome and is an AIDS-de \201 ning disorder. Clinical features are classi \201 ed as cognitive (subcortical dementia, fo cal cognitive de \201 cits, amnesia, mutism), motor (movement disorders, e.g. tremor, atax ia, choreo-athetosis, spasticity, myoclonus), and affective (depression, apathy, agitation, disinhibition, mania). Cognitive decline can be assessed using the HIV Dementia Scale and the Global Deterioration Scale of Reisberg (functional measure). : asymptomatic HIV +ve patients may Investigations CT/MRI: atrophy, iT2 signal. CSF: opportunistic infection, cytology, enzyme- linked immunosorbent assay (ELISA) +ve. EEG: general- ized slowing. Treatment Reverse transcriptase inhibitors (zidovudine [AZT]) delay HAD progression, while protease inhibitors reduce HIV load. 05_Semple_Chap05.indd 163 12/7/2012 3:28:36 PM CHAPTER 5 Organic illness 164 Neuropsychiatric aspects of CNS infections Viral encephalitis \200 Mumps , varicella-zoster, arbovirus, rubella \200\224may result in behavioral problems, lea rning dif \201 culties, and ADHD-like symptoms in children. HSV 1 \200\224involves inferi or frontal and anterior temporal lobes, resulting \200\224in the acute phase \200\224in del irium, hallucinations, and TLE. Chronic outcomes include Korsakoff psychosis, dementia, and Kluwer \200\223Bucy syndrome. EEG: slowing with bursts of increased slow- wave in the temp oral region. In \202 uenza (see b p. 167), characterized by Parkinsonism, oculogyric crise s, and psychosis. Epstein \200\223Barr virus/infectious mononucleosis encephalitis or chron ic fatigue syndrome. Measles slow virus infection. Features: behavioral problems, deteriora ting intellectual function, movement disorders (ataxia, myoclonus), seizures, and, \201 n ally, dementia in a child. Pathology: white and gray matter changes to occiput, cerebellum and basal ganglia. EEG: periodic complexes. \200\224a rare chronic outcome is encephalitis lethargica \200\224may result in myalgic \200\224rarely gives rise to subacute sclerosing panencephalitis, a Tuberculosis \200 TB meningitis exudate covers the base of the skull leading to vascular infarcts and hydrocephalus; cranial nerves may become involved. Psychiatric symptoms: apath y; withdrawal; insidious personality changes; delirium; hallucinations; chronic behavioral problems. Tuberculoma pressure (ICP). \200\224especially in children and young adults; caseating \200\224presents with focal signs, seizures, raised intracranial