NEURO FORM 4 by Sergio Angulo

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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

42
43
44
45
46
47
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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 with 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 virus (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)

Protein (mg/dL)

Total white blood cell count

(cells/microL)

<10

100 to
500

50 to 300

>1000

Viral meningitis

Bacterial or Early bacterial

meningitis
viral
meningitis
Bacterial
meningitis
Viral
meningitis
TB
meningitis

10 to 40

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)

Enterovirus

100-1000

None

NL/SL <160

Herpes simplex virus

~100

None
or

~100 or
higher

Epstein Barr virus

None

NL

Cytomegalovirus
Lymphocytic
choriomeningitis virus
Influenza

None

None

NL/SL NL/SL

NL/SL

None

NL

NL/SL

Viral diagnosis
Cell culture: NP, rectal, CSF; PCR:
CSF
Cell culture: skin or mucosal
lesion; PCR: CSF, blood
PCR: CSF
Serum serology
Cell culture and CSF PCR
Serology, cell culture and PCR
NP cell culture, antigen testing and
PCR

CSF PCR
Arboviruses
<200

Serum and CSF serology testing

Eastern equine encephalitis 400-4000
Western equine
2000
None NL
Cell culture: research laboratories
encephalitis (WEE)
West Nile virus
<200
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 prodrome 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 heart 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
proliferative diabetic retinopathy (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 further 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, leading
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

DM I
(Steinert's
disease)

DM II
(Proximal
myotonic
myopathy)

Very
common;
almost
universal
late in
course*

Common
(78% in
subjects
>50 years)

Cognitive
impairment;
Cardiac abnormalities
personality
disturbance
Conduction
disturbances well
recognized and
common late in
course*

Mental retardation
(congenital form)
is common; mild
to moderate
cognitive and
personality
Progressive
defects in adult
cardiomyopathy also form
described
Conduction
disturbances less
problematic than DM
I (19 percent when
considered across age Mild cognitive
spectrum [21 - >50]) impairment may
be seen
Progressive
cardiomyopathy also
described

Clinical Feature
Weakness

DM1* DM2, percent

Endocrine
disturbance
Glucose
intolerance: well
recognized and
common late in
course*
Hypogonadism:
common and
almost universal
late in course*
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
+++
Facial muscles
++
Hip flexion
+
Thumb/finger deep flexors +++
Ankle dorsiflexion
++
Shoulder abductors
++
Elbow extension
++
Myotonia on examination +++
Cataracts
++

75
12
64
55
16
20
31
75
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
In vitro studies have also shown that terbinafine inhibits CYP2D6-mediated metabolism.
This may be of clinical relevance for compounds predominantly metabolized by this
enzyme, such as tricyclic antidepressants, -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

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 contrary 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
o Schizophrenia
o Schizoaffective disorder
o Brief psychotic disorder
o Schizophreniform disorder
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, an
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 inflammatory, 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 whom 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 39C (102.2F),
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