SANS Juni 2013 Flashcards

1
Q

Kelainan batang otak yang paling umum apakah yang memerlukan biopsi stereotaktik dalam populasi dewasa ?

A. Metastasis
B. Inflamatory Lession
C. Infection
D. Glioma

A

D. Glioma

Glial neoplasm in its various forms, either low grade or high grade, is the most commonly encountered pathology that requires biopsy in the brain stem. According to the study by Kichingereder et al., approximately 76% of brain stem tumors will be gliomas, which has been found in other studies as well. Biopsies of inflammatory lesions appear to be relatively infrequent, which may be accounted for by its typical clinical and radiographic features. Infectious pathology of the brain stem is very uncommon. Interestingly, as opposed to all sites within the brain (cerebrum and cerebellum) where metastasis represent the most common cancerous lesion, in the brainstem it is 20 times less likely to occur.

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2
Q

Berapakah persentase mortalitas dari biopsi stereotaktik batang otak untuk kecurigaan keganasan batang otak ?

A. 5%
B. 3%
C. 1%
D. 7%
E. 9%
A

C. 1%

According to a recent meta-analysis conducted by Kickingereder et al. on 38 studies describing 1480 biopsy procedures for brain stem tumors, the mortality rate is 0.9%. In the past, the risk of brain stem biopsy was considered higher; however this percentage seems appropriate given the general risk of stereotactic biopsy which is 0.7-0.9% regardless of location. Therefore there appears to be no increased risk of mortality with stereotactic biopsy of the brainstem. However one must keep in mind that this is a highly select group of patients. There are few studies presently reporting 0% mortality. Permanent morbidity is approximately 2%.

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3
Q

Berapakah persentase kesuksesan dari biopsi stereotaktik batang otak untuk kecurigaan tumor ?

A. 70-84%
B. 55-69%
C. 40-54%
D. 85-99%

A

D. 85-99%

In the study by Kickingereder et al., meta-analysis on 1480 patients demonstrated that stereotactic biopsy of brain stem tumors yields a diagnosis (96.2%), and is associated with a low rate of procedure-related complications (overall morbidity: 7.8%, permanent morbidity:1.7%, mortality: 0.9%). Although the diagnostic yield of a brain biopsy varies from study to study, the success rate in brainstem stereotactic biopsies is disproportionately high.

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4
Q

Manakah dari tindakan-tindakan bedah untuk neuropaty ulnar pada siku lengan berikut ini yang secara konsisten telah terbukti lebih efektif dalam meta-analisis dan percobaan klinis ?

A. Subcutaneous anterior transposition
B. Decompression and Medical epicodylectomy
C. Simple Decompression
D. Submuscular anterior transposition
E. None
A

E. None

For ulnar neuropathy at the elbow, the current evidence reports no significant differences in effectiveness among the available types of surgeries. Therefore, simple ulnar decompression should be sufficient in most cases. To decompress the ulnar nerve at the elbow, a 3-centimeter incision is made posterior to the medial epicondyle, taking care to preserve the medial antebrachial cutaneous nerve. The ulnar nerve is identified proximally and Osborne’s ligament is divided. The elbow is then flexed to determine if the ulnar nerve subluxes anteriorly. If the ulnar nerve subluxes over the medial epicondyle, an anterior transposition procedure should be performed.

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5
Q

Manakah dari grup pasien dengan neuropaty ulnar pada siku lengan berikut ini, yang menunjukkan perbaikan paling signifikan pada outcome fungsi motorik dan sensorik, 12 bulan setelah dekompresi pada siku lengan ?

A. Severe Ulnar Neuropathy
B. Moderate Ulnar Neuropathy
C. Mild Ulnar Neuropathy
D. All Improve Equally

A

A. Severe Ulnar Neuropathy

Twelve months after ulnar decompression at the elbow, patients with severe ulnar neuropathy demonstrate the most significant absolute improvement in functional outcomes, both in the sensory and motor domains. According the multicenter outcomes study by Song and colleagues, by 3 months and beyond, sensation and motor scores for patients with severe ulnar neuropathy did not show a difference from patients with milder neuropathy. This is in sharp contrast to the differences in baseline sensory and motor examinations at baseline for patients with degrees of ulnar neuropathy. According to Delon (1993), mild ulnar neuropathy is defined by intermittent parasthesias with subjective weakness, moderate ulnar neuropathy is defined by intermittent parasthesias with measurable weakness, and severe ulnar neuropathy is defined by permanent parasthesias with palsy.

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6
Q

Setelah pasien mengalami dekompresi nervus ulnar pada siku lengan, parameter outcome manakah yang telah menunjukkan perbaikan klinis paling responsif ?

A. Carpal Tunnel Questionnaire
B. Ulnar Neuropathy at the Elbow Questionnaire (UNEQ)
C. Michigan Hand Questionnaire (MHQ)
D. 2-point discrimination test
E. SF-36
A

C. Michigan Hand Questionnaire (MHQ)

The satisfaction domain of the Michigan Hand Questionnaire (MHQ) demonstrated the strongest responsive to clinical change in the multicenter outcomes study by Song and colleagues. The Disabilities of the Arm, Shoulder, and Hand was also equally responsive. The Ulnar Neuropathy at the Elbow Questionnaire was evaluated by Mondelli and colleagues but was found to only have moderate responsive for assessing conservative management of ulnar neuropathy, but has not been rigorously evaluated in the surgical management of ulnar neuropathy. SF-36 is a general patient-outcome assessment tool but has not been specifically tested in the outcomes assessement of ulnar neuropathy. Finally, 2-point discimrination test is not a patient-reported outcomes tool.

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7
Q

Berapakah persentase dari vestibular schwannomas akan membesar dalam 5 tahun pada pasien NF2 yang diketahui ?

A. 60-75%
B. 45-60%
C. 30-45%
D. 75-90%

A

D. 75-90%

In a study by Peyre et al., 86% of vestibular schwannomas that were observed in NF2 patients grew. The mean length of clinical follow-up was 6.0 years (median: 4.9 years; range: 1.2 - 17.6 years) and the mean length of radiological followup was 4.2 years (median: 3.7 years; range: 1.2 - 11.6 years).This is in stark contrast to similar size tumors (here the mean was 1.6 cm) in those without NF2. In patients without NF2 it appears that tumors less than 2 cm that are observed grow 15% of the time if intracanulicular, and 30% if in the cistern. Although these numbers may be tempered by measurement technique there appears to be a difference in growth propensity between patients with NF2 and those without.

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8
Q

Faktor apakah yang dapat mempengaruhi vestibular schwannoma tumor growth rate pada pasien NF2 ?

A. Initial Main Tumor Diameter
B. Age at Diagnosis
C. Gender
D. Truncation Mutation

A

B. Age at Diagnosis

In the study Peyre et al., there was an inverse correlation between age at diagnosis and tumor growth rate. The study demonstrated that tumor growth rate was statistically higher in patients younger than 20 years at diagnosis of NF2 compared to those diagnosed at 20 years or older (2.6 mm/year vs. 0.9 mm/year, Mann-Whitney test, p < 0.0001). There was no correlation between initial mean tumor diameter and tumor growth rate (r2 =0.002, p=0.64). Mutation analysis was carried out and the growth rate of VS in patients with truncating mutations was not different from that of patients with non-truncating mutations (t-test, p>0.05).

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9
Q

Merlin adalah protein yang dipercaya tidak terdapat dalam NF2. Apakah fungsi oncogenetic primer dari Merlin?

A. Cell Cycle Control
B. Tumor Supressor
C. Growth Factor
D. None of the above

A

B. Tumor Supressor

Merlin is a tumor suppressor gene; it stands for Moesin-ezrin-radixin-like protein, also called schwannomin, and is a 69 kDa protein encoded by the NF2 gene. While the protein itself appears to be involved in cell to cell contact and adhesion, it also appears to have a suppressive role of the Ras-Raf-Mek system. Therefore it is functionally idenpendent of growth factor receptor mutations (such as EGFR in Glioma) or in Cell Cycle control or regulation unlike p53 or RB mutations.

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