NEURO FORM 4 by 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41
E E A B D A E E B E G G E C C E B B B E A C C A F E C C C D D K E B A D E A C
Sergio Angulo
42 43 44 45 46 47 48 49 50
C A C G A D A C A 1. 2. 3. 4.
E E A B. looks like a hernia
5. D. normal opening pressure: 10-18 cm H2O in supine glucose: 50-80 mg/dL protein: 15-40 mg/dL RBC: negative WBC: 0-3/mm3 The primary symptom of aneurysmal SAH is a sudden, severe headache (97 percent of cases) classically described as the "worst headache of my life" [ 5]. The headache is lateralized in 30 percent of patients, predominantly to the side of the aneurysm. Consistent with the rapid spread of blood, the symptoms of SAH typically begin abruptly. Hypertension, cigarette smoking and family history are among the most consistently observed risk factors
The onset of the headache may or may not be associated with a brief loss of consciousness, nausea or vomiting, and meningismus Meningismus and often lower back pain may not develop until several hours after the bleed since it is caused by the breakdown of blood products within the CSF, which lead to an aseptic meningitis [7]. While many patients have an altered level of consciousness, coma is unusual. Seizures occur during the first 24 hours in less than 10 percent of patients, but are a predictor of poor outcome [8]. SAH may also present as sudden death; at least 10 to 15 percent of patients die before reaching the hospital. The cornerstone of SAH diagnosis is the noncontrast head CT scan [20,21]. Clot is demonstrated in the subarachnoid space in 92 percent of cases if the scan is performed within 24 hours of the bleed [21,22]. Intracerebral extension is present in 20 to 40 percent of patients and intraventricular and subdural blood may be seen in 15 to 35 and 2 to 5 percent, respectively. The head CT scan should be performed with thin cuts through the base of the brain to increase the sensitivity to small amounts of blood. The sensitivity of head CT for detecting SAH is highest in the first 6 to 12 hours after SAH (nearly 100 percent) and then progressively declines over time to about 58 percent at day five. If the suspicion is high and the CT scan fails to show blood in subarachnoid space, a lumbar puncture must be obtained. An estimated 15 to 20 percent of patients with subarachnoid hemorrhage (SAH) are nonaneurysmal . The classic findings of SAH are an elevated opening pressure and an elevated red blood cell (RBC) count that does not diminish from CSF tube one to tube four. The differential of RBC between tubes one and four, and immediate centrifugation of the CSF can help differentiate bleeding in SAH from that due to a traumatic spinal tap. Xanthochromia (pink or yellow tint) represents hemoglobin degradation products.
Viral encephalitis: Patients with aseptic meningitis most commonly present wi th fever and headache with meningismus on examination. Patients may be lethargic but have a normal sensorium. By contrast, patients with encephalitis present with mental status changes. Patients with features of both may be considered to have a meningoencephalitis. Patients with encephalitis have an altered mental status ranging from subtle deficits to complete unresponsiveness. Symptoms and signs of meningeal irritation (photophobia and nuchal rigidity) are usually absent with a pure encephalitis but often accompany a meningoencephalitis. Seizures are common with encephalitis, and focal neurologic abnormalities can occur, including hemiparesis, cranial nerve palsies, and exaggerated deep tendon and/or pathologic reflexes. Patients may appear confused, agitated, or obtunded. The clinical presentation of aseptic meningitis is generally nonspecific, with fever, headache, nausea and vomiting, occasionally accompanied by photophobia and a stiff neck. Physical examination typically reveals signs of nuchal rigidity . Flaccid paralysis that evolves into an encephalitis strongly suggests the possibility of West Nile virus infection Results of imaging in patients with encephalitis may or may not demonstrate abnormal radiographic findings on CT or MRI modalities.
Examination of the cerebrospinal fluid (CSF), although not diagnostic, will usually confirm the presence of inflammatory disease of the CNS. The findings with aseptic meningitis and meningoencephalitis are generally indistinguishable (although rarely there may be few, if any, CSF abnormalities with a pure encephalitis). The following findings are characteristic of viral CNS infections :
6.
Increased white blood cell (WBC) count, but usually less than 250/mm3. The differential shows a predominance of lymphocytes , although early infection may reveal a predominance of neutrophils. In the latter setting, a repeat CSF cell count eight hours later will generally show a shift from neutrophils to lymphocytes [29]. Elevated protein concentration , but usually less than 150 mg/dL. Usually normal glucose concentration (>50 percent of blood value ), but moderately reduced values are occasionally seen with HSV, mumps, or some enteroviruses. Red cells are usually absent (in a nontraumatic tap); their presence in the appropriate clinical setting suggests HSV-1 infection or other necrotizing encephalitides.
A. Neonates may have an abrupt onset of fever accompanied by nonspecific symptoms (eg, poor feeding, vomiting, diarrhea, rash, respiratory symptoms). Neurologic manifestations may be minimal, ranging from no symptoms, to irritability and lethargy, to frank nuchal rigidity or bulging fontanelle [1,6,8]. Central nervous system (CNS) disease may progress to encephalitis with seizures and/or focal neurologic findings [9]. Neonates are at increased risk for severe systemic disease, particularly with herpes simplex vi rus (HSV). Systemic manifestations may include pneumonia, hepatitis with necrosis, myocarditis, and necrotizing enterocolitis [9]. Disseminated intravascular coagulation and other findings of sepsis can mimic overwhelming bacterial infection. Clinical manifestations of neonatal HSV CNS disease include seizures (focal or generalized), lethargy, irritability, tremors, poor feeding, temperature instability, and full anterior fontanel [28,30,31]. Early in the course of HSV CNS disease, none of these signs or symptoms may be apparent. In the absence of vesicles, the initial presentation of HSV CNS disease may be indistinguishable from other causes of neonatal sepsis or meningitis [11,12]. Many experts recommend evaluation for HSV CNS disease with HSV deoxyribonucleic acid (DNA) polymerase chain reaction (PCR) and other CSF studies (ie, cell counts, protein, and glucose), and empiric treatment with acyclovir in all neonates with aseptic meningitis or other signs and symptoms of meningoencephalitis without an obvious bacterial cause Early intravenous administration of glucocorticoids (usually dexamethasone) has been evaluated as adjuvant therapy in an attempt to diminish the rate of hearing loss and other neurologic complications as well as mortality in selected patients with bacterial meningitis. The effects of dexamethasone on viral meningitis are not fully known; very few studies have examined the long-term outcome of children with viral meningitis who may have received dexamethasone at the time of presentation when bacterial meningitis was a consideration
Glucose (mg/dL) <10 ¶
10 to 40Δ
Protein (mg/dL)
Total white blood cell count (cells/microL)
100 to ◊ 500
50 to 300§
>1000
Viral meningitis
Bacterial or Early bacterial meningitis viral Bacterial meningitis meningitis Viral meningitis TB meningitis
Bacterial Bacterial Bacterial Nervous system More common meningitis meningitis meningitis Lyme disease (neuroborreliosis)
100 to 1000 5 to 100
Neurosyphilis
Neurosyphilis
TB meningitis¥
TB meningitis
Pathogen
WBC RBC (cells/mm3)
Glucose Protein (mg/dL) (mg/dL)
Viral diagnosis
Enterovirus
100-1000
None
NL/SL↓ <160
Cell culture: NP, rectal, CSF; PCR: CSF
Herpes simplex virus
~100
None or ↑
~
~100 or higher
Cell culture: skin or mucosal lesion; PCR: CSF, blood
PCR: CSF Epstein Barr virus
↑
None
NL
↑
Serum serology Cytomegalovirus
↑
None
↓
Lymphocytic choriomeningitis virus
↑
None
NL/SL↓ NL/SL↑
Influenza
NL/SL↑
None
NL
↑
NL/SL↑
Cell culture and CSF PCR Serology, cell culture and PCR NP cell culture, antigen testing and PCR CSF PCR
Arboviruses
<200
↑
Eastern equine encephalitis 400-4000 Western equine encephalitis (WEE)
≤2000
West Nile virus
<200
None
NL
Serum and CSF serology testing Cell culture: research laboratories
Up to 900
WEE virus: PCR: CSF St. Louis encephalitis virus <200 ~200 NL: normal; SL: slightly; NP: nasopharyngeal aspirate; CSF: cerebrospinal fluid; PCR: polymerase chain reaction; ↓: decrease; ↑: increase.
7. E. Cognitive findings in patients with hepatic encephalopathy vary from subtle deficits that are not apparent without specialized testing (minimal hepatic encephalopathy), to more overt findings, with impairments in attention, reaction time, and working memory Patients with severe hepatic encephalopathy may progress to hepatic coma. Neuromuscular impairments include bradykinesia, hyperreflexia, rigidity, myoclonus, and asterixis. Disturbances in the diurnal sleep pattern (insomnia and hypersomnia) are common initial manifestations of hepatic encephalopathy and typically precede other mental status changes or neuromuscular symptoms. As hepatic encephalopathy progresses, patients may develop mood changes (euphoria or depression), disorientation, inappropriate behavior, somnolence, confusion, and unconsciousness. Neuromuscular impairment in patients with overt hepatic encephalopathy includes bradykinesia, asterixis (flapping motions of outstretched, dorsiflexed hands), slurred speech, ataxia, hyperactive deep tendon reflexes, and nystagmus. Less commonly, patients develop loss of reflexes, transient decerebrate posturing, and coma.
8. E 9. B 10. E 11. G 12. G 13. E 14. C 15. C Carotid sinus hypersensitivity. Hypersensitivity of the afferent or efferent limbs of the carotid sinus reflex arc results in vagal activation and/or sympathetic inhibition, which leads to bradycardia and/or vasodilation; this is also called the carotid sinus syndrome or carotid sinus syncope.
Carotid sinus syncope is similar to vasovagal (neurocardiogenic) syncope since both are forms of reflex syncope reflecting alterations in autonomic tone with similar clinical manifestations. However, precipitating factors for these two types of syncope differ and carotid sinus hypersensitivity tends to occur in older patients. A history of syncope following accidental manipulation of the carotid sinuses should be sought although it is rarely present. Absence of such a history does not exclude carotid sinus syndrome.
Vasovagal syncope: Patients with vasovagal syncope are most commonly young and otherwise healthy . Typical triggers and premonitory symptoms are strongly suggestive of vasovagal syncope, although these may be absent or difficult to correlate to the syncopal episode. Women and patients younger than 40 are more likely to have typical symptoms [25]. However, older patients are also frequently diagnosed with vasovagal syncope [26]. Older individuals have specific triggers that may be absent in younger individuals (ie, micturition, cough, defecation, deglutition). Vasovagal syncope (“classical”) refers to syncope triggered by emotional or orthostatic stress such as venipuncture (experienced or witnessed), painful or noxious stimuli, fear of bodily injury, prolonged standing, heat exposure, or exertion
Vasovagal syncope is often associated with a pr odrome and persistence of nausea, pallor, and diaphoresis, consistent with increased vagal tone. Syncope is typically of short duration and occurs in the sitting or standing position. The supine position restores adequate blood flow to the brain. However, full recovery may be delayed, as the patient may feel depressed or fatigued. This course may help distinguish vasovagal syncope from syncope associated with arrhythmia, which is typically of abrupt onset and of short duration. Loss of consciousness may be prolonged with some other causes of syncope, such as seizures and aortic stenosis, but rarely with vasovagal syncope.
16. E Vascular dementia Patients with cognitive impairment and clinical or radiologic evidence of cerebrovascular pathology should be screened and treated for vascular
risk factors, especially hypertension, although this may be more helpful in preventing rather than ameliorating dementia. Antiplatelet agents — While of established efficacy in secondary stroke prevention, no randomized trials have found a benefit for aspirin or other antiplatelet therapy in the prevention or treatment of VaD [39]. The Aspirin for Asymptomatic Atherosclerosis (AAA) trial randomly assigned 3350 participants (50 to 75 years) to aspirin or placebo, but after five years of follow-up, there was no difference in cognitive test scores between the groups [40]. Other trials comparing antiplatelet agents to each other have also found no benefit on cognitive function or recurrent lacunar stroke Evidence that specific treatments with acetylcholinesterase inhibitors and/or memantine are helpful in VaD is not conclusive. However, it is reasonable to use them in patients with suspected VaD because of the high prevalence of comorbid Alzheimer disease (AD) and the difficulty of reliably distinguishing between the primary etiologic entities. (See 'Disease modifying therapy' above.) A typical regimen aimed to improve symptoms in VaD is donepezil 10 mg/day plus memantine 20 mg/day; however, there are no data supporting the use of one acetylcholinesterase inhibitor over another. Cortical syndrome — In primarily cortical VaD, cognitive features are specific to the areas affected [106]:
Medial frontal: executive dysfunction, abulia, or apathy. Bilateral medial frontal lobe infarction may cause akinetic mutism. Left parietal: aphasia, apraxia, or agnosia. Right parietal: hemineglect (anosognosia, asomatognosia), confusion, agitation, visuospatial and constructional difficulty. Medial temporal: anterograde amnesia.
Cortical branch occlusions are often caused by embolism from the hea rt or large arteries and may present with clinical stroke. However, when the superior division of the middle cerebral artery is not involved, hemiparesis may not be an obvious signal that stroke has occurred. Onset may appear more insidious as a result, and it is not uncommon for the patient to improve again before the next event. The course is thus often perceived as fluctuating or stepwise. As few as one-third of patients with multi-infarct dementia (MID) experience both an abrupt onset and stepwise deterioration [107].
Subcortical syndrome — In subcortical pathology, both lacunar infarctions and chronic ischemia affect the deep cerebral nuclei and white matter pathways. These often disrupt frontal lobe and other cortico-cortico circuits, producing deficits attributable to remote brain areas [96,106,108,109]. Characteristic features include:
Focal motor signs Early presence of gait disturbance (marche a petit pas or magnetic, apraxic gait or Parkinsonian gait) History of unsteadiness and frequent, unprovoked falls Early urinary frequency, urgency, and other urinary symptoms not explained by urologic disease Pseudobulbar palsy Personality and mood changes, abulia, apathy, depression, emotional incontinence [110] Cognitive disorder characterized by relatively mild memory deficit, psychomotor retardation, and abnormal executive function [111]
The course of subcortical VaD may be gradual or stepwise and either slow or fast in decline. MRI will show the fundamental hallmarks of VaD including cortical and subcortical infarctions as well as the presence of subcortical ischemic changes or leukoaraiosis. However, radiographic criteria alone have been shown to be inadequate at differentiating between poststroke patients with and without dementia. 17. B Migraine prophylaxis. In the absence of contraindications, we prefer amitriptyline, one of the beta blockers (metoprolol, propranolol, or timolol), or topiramate for initial treatment . For women of childbearing age, reasonable options include verapamil or flunarizine. In this group, valproate is problematic because it is teratogenic and is associated with an increased risk of birth defects. However, it can be considered for women utilizing effective contraception if other options are not effective or tolerated. 18. B 19. None
20. B 21. E Arterial TOS — Symptoms of arterial compression are the least common type of thoracic outlet syndrome, accounting for only about 1 percent of cases. Symptoms develop spontaneously unrelated to work or trauma [1]. Arterial TOS (aTOS) is almost always associated with a cervical rib or anomalous rib. It occurs in young patients without typical atherosclerotic risk factors distinguishing it from peripheral artery disease. (See "Overview of upper extremity peripheral artery disease".) Hand ischemia with symptoms of pain, pallor, paresthesia, and coldness is the most common presentation. Magnetic resonance – Contrast-enhanced magnetic resonance (MR) angiography using provocative arm positioning can allow excellent imaging to the vessels and can be a useful diagnostic tool.
22. A Multiple sclerosis affects more women than men; the estimated female-to-male ratio of MS incidence is approximately 2:1, with some data suggesting the ratio is even higher. The median and mean ages of MS onset are 23.5 and 30 years of age, respectively. The peak age of onset is about five years earlier for women than for men. Onset of MS can rarely occur as late as the seventh decade. (See 'Epidemiology and risk factors' above.)
Genetic factors appear to contribute to the risk of MS, especially variation involving the HLADRB1 locus. (See 'Genetic susceptibility' above.) Although many viruses, and particularly the Epstein-Barr virus, have been associated with MS, there is no specific evidence linking viruses directly to the development of MS. (See 'Viral infections' above.) The incidence and prevalence of MS vary geographically. The incidence and prevalence of MS varies geographically [112,113]. High frequency areas of the world (prevalence of 60 per 100,000 or more) include all of Europe (including Russia), southern Canada, northern United States, New Zealand, and southeast Australia.. This geographic variance was previously thought to be explained in part by racial differences; white populations, especially those from Northern Europe, appeared to be most susceptible, while people of Asian, African, or American Indian origin appeared to have the lowest risk, with other groups intermediate. However, subsequent studies in the United States demonstrated an increased incidence of MS in black adults [114,115] and children [116], suggesting that this racial susceptibility may be changing There is an inverse relationship between sun exposure, ultraviolet radiation exposure, or serum vitamin D levels, and the risk or prevalence of MS. (See 'Sunlight and vitamin D' above.) There is no association between vaccines and the risk of MS.
23. C Myophosphorylase deficiency (McArdle disease, glycogen storage disease V [GSD V]) usually presents in adolescence or early adulthood with exercise intolerance, fatigue, myalgia, cramps, poor endurance, muscle swelling, and fixed weakness [1,4]. Typical laboratory findings include myoglobinuria and elevated creatinine kinase (CK). The presentation is somewhat different in older adults and very young children. Older patients may present with progressive weakness without history of cramps or myoglobinuria. 24. C
Diabetic retinopathy. Each year in the United States, diabetic retinopathy accounts for 12% of all new cases of blindness. It is also the leading cause of blindness for people aged 20 to 64 years. [5]. Even macular edema, which can cause rapid vision loss, may not have any warning signs for some time. In general, however, a person with macular edema is likely to have blurred vision, making it hard to do things like read or drive. In some cases, the vision will get better or worse during the day. In the first stage which is called non-proliferative diabetic retinopathy (NPDR) there are no symptoms, the signs are not visible to the eye and patients will have 20/20 vision. The only way to detect NPDR is by fundus photography, in which microaneurysms (microscopic blood-filled bulges in the artery walls) can be seen. If there is reduced vision, fluorescein angiography can be done to see the back of the eye. Narrowing or blocked retinal blood vessels can be seen clearly and this is called retinal ischemia (lack of blood flow). Macular edema in which blood vessels leak their contents into the macular region can occur at any stage of NPDR. The symptoms of macular edema are blurred vision and darkened or distorted images that are not the same in both eyes. Ten percent (10%) of diabetic patients will have vision loss related to macular edema. Optical Coherence Tomography can show the areas of retinal thickening (due to fluid accumulation) of macular edema. [6] In the second stage, abnormal new blood vessels (neovascularisation) form at the back of the eye as part of p r o l i f er a t i v e d i a b e ti c r e t i n o p a t h y (PDR); these can burst and bleed (vitreous hemorrhage) and blur the vision, because these new blood vessels are fragile. The first time this bleeding occurs, it may not be very severe. In most cases, it will leave just a few specks of blood, or spots floating in a person's visual field, though the spots often go away after a few hours. These spots are often followed within a few days or weeks by a much greater leakage of blood, which blurs the vision. In extreme cases, a person may only be able to tell light from dark in that eye. It may take the blood anywhere from a few days to months or even years to clear from the inside of the eye, and in some cases the blood will not clear. These types of large hemorrhages tend to happen more than once, often during sleep.
Age-related macular degeneration (AMD) is a degenerative disease of the central portion of the retina (the macula) that results primarily in loss of central vision. Central vision is required for activities such as driving, reading, watching television, and performing activities of daily living. AMD is classified as dry (atrophic) or wet (neovascular or exudative) for clinical purposes. Different classifications and grading schemes have been used in epidemiologic and therapeutic studies of AMD. Many epidemiologic studies make a distinction between age-related maculopathy (ARM) and age-related macular degeneration (AMD). All wet lesions are AMD, but early dry lesions that do not reduce vision may be classified as ARM rather than AMD. A furt her source of confusion is that AMD is often abbreviated as ARMD. The finding of either large soft drusen or RPE pigmentary clumping increases the risk of wet AMD. Wet AMD is characterized by growth of abnormal vessels into the subretinal space, usually from the choroidal circulation and less frequently from the retinal circulation [13]. These abnormal blood vessels leak, leadi ng to collections of subretinal fluid and/or blood beneath the retina (figure 1 and picture 4 and picture 5). Wet type AMD is also referred to as choroidal neovascularization. Wet AMD is more common than dry AMD among patients with advanced AMD [14]. Although wet AMD is found in only 10 to 15 percent of patients with AMD, wet AMD accounts for more than 80 percent of cases with severe visual loss or legal blindness [10]. In contrast to dry AMD, in which vision loss is slow and gradual, wet AMD is characterized by rapid distortion and loss of central vision over a period of weeks to months.
In wet AMD, dilated examination may reveal subretinal fluid and/or hemorrhage (picture 4). Neovascularization appears as a grayish-green discoloration in the macular area (picture 5). The presence of subretinal hemorrhage or a gray subretinal membrane is strongly suggestive of a subretinal choroidal membrane. These patients require an office-based fluorescein angiogram delineate and
characterize the neovascular membrane and optical coherence tomography to identify the presence of subretinal fluid or retinal edema.
25. A 26. F 27. E 28. C
Aneurysms of the posterior communicating artery are the third most common circle of Willis aneurysm [1] (the most common are anterior communicating artery aneurysms) and can lead to oculomotor nerve palsy. Aneurysms of the anterior communicating artery are the most common circle of Willis aneurysm [1] and can cause visual field defects such as bitemporal heteronymous hemianopsia (due to compression of the optic chiasm),[2] psychopathology and frontal lobe pathology. [3] 29. C 30. C REM sleep behavior disorder (RBD) is another nocturnal disorder commonly seen in patients with PD [96]. This disorder is characterized by vigorous movements that are related to increased muscle tone during REM sleep [97]. Patients with RBD often act out their dreams and exhibit vocalizations as well as flailing, kicking, and punching motions of the
limbs. Some patients may injure themselves or their bed partners. Behaviors related to RBD are reported to occur in 15 to 47 percent of patients with PD [96,98,99], and over three-quarters of spontaneous RBD cases eventually develop PD or other alpha-synucleinopathies, often years after the onset of RBD. Polysomnography is necessary for definitive diagnosis of RBD and to exclude other sleep disorders that can mimic RBD. 31. D Myotonic dystrophy type 1 (DM1) and type 2 (DM2) are similar in that both are multisystem disorders characterized by skeletal muscle weakness and myotonia, cardiac conduction abnormalities, cataracts, testicular failure, hypogammaglobulinemia, and insulin resistance. However, DM2 is generally a less severe disease than DM1. In addition, there are congenital, juvenile, and adult onset forms of DM1, whereas adult onset (typically in the fourth decade) is the most common presentation for DM2 [10,26]. Nevertheless, there is a wide range of symptom onset in DM2 for myotonia (range 13 to 67 years, median 30) and weakness (range 18 to 66 years, median 41) Premature, male-pattern frontal balding is seen in both DM1 and DM2.
Type of DM Cataracts
Cognitive impairment; Cardiac abnormalities personality disturbance
Glucose Mental retardation intolerance: well (congenital form) recognized and is common; mild common late in course* to moderate cognitive and personality Hypogonadism: Progressive defects in adult common and cardiomyopathy also form almost universal described late in course* Conduction disturbances well recognized and common late in course*
DM I (Steinert's disease)
Very common; almost universal late in course*
DM II (Proximal myotonic myopathy)
Conduction disturbances less problematic than DM Common I (19 percent when Mild cognitive considered across age (78% in impairment may ¶ spectrum [21 - >50] ) subjects be seen >50 years) ¶ Progressive cardiomyopathy also described
Clinical Feature Weakness
Endocrine disturbance
DM1* DM2, percent
Glucose intolerance: common, seen in 75 percent ¶ Hypogonadism: ¶ seen in 29 percent
Gastrointestinal disorder
Irritable bowel symptoms; dysphagia; gall stones
Yes, but not as pronounced as in DM1; dysphagia common but relatively mild
Neck flexion
+++
75
Facial muscles
++
12
Hip flexion
+
64
Thumb/finger deep flexors +++
55
Ankle dorsiflexion
++
16
Shoulder abductors
++
20
Elbow extension
++
31
Myotonia on examination +++
75
Cataracts
60
++
Spinal ms athrophy disorders are characterized by degeneration of the anterior horn cells in the spinal cord and motor nuclei in the lower brainstem. These diseases are classified as types 1 through 4 depending upon the age of onset and clinical course. Patients with all forms of SMA have diffuse symmetric proximal muscle weakness that is greater in the lower than upper limbs and absent or markedly decreased deep tendon reflexes Facioscapulohumeral muscular dystrophy (FSHD) is the third most common type of muscular dystrophy. It is a complex genetic disorder characterized in most cases by slowly progressive muscle weakness involving the facial, scapular, upper arm, lower leg, and hip girdle muscles, usually with asymmetric involvement. The age of symptom onset varies from infancy to middle age, but is usually in the second decade. By age 20 years, findings are seen in approximately 90 percent of affected patients [4], although some or all of the signs may be subclinical [26]. Progression is usually slow with a normal or near-normal life span. 32. D studies have also shown that terbinafine inhibits CYP2D6-mediated metabolism. This may be of clinical relevance for compo unds predominantly metabolized by this enzyme, such as tricyclic antidepressants, ß-blockers, selective serotonin reuptake inhibitors (SSRIs), and monoamine oxidase inhibitors (MAO-Is) Type B, if they have a narrow therapeutic window. Epileptic seizures are the most important
In vitro
adverse effect of bupropion. Bupropion acts as an norepinephrine-dopamine reuptake inhibitor (NDRI), and it serves as an atypical antidepressant different from most commonly prescribed antidepressants such as selective serotonin reuptake inhibitors (SSRIs). The only problem with this is that Bupropion is also an inhibitor of CYP2D6. Bupropion is metabolized by CYP2B6.
The core feature of catatonia is a motor disturbance in which patients are unable to move normally despite full physical capacity in the limbs and trunk [1]. The disturbance can range from marked reduction in movements to marked agitation. Starting, stopping, and planning movement can be impaired, and motor behavior may be repetitive, purposeless, impervious to external stimuli, and con trary to intent [11]. Although catatonia can occur in the context of many mental disorders, it is most often found in [2,39-42]:
Bipolar I disorder Bipolar II disorder Unipolar major depression (major depressive disorder) Psychotic disorders Schizophrenia o Schizoaffective disorder o Brief psychotic disorder o Schizophreniform disorder o Autism spectrum disorder Delirium
33. K
The superficial branch of the radial nerve passes along the front of the radial side of the forearm to the commencement of its lower third. It is a sensory nerve. It lies at first slightly lateral to the radial artery, concealed beneath the Brachioradialis. In the middle third of the forearm, it lies behind the same muscle, close to the lateral side of the artery. It quits the artery about 7 cm. above the wrist, passes beneath the tendon of the Brachioradialis, and, piercing the deep fascia, divides into two branches: lateral and medial.
34. E? This one is very confusing
Side effects, especially with higher doses, include dizziness, drowsiness, fatigue, diarrhea, unusual dreams, ataxia, trouble sleeping, depression, and vision problems. It may also reduce blood flow to the hands and feet, causing them to feel numb and cold; smoking may worsen this effect. [16] Due to the high penetration across the blood-brain barrier, lipophilic beta blockers such as propranolol and metoprolol are more likely than other less lipophilic beta blockers to cause sleep disturbances such as insomnia and vivid dreams and nightmares.[17] Serious side effects that are advised to be reported immediately
include symptoms of bradycardia (resting heart rate slower than 60 beats per minute), persistent symptoms of dizziness, fainting and unusual fatigue, bluish discoloration of the fingers and toes, numbness/tingling/swelling of the hands or feet, sexual dysfunction, erectile dysfunction (impotence), hair loss, mental/mood changes, depression, trouble breathing, cough, dyslipidemia, and increased thirst. Taking it with alcohol might cause mild body rashes, so is not recommended. 35. B 36. None 37. A 38. D 39. E 40. A 41. C Toxoplasma encephalitis — Toxoplasma encephalitis (TE) represents reactivation disease from prior infection. Affected patients present with fever, headache, altered mental status, and focal neurologic complaints or seizures. Supporting laboratory findings include the presence of Toxoplasma antibodies, which is consistent with past exposure, and advanced immunosuppression with CD4 counts <100 cells/microL. (See "Toxoplasmosis in HIV-infected patients".) TE lesions are generally multiple and are localized in the parietal or frontal lobes, in the thalamus or basal ganglia, or at the cortico-medullary junction [10]. Ring enhancement is present in approximately 90 percent and surrounding edema with mass effect is often seen
The neuroradiologic characteristics of TE are not pathognomonic and may be observed in other conditions, particularly lymphoma. If a single lesion is seen, a n MRI should be obtained to determine whether the lesion is truly solitary. Although single lesions can be seen in TE infection, solitary large (>4 cm) lesions are more suspicious for primary CNS lymphoma.
The initial therapy of choice for TE consists of the combination of pyrimethamine plus sulfadiazine plus leucovorin (AI) (203--206 ). Pyrimethamine penetrates the brain parenchyma efficiently even in the absence of inflammation ( 207 ). Use of leucovorin reduces the likelihood of the hematologic toxicities associated with pyrimethamine therapy (208,209). The preferred alternative regimen for patients with TE who are unable to tolerate or who fail to respond to first-line therapy is pyrimethamine plus clindamycin plus leucovorin (AI) (203,204).
Corticosteroid therapy should be considered in two settings: ●When substantial mass effect can be demonstrated on imaging and the mental status is significantly depressed. Such patients are at risk for cerebral herniation. ●When the diagnosis of PCNSL has already been established, since steroids can cause false negative results on a subsequent brain biopsy in patients with lymphoma.
42. C 43. A Adverse effects associated with anticholinergic use in older adults include memory impairment, confusion, hallucinations, dry mouth, blurred vision, constipation, nausea, urinary retention, impaired sweating, and tachycardia 44. C Lumbosacral radiculopathy is often extremely painful. Analgesic medications such as nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen and activity modification are the mainstay of treatment. Physical therapy is often tried for patients with mild to moderate persistent symptoms, but evidence of effectiveness is lacking. The utility of systemic glucocorticoids and epidural glucocorticoids is limited. For imaging of the lumbar spine, MRI, CT, and CT myelography (CT scan after intrathecal administration of contrast media) are equally sensitive for the diagnosis of disc herniation [34]. For routine initial assessment, an MRI (image 1 and image 2 and image 3) is more informative than CT because it can also identify other intraspinal pathologies, including inflammator y, malignant, and vascular disorders. In addition, MRI is not associated with ionizing radiation and is less invasive than CT myelography. For patients with persistent or severe findings in who m the etiology is not confirmed on neuroimaging, we suggest electromyography and nerve conduction studies
45. G 46. A 47. D 48. A 49. C In patients who do not respond to nonpharmacologic therapy and correction of iron deficiency, we recommend pharmacologic treatment with a dopamine agonist (Ropirinole,, pramipexole) or an alpha-2-delta calcium channel ligand Pregabalin, gabapentin) as first-line therapy (table 2). These classes of drugs have been shown to be effective compared with placebo in multiple randomized controlled trials 50. A
EXTRA Qs
A 72-year-old man is brought to the emergency department because of a decreased level of consciousness for the past 6 hours. Three days ago, he had fever, shortness of breath, and productive cough treated with an antibiotic, but his symptoms did not improve. On arrival, his temperature is 39°C (102.2°F), pulse is 110/min, respirations are 28/min, and blood pressure is 110/75 mm Hg. Breath sounds are decreased over the right midlung field. On neurologic examination, he is unarousable but responds to tactile stimuli by moaning. Cranial nerves are intact. There is resistance to passive flexion of the neck. Which of the following is the most likely pathogen? A) Herpes simplex virus 1B) Listeria monocytogenesC) Pseudomonas aeruginosa D) Streptococcus pneumoniae E) Toxoplasma gondii
A 72-year-old man with a 3-year history of Parkinson disease is brought to the physician by his wife for a follow-up examination. Three weeks ago, his dosage of carbidopa-levodopa was increased. Since then, he has reported seeing people spying on him from across the street. He has no other history of serious medical or psychiatric illness and takes no other medications. Physical examination shows a resting tremor. There is cogwheel rigidity and increased muscle tone. On mental
status examination, he reports seeing people spying on him and says his wife is "one of them." Addition of which of the following medications is the most appropriate next step in pharmacotherapy? A) Haloperidol B) Lorazepam C) Paroxetine D) Quetiapine E) Valproic acid
