Respuestas Examen Neurologia 1

RESPUESTAS EXAMEN NEUROLOGIA
Respuesta No 1
Item 16 Answer: (D) Acute demyelinating polyneuropathy

Educational Objective: Recognize the most common peripheral neurologic manifestations
of HIV infection.
The peripheral nervous system is affected in about 30% of patients with HIV infection. The most
common peripheral neuropathy is a distal, symmetric, predominantly sensory polyneuropathy.
Mild motor manifestations may also occur. The decreased reflexes and mild sensorimotor
peripheral manifestations noted in this patient suggest a peripheral nerve process. The lack of
an acute or subacute onset would argue against the acute inflammatory demyelinating
polyradiculoneuropathy resembling Guillain-Barré syndrome that can also occur in patients with
HIV infection. Myopathy can occur in patients with HIV infection for several reasons, such as
primary infection or medication use, and presents with painless progressive proximal weakness.
Reflexes are usually maintained. HIV-associated cognitive-motor complex is the most common
complication of HIV-1 infection and is characterized by a progressive dementing illness that
occurs in the later stages of the disease. Damage to the spinal cord that resembles subacute
combined degeneration may also occur and is characterized by progressive ataxia and lower
extremity spasticity accompanied by marked proprioceptive loss.
Bibliography
1. Elovaara I, Iivanainen M, Valle SL, Suni J, Tervo T, Lahdevirta J. CSF protein and
cellular profiles in various stages of HIV infection related to neurological manifestations. J
Neurol Sci. 1987;78:331-42. PubMed Link
2. Fuller GN, Jacobs JM, Guiloff RJ. Nature and incidence of peripheral nerve syndromes in
HIV infection. J Neurol Neurosurg Psychiatry. 1993;56:372-81. PubMed Link
Respuesta pregunta No 2
Item 18 Answer: (B) MRI scan with MR venography and MR angiography

Educational Objective: Recognize the clinical presentation and differential diagnosis of
“thunderclap” headache, and identify the most appropriate management options.
Because of the potential for catastrophic underlying intracranial disorders, the presentation of a
“thunderclap” (abrupt, split-second onset) headache always demands a thorough evaluation,
despite this patient’s history of headaches. Although her current headache resembles a
migraine attack, the onset was distinctly abrupt and unusual for this patient. In a headache
evaluation, the most important question to ask or ascertain is how the presenting headache
compares with previous headaches, especially with respect to acuity or suddenness of onset. A
diagnosis of “crash” migraine or idiopathic thunderclap headache should therefore never be
made without appropriate investigations.
This presentation is compatible with subarachnoid hemorrhage; however, the probability of

detecting subarachnoid blood on a CT scan more than 12 hours but less than 24 hours after the
development of a subarachnoid hemorrhage exceeds 95%, and this patient’s CT scan was
normal. Since xanthochromia cannot be detected by the human eye in about 50% of patients,
the detection of blood breakdown products by spectrophotomety is the most sensitive method
(100% sensitivity) 12 hours after a subarachnoid hemorrhage. However, this patient’s
spectrophotometric examination did not show blood in the cerebrospinal fluid.
Cerebral venous sinus thrombosis is now a well-recognized cause of acute thunderclap

headache. Headache, papilledema, focal neurologic symptoms, seizures, and mental status
changes are the most common features at presentation. This patient had signs of increased
intracranial pressure, including early papilledema and an elevated opening cerebrospinal fluid
pressure. She is also on an oral contraceptive agent, which may be associated with an
increased risk for venous thrombosis. CT with and without contrast detects only about 20% of
cerebral venous sinus thromboses. However, MRI with MR venography is highly sensitive for
detecting this entity and should be done prior to catheter angiography, particularly for this
indication and when the likelihood of a ruptured cerebral aneurysm is so low. If MRI and MR
venography are not immediately available, the patient should be transferred to a facility where
these studies can be performed. MR angiography will detect aneurysms as small as 5 mm and
is an appropriate screening procedure for this patient.
Bibliography
1. Vermeulen M, van Gijn J. The diagnosis of subarachnoid -haemorrhage. J Neurol
Neurosurg Psychiatry. 1990;53:365-72. PubMed Link
2. de Bruijn SF, Stam J, Kappelle LJ. for the CVST Study Group. Thunderclap headache as
first symptom of cerebral venous sinus thrombosis. CVST Study Group. Lancet.
1996;348:1623-5. PubMed Link
Respuesta Pregunta No 3
Item 19 Answer: (B) Mitochondrial myopathy

Educational Objective: Recognize the clinical presentation and differential diagnosis of
mitochondrial myopathy.
This patient presents with the typical clinical features of a mitochondrial myopathy. In many
adults, the major clinical manifestation is progressive external ophthalmoplegia that is
symmetric and develops over many years. In addition, patients have ptosis and proximal muscle
weakness. Patients may develop diabetes mellitus, retinitis pigmentosa, epilepsy,
hypothyroidism, cardiomyopathy, and heart block. When these features develop in childhood or
adolescence, the term Kearns-Sayre syndrome is used. Although patients with Becker’s
muscular dystrophy or fascioscapular muscular dystrophy have progressive muscular
weakness, they do not develop the progressive external ophthalmoplegia that is typical of
mitochondrial myopathy.
Myotonic dystrophy is an autosomal dominant multisystemic disease characterized by myotonia,

which is prolongation of a muscle contraction. Patients usually describe a long-lasting difficulty
in letting go of a door knob or a handshake. Ptosis with temporal and masseter muscle wasting
and facial muscle involvement produces a characteristic facial appearance. Extramuscular
features include cataracts, frontal balding, diabetes mellitus, testicular atrophy, and cardiac
conduction defects. Patients with polymyositis have proximal muscle weakness and tenderness
but do not have facial or ocular muscle involvement. The serum creatine kinase level is often
markedly elevated in patients with polymyositis.
Bibliography
1. Simon DK, Johns DR. Mitochondrial disorders: clinical and genetic features. Annu Rev
Med. 1999;50:111-27. PubMed Link
2. Chinnery PF, Turnbull DM. Clinical features, investigation, and management of patients
with defects of mitochondrial DNA. J Neurol Neurosurg Psychiatry. 1997;63:559-63.
PubMed Link
3. Jones KJ, North KN. External ophthalmoplegia in neuromuscular disorders: case report and
review of the literature. Neuromuscul Disord. 1997;7:143-51. PubMed Link
Item 21 Answer: (C) Carbamazepine
Educational Objective: Understand the management of a patient with complex partial seizures.
Complex partial seizures typically present with brief episodes (typically about 90 seconds) of
transient unawareness of surroundings and automatisms. The seizures can begin at any age.
They uncommonly occur more than once each day and often are preceded by an aura and are
followed by postical confusion. Appropriate anticonvulsant management should be initiated.
Carbamazepine is an appropriate initial agent. Phenytoin may also be effective but may cause
cosmetic side effects, including gingival hyperplasia, hirsutism, and coarsening of facial
features. Valproic acid should be considered as a second-line agent along with gabapentin.
Ethosuximide is the initial management choice for absence seizures but is not effective for
complex partial seizures. Gabapentin is approved only as an add-on agent for patients with
recalcitrant seizures. Felbamate is typically not used as a first-line agent because of possible
increased risk of hepatic failure and bone marrow toxicity.
Bibliography
1. Mosewich RK, So EL. A clinical approach to the classification of seizures and epileptic
syndromes. Mayo Clin Proc. 1996; 71:405-14. PubMed Link
2. Britton JW, So EL. Selection of antiepileptic drugs: a practical approach. Mayo Clin Proc.
1996;71:778-86. PubMed Link
Item 22 Answer: (A) Multiple areas of increased T2 signal in the cortical and

subcortical regions on MRI scan of the brain
Educational Objective: Diagnose the neurologic manifestations of systemic lupus erythematosus
and recognize other neurologic manifestations of connective tissue disorders.
Central nervous system involvement occurs in approximately 50% of patients with systemic
lupus erythematosus. Manifestations include behavioral changes, such as dementia, psychosis,
and depression. Other neurologic symptoms, including chorea and seizures, may be noted.
Focal neurologic findings may include hemiparesis, unsteadiness of gait, and brainstem
findings, such as cranial neuropathy. Peripheral nerves may be involved and may cause either a
sensorimotor peripheral neuropathy or mononeuritis multiplex. Inflammatory myopathy may also
occur. In patients with central nervous system involvement, the MRI scan shows cortical and
subcortical areas of increased T2 signal. The cerebral arteriogram is often normal. True
vasculitis is typically not found at biopsy or on autopsy. Antinuclear antibody positivity is usually
present. Neurologic syndromes associated with an elevated rheumatoid factor typically involve
the peripheral nervous system. Likewise, rheumatoid arthritis may be associated with a
myelopathy caused by atlantoaxial subluxation.
Patients with systemic lupus erythematosus may also present with stroke caused by a
hypercoagulable state related to lupus anticoagulant positivity or antiphospholipid antibody
positivity. Embolization from Libman-Sacks endocarditis may also cause a cerebral infarction.
Bibliography
1. West SG. Lupus and the central nervous system. Curr Opin Rheumatol. 1996;8:408-14.
PubMed Link

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RESPUESTAS EXAMEN NEUROLOGIA

Item 16 Answer: (D) Acute demyelinating polyneuropathy

Item 18 Answer: (B) MRI scan with MR venography and MR angiography

This presentation is compatible with subarachnoid hemorrhage; however, the probability of

Cerebral venous sinus thrombosis is now a well-recognized cause of acute thunderclap

Item 19 Answer: (B) Mitochondrial myopathy

Myotonic dystrophy is an autosomal dominant multisystemic disease characterized by myotonia,

Item 22 Answer: (A) Multiple areas of increased T2 signal in the cortical and

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