INBR 7 - Neurology Flashcards Preview

UNAIR - INBR 7 > INBR 7 - Neurology > Flashcards

Flashcards in INBR 7 - Neurology Deck (175)
Loading flashcards...
1

1. Sindroma HORNER dan hipestesi wajah
A. Sindroma TOLOSA - HUNT
B. Sindroma GRANDENIGO
C. Sindroma RAEDER

(C)
Raeder's para trigeminal neuralgia is often localized adjacent to the trigeminal nerve as it courses through the middle cranial fossa. The cause of this syndrome is often unclear, but it is usually characterized by a partial Horner's syndrome and unilateral trigeminal nerve problems, including tic-like pain, numbness, and/or masseter weakness. Gradenigo's syndrome, also known as apical petrositis, often consists of the classic triad of abducens nerve palsy, retroorbital pain, and a draining ear. Tolosa-Hunt syndrome is a diagnosis of exclusion; this condition is believed to result from inflammation adjacent to the superior orbital fissure. It is characterized by painful ophthalmoplegia, cranial nerves III, IV, and VI palsies, and recurrent attacks and remissions; it is typically treated with intravenous steroids (Greenberg, pp. 581-582 )

2

2. Nyeri retro-orbital dan paralise saraf keenam
A. Sindroma TOLOSA - HUNT
B. Sindroma GRANDENIGO
C. Sindroma RAEDER

(B)
Raeder's para trigeminal neuralgia is often localized adjacent to the trigeminal nerve as it courses through the middle cranial fossa. The cause of this syndrome is often unclear, but it is usually characterized by a partial Horner's syndrome and unilateral trigeminal nerve problems, including tic-like pain, numbness, and/or masseter weakness. Gradenigo's syndrome, also known as apical petrositis, often consists of the classic triad of abducens nerve palsy, retroorbital pain, and a draining ear. Tolosa-Hunt syndrome is a diagnosis of exclusion; this condition is believed to result from inflammation adjacent to the superior orbital fissure. It is characterized by painful ophthalmoplegia, cranial nerves III, IV, and VI palsies, and recurrent attacks and remissions; it is typically treated with intravenous steroids (Greenberg, pp. 581-582 )

3

3. Optalmoplegia yang terasa sangat nyeri disertai paralise saraf ketiga, keempat dan kelima
A. Sindroma TOLOSA - HUNT
B. Sindroma GRANDENIGO
C. Sindroma RAEDER

(A)
Raeder's para trigeminal neuralgia is often localized adjacent to the trigeminal nerve as it courses through the middle cranial fossa. The cause of this syndrome is often unclear, but it is usually characterized by a partial Horner's syndrome and unilateral trigeminal nerve problems, including tic-like pain, numbness, and/or masseter weakness. Gradenigo's syndrome, also known as apical petrositis, often consists of the classic triad of abducens nerve palsy, retroorbital pain, and a draining ear. Tolosa-Hunt syndrome is a diagnosis of exclusion; this condition is believed to result from inflammation adjacent to the superior orbital fissure. It is characterized by painful ophthalmoplegia, cranial nerves III, IV, and VI palsies, and recurrent attacks and remissions; it is typically treated with intravenous steroids (Greenberg, pp. 581-582 )

4

4. Seorang laki-laki berusia 56 tahun dengan riwayat merokok dan hipertensi dibawa kepada seorang neurolog dengan kelainan vaskular yang melibatkan bagian kranial dari nucleus ambiguus. Problem klinis manakah yang seringkali dapat dihindari pada kelainan ini ?
A. Paralisis palatal
B. Paralisis paringeal
C. Paralisis laryngeal
D. Bukan salah satu dari A sampai dengan C
E. A, B dan C semuanya benar

C .
Lesions within the nucleus ambiguus may occur as a result of vascular insults, tumors, syringobulbia, motor neuron disease, and inflammatory disease. Lesions in this location often result in palatal, pharyngeal, and laryngeal paralysis that is often associated with other adjacent cranial nerve and brainstem abnormalities. If the cephalad portion of the nucleus ambiguus is injured, however, laryngeal function is often spared due to the somatotopic organization of this motor nucleus. This is referred to as "palatopharyngeal paralysis of Avellis" (Brazis, pp. 321-322 ) .

5

5. Kelemahan muskulus trapezius dan sternokleidomastoideus ipsilateral, disponia dan dispagia, hilang rasa pengecap pada sepertiga belakang dari lidah, dan berkurangnya sensasi pada faring merupakan ciri-ciri khas sindroma:
A. Collet-Sicard
B. Vernet
C. Schmidt
D. Garcin
E. Weber

B.
The spinal accessory nerve enters the j ugular foramen accompanied by cranial nerves IX and X. Lesions of the jugular foramen including tumors, infections, and fractures can result in Verner's syndrome, which is characterized by ipsilateral trapezius and sternocleidomastoid muscle weakness, dysphonia and dysphagia, loss of taste over the posterior third of the tongue, and depressed sensation over the pharynx (Brazis, pp. 330-331).

6

6. Seorang anak berusia 7 tahun dibawa ke ruang gawat darurat dengan gejala terus tertawa tanpa dapat dikendalikan (kejang gelastik) dan pubertas prekok. Pemeriksaan MRI otak bisa menunjukkan lokasi kelainan dimana?
A. Amigdala
B. Hipokampus
C. Girus singulate
D. Hipotalamus
E. Sella turcica

D.
An .MRI study of the brain in a patient with seizures accompanied by involuntary laughter (gelastic seizures) that alternates with crying or sobbing spells would likely show a lesion in the hypothalamus. A small series of cases hm•e been reported in the literature to date describing this phenomenon; in one report, 4 of 16 patients were found to harbor a hypothalamic hemartoma. Gelastic seizures or laughing fits have been reported to occur in up to 2 1 % of patients with hypothalamic hemartomas. Most patients with this lesion present with isosexual precocious puberty by the age of 3 years, although patients as old as 8 years have been reported. Hypothalamic hemartomas may be associated with midline deformities such as callosal agenesis, optic malformations, and hemispheric dysgenesis ( Kaye and Laws, pp. 593-596).

7

Seorang pekerja bangunan, 52 tahun mencatat beberapa kelemahan pada tangannya saat dia
bekerja, yang diikuti oleh semakin parahnya masalah bicara dan menelan beberapa bulan kemudian. Meski pun kelemahannya adalah letih dan gatal extrenitas atas & bawah, pemeriksaan fisik tidak mencatat adanya mati rasa atau gangguan panca indera. Lengan, kaki,
dan lidahnya mengalami faskikulasia dan atropi yang jelas. serta gangguan refleks hiperaktif dan tanda-tanda Babinski
7. Diagnosis yang paling mungkin pada laki-laki usia pertengahan ini adalah sebagai berikut:
A. Mielopati servikal
B. Sklerosis majemuk
C. Miastenia Gravis
D. Sindroma GUILLAIN-BARRE
E. Sklerosis lateral amiotropik

E
In this patient , the multiple motor deficits and signs of anterior horn cell disease unaccompanied by any sensory abnormalities suggest a diagnosis of amyotrophic lateral sclerosis (ALS) . Most individuals with ALS die withir1 5 years of symptom onset, especially if there are both upper and lower motor neuron signs. There is a very high incidence of this disease among the Chamorro Indians of Guam, and there is also an association between ALS and a Parkinsonlike dementia complex (Merritt, pp. 7 10-7 12) .

8

Seorang pekerja bangunan, 52 tahun mencatat beberapa kelemahan pada tangannya saat dia
bekerja, yang diikuti oleh semakin parahnya masalah bicara dan menelan beberapa bulan kemudian. Meski pun kelemahannya adalah letih dan gatal extrenitas atas & bawah, pemeriksaan fisik tidak mencatat adanya mati rasa atau gangguan panca indera. Lengan, kaki,
dan lidahnya mengalami faskikulasia dan atropi yang jelas. serta gangguan refleks hiperaktif dan tanda-tanda Babinski
8. Apa prognosisnya?
A. Relatif baik jika kadar gula di dalam darah dinaikkan
B. Seringkali pasien akan sembuh dalam jangka waktu yang lama dengan perawatan plasmaperesis dan steroid
C. Baik sekali jika diberikan medikasi antikolinergik
D. Pembedahan dapat membantu mencegah gejalanya menyebar menjadi neurologik
E. Seringkali akan menjadi fatal dalam jangka waktu 3 – 5 tahun

E
In this patient , the multiple motor deficits and signs of anterior horn cell disease unaccompanied by any sensory abnormalities suggest a diagnosis of amyotrophic lateral sclerosis (ALS) . Most individuals with ALS die withir1 5 years of symptom onset, especially if there are both upper and lower motor neuron signs. There is a very high incidence of this disease among the Chamorro Indians of Guam, and there is also an association between ALS and a Parkinsonlike dementia complex (Merritt, pp. 7 10-7 12) .

9

Seorang pekerja bangunan, 52 tahun mencatat beberapa kelemahan pada tangannya saat dia
bekerja, yang diikuti oleh semakin parahnya masalah bicara dan menelan beberapa bulan kemudian. Meski pun kelemahannya adalah letih dan gatal extrenitas atas & bawah, pemeriksaan fisik tidak mencatat adanya mati rasa atau gangguan panca indera. Lengan, kaki,
dan lidahnya mengalami faskikulasia dan atropi yang jelas. serta gangguan refleks hiperaktif dan tanda-tanda Babinski
9. Penyakit ini bisa berasosiasi dengan penyakit atau kelainan apa?
A. Kejang-kejang bayi
B. Sindroma KLUVER-BUCY
C. Parkinson
D. Sindroma Down
E. Degenerasi sistem saraf otonom

C
In this patient , the multiple motor deficits and signs of anterior horn cell disease unaccompanied by any sensory abnormalities suggest a diagnosis of amyotrophic lateral sclerosis (ALS) . Most individuals with ALS die withir1 5 years of symptom onset, especially if there are both upper and lower motor neuron signs. There is a very high incidence of this disease among the Chamorro Indians of Guam, and there is also an association between ALS and a Parkinsonlike dementia complex (Merritt, pp. 7 10-7 12) .

10

10. Mutisme akinetik merujuk pada keadaan, dimana pasien meskipun tampak bangun, tetapi tetap tak bergerak dan diam. Keadaan umumnya dikaitkan dengan lesi yang melibatkan seluruh struktur di bawah ini, KECUALI
A. Hipotalamus
B. Sistem aktivasi dopaminergik menanjak
C. Girus singulate
D. Talamus
E. Nucleus raphe

E
Animal studies and case reports have disclosed two primary lesion sites that

11

11. Semua di bawah ini adalah karakteristik meningitis tuberculosa, KECUALI
A. Tingkat kematiannya seringkali lebih tinggi dibandingkan dengan meningitis bakterial
B. Perawatan awalnya meliputi isoniazid, rifampin, pirazinamid, dan etambutol
C. Jika perawatannya dimulai sejak dini, maka perawatan ini efektif dalam mencegah hasil yang buruk pada mayoritas pasien
D. Fokus utama infeksi tuberculosis kemungkinan berasal dari daerah di luar otak.
E. Meningitis bakterial ditandai oleh meningitis basal, sementara pada meningitis, basis pada otak relatif bebas infeksi.

E.
Tuberculous meningitis is always secondary to an infection elsewhere in the body, especially the lungs. It differs from infections caused by other common bacteria in that the time course is often more protracted, the mortality rate is higher, and the CSF changes may not be very helpful or diagnostic initially. Tuberculous meningitis is also often characterized by marked basal meningitis, as . opposed to bacterial meningitis, which tends to produce a n;eningeal reaction over the convexities of the brain. The diagnosis is often established by isolating the organism from the CSF. CSF findings include slightly increased pressure, moderate pleocytosis of 25 to 500 cells/mm3 with lymphocytic predominance, increased protein content, decreased glucose values in the range of 20 to 40 mg/dL, and the absence of growth on routine CSF culture media. The natural course of the disease is death within 6 to 8 weeks if left untreated. With early diagnosis and treatment, the recovery rate approaches 90%. Treatment is commonly started with four drugs , including isoniazid, rifampin, pyrazinamide, and ethambutol; streptomycin is an alternative in case one of the agents cannot be used. The drug regimen can be modified later, once sensiti\ •ities of the mycobacterium are known, but are typically administered for 18 to 24 months (Merritt, pp. 108-111).

12

12. Sindrom GERSTMANN meliputi semua hal di bawah ini, KECUALI
A. Agnosia jari
B. Akalkulia
C. Agrafia
D. Kebingungan kanan dan kiri
E. Anosognia

E.
Gerstmann's syndrome can result from injury in the dominant inferior parietal lobule (angular and supramarginal gyri) and is characterized by confusion of the right and left limbs, difficulty in distinguishing the fingers on the hand (finger agnosia) , acalculia, and agraphia. Anosognosia, which is characterized by unawareness of the opposite side of the body, often results from lesions in the nondominant • parietal lobe and is not a feature of Gerstmann's syndrome (Carpenter, p. 429).

13

13. Cacad biokimiawi pada penyakit REFSUM telah dipastikan sebagai kekurangan enzim apa?
A. Arilsulfatase A
B. Pitanoil-koenzim A hidroksilase
C. β-glukosilase
D. α-galaktosidase
E. Asam seramidase

B.
Refsum disease is an autosomal recessive disease caused by a deficiency of phytanoyl-coenzyme A hydroxylase and accumulation of phytanic acid in the body. This disease is unique among the lipidoses because phytanic acid is not synthesized in the body but is obtained exclusively from the diet. Limiting phytanic acid or its precursor, phytol (dairy products, ruminant fat, and chlorophyl-containing foods), from the diet reduces plasma phytanic acid levels. Plasmapheresis may further help eliminate phytanic acid from the body in seYere cases. Symptoms typically begin in childhood but in some patients may be delayed until the fifth decade. Night blindness typically appears first, followed by limb weakness and gait abnormalities. Some patients may develop psychiatric symptoms, peripheral neuropathy, pigmentary retinopathy, deafness, cataracts, bone deformities, or cardiac arrhythmias (Merritt, pp. 514-529 , 539-540 ) .

14

Seorang perempuan berusia 65 tahun didiagnosis skizofrenia paranoid dan menjalani rawat inap. Perawatan dimulai dengan pemberian risperidon dosis rendah (Risperdal). Beberapa hari sebelum masuk perawatan rumah sakit ini, dia mengeluh karena sakit perut yang semakin lama semakin parah. Hasil pemeriksaan menunjukkan tidak terdapatnya kejang perut, tapi ditemukan adanya demam, leukositosis, diare, hipertensi dan tacikardia. Pemeriksaan melalui pencitraan otak dan rongga perut tidak begitu mencolok.
14. Diagnosis manakah yang paling mungkin?
A. Gangguan konversi
B. Skizofrenia dengan fitur-fitur depresif
C. Poripiria intermiten akut
D. Appendisitis
E. Sindroma infuse dengan picuan riperidon

C
The symptoms o f acute intermittent porphyria (AlP) are most commonly gastrointestinal, psychiatric, and neurologic. The precise etiology remains uncertain, but large and small nerve fibers, as well as autonomic nerves, have been shown to be affected. Abdominal pain is the most common finding, but often occurs concomitantly with a psychiatric or neurologic disorder. There is usually no abdominal rigidity, but fever, leukocytosis, diarrhea, tachycardia, and hypertension are often evident. Appendicitis or other serious abdominal problems may be difficult to rule out; in fact, some patients have been subjected to laparotomy for abdominal exploration. The most reliable test to confirm AlP is the assay for porphobilinogen deaminase activity in red blood cells, but often a good clinical histor

15

Seorang perempuan berusia 65 tahun didiagnosis skizofrenia paranoid dan menjalani rawat inap. Perawatan dimulai dengan pemberian risperidon dosis rendah (Risperdal). Beberapa hari sebelum masuk perawatan rumah sakit ini, dia mengeluh karena sakit perut yang semakin lama semakin parah. Hasil pemeriksaan menunjukkan tidak terdapatnya kejang perut, tapi ditemukan adanya demam, leukositosis, diare, hipertensi dan tacikardia. Pemeriksaan melalui pencitraan otak dan rongga perut tidak begitu mencolok.
15. Uji atau intervensi yang paling dapat diandalkan untuk menguatkan diagnosis ini meliputi:
A. Sintase asam aminolevulinik
B. Uji sintetase homokistein
C. Uji aktivitas deaminase porpobilinogen
D. seliotomi eksploratoris
E. Diskriminasi medikasi antipsikotik dengan resolusi gejala

C
The symptoms o f acute intermittent porphyria (AlP) are most commonly gastrointestinal, psychiatric, and neurologic. The precise etiology remains uncertain, but large and small nerve fibers, as well as autonomic nerves, have been shown to be affected. Abdominal pain is the most common finding, but often occurs concomitantly with a psychiatric or neurologic disorder. There is usually no abdominal rigidity, but fever, leukocytosis, diarrhea, tachycardia, and hypertension are often evident. Appendicitis or other serious abdominal problems may be difficult to rule out; in fact, some patients have been subjected to laparotomy for abdominal exploration. The most reliable test to confirm AlP is the assay for porphobilinogen deaminase activity in red blood cells, but often a good clinical histor

16

Seorang perempuan berusia 65 tahun didiagnosis skizofrenia paranoid dan menjalani rawat inap. Perawatan dimulai dengan pemberian risperidon dosis rendah (Risperdal). Beberapa hari sebelum masuk perawatan rumah sakit ini, dia mengeluh karena sakit perut yang semakin lama semakin parah. Hasil pemeriksaan menunjukkan tidak terdapatnya kejang perut, tapi ditemukan adanya demam, leukositosis, diare, hipertensi dan tacikardia. Pemeriksaan melalui pencitraan otak dan rongga perut tidak begitu mencolok.
16. Sakit perutnya dapat segera sembuh secara dramatis dengan obat yang mana?
A. Propranolol
B. Barbiturat
C. Sulfonamid
D. Gabapentin
E. Merkuri

A
The symptoms o f acute intermittent porphyria (AlP) are most commonly gastrointestinal, psychiatric, and neurologic. The precise etiology remains uncertain, but large and small nerve fibers, as well as autonomic nerves, have been shown to be affected. Abdominal pain is the most common finding, but often occurs concomitantly with a psychiatric or neurologic disorder. There is usually no abdominal rigidity, but fever, leukocytosis, diarrhea, tachycardia, and hypertension are often evident. Appendicitis or other serious abdominal problems may be difficult to rule out; in fact, some patients have been subjected to laparotomy for abdominal exploration. The most reliable test to confirm AlP is the assay for porphobilinogen deaminase activity in red blood cells, but often a good clinical histor

17

17. Hal tersebut di bawah ini merupakan karakteristik neuropati dipteritis
(Diphtheritic Neuropathy), KECUALI
A. Organisme yang diisolasi dari laring dan paring seringkali adalah Corynebacterium diptheriae
B. Orgaisme-organisme seringkali melepaskan endotoksin yang dapat menyebabkan miokarditis atau neuropati asimetris
C. Neuropati seringkali dimulai dengan gangguan pada fungsi penglihatan
D. Dipteri dan neuropati yang berkaitan dengannya dapat dicegah dengan imunisasi
E. Seringkali menyebabkan neuropati demielinasi

B.
Diphtheria infection produces neuropathy in about 20% of infected patients. Corynebacterium diphtheriae is often isolated from the throat and releases an exotoxin (not endotoxin) that can cause myocarditis or symmetric neuropathy. The neuropathy is often characterized by poor visual accommodation, paresis of. throat muscles, quadriparesis, and slow ner;e conduction velocities secondary to demyelinating neuropathy. This disease can be prevented by immunization and often responds to antibiotics (Merritt, pp. 620-621).

18

18. Luka psikosis dari Korsakoff seringkali melibatkan struktur otak yang mana?
A. Nucleus dorsomedial (DM) dari thalamus
B. Nukleus globose
C. Amigdala
D. Vermis dari serebelum
E. Girus dentate

A.
Although \\-ernicke's and Korsakoff's syndromes are often described together, these appear to be two distinct entities that result from thiamine deficiency. Wernicke's syndrome consists of mental symptoms (global confusional state) , eye movement problems (nystagmus, lateral rectus palsy, and lateral gaze palsy), and gait ataxia, while Korsakoff's syndrome is a purely amnestic syndrome usually associated with lesions in the DM nucleus of the thalamus and mammillary bodies. With treatment (thiamine) , ocular abnormalities, nystagmus, and global confusion often improve to varying degrees, leaYing Korsakoff's amnesia in about 80% of patients. ( Merritt, pp. 924-925).

19

19. Di antara ciri klinis di bawah ini, ciri klinis manakah yang merupakan ciri klinis neuroborreliosis?
A. Radikulitis sensori yang terasa sangat sakit dan muncul sekitar 3 minggu setelah eritema migrans
B. Mononeuropati kranial
C. Paresis anggota badan
D. Arthalgia
E. Vertigo

A.
Lyme disease is caused b

20

20. Diantara gangguan di bawah ini, manakah yang BUKAN merupakan kelainan DNA mitokondrial?
A. Miopati mitokondrial, enselopalopati, asidosis laktik, dan episode-episode mirip stroke (MELAS)
B. Epilepsi mioklonik dengan serat-serat merah (MERRF)
C. Neuropati optik bawaan dari Leber (LHON)
D. Sindrom KEARNS-SAYRE (KSS)
E. Penyakit LEIGH

E.
MELAS, MERFF, LHON, and KSS are a group of disorders related to mitochondrial (mt) DNA abnormalities. Although specific syndromes are often identified by a variety of signs/symptoms, several clinical manifestations seem to be prevalent with mtDNA abnormalities and include short stature, hearing loss, and diabetes mellitus. Lactic acidosis is the most common laboratory finding, while pathologic sectioning reveals enlarged mi tochondria in muscle fibers, which forms the basis for ragged red fibers (RRF) . Leigh's disease is a disorder of mitochondrial metabolism in which the primary defect involves proteins encoded by nuclear DNA instead of mitochondrial DNA (El l ison, pp. 457-465) .

21

Tn. X, 74 tahun dengan penyakit vaskular peripheral dibawa ke ruang gawat darurat dengan lengan kiri terus terayun-ayun tanpa terkendali. Pencitraan resonansi magnetik (MRI) atas otak mengungkapkan adanya ventrikulomegali, atropi kortikal difusi, dan infark lakunar multiple

21. Diantara struktur-struktur di bawah ini, struktur manakah yang paling mungkin terkena sehingga menunjukkan ciri ini?
A. Globus pallidus lateral
B. Nukleus subtalamik
C. Talamus
D. Nukleus merah
E. Nukleus caudate

B
Wild, involuntary flinging of an extremity may be secondary to a cerebrovascular accident affecting the subthalamic nucleus and is commonly referred to as hemiballismus. Normally, glutamatergic projections from the subthalamic nucleus to the Gpi and SNr suppress the motor nuclei of the thalamus. After damage to the subthalamic nucleus, the thalamic motor neurons are disinhibited and provide excessive activation of the motor cortex. The dopaminergic projections, howeYer, remain intact. pro,•iding a constant and unbalanced acti,•ation of motor neurons throughout the basal ganglia. This is the basis for using neuroleptic agents, including dopamine receptor blockers (haloperidol and perphenazine) and presynaptic dopamine depletors (reserpine and tetrabenazine ) to treat this disease process (Tarsy, p. 9; Pritchard, pp. 330-331).

22

Tn. X, 74 tahun dengan penyakit vaskular peripheral dibawa ke ruang gawat darurat dengan lengan kiri terus terayun-ayun tanpa terkendali. Pencitraan resonansi magnetik (MRI) atas otak mengungkapkan adanya ventrikulomegali, atropi kortikal difusi, dan infark lakunar multiple

22. Di antara kelainan neurotransmitter di bawah ini, kelainan neurotransmitter manakah yang paling mungkin diderita pasien yang bersangkutan?
A. Dopamin
B. Glutamat
C. GABA
D. Norepineprin
E. Asetilkolin

B
Wild, involuntary flinging of an extremity may be secondary to a cerebrovascular accident affecting the subthalamic nucleus and is commonly referred to as hemiballismus. Normally, glutamatergic projections from the subthalamic nucleus to the Gpi and SNr suppress the motor nuclei of the thalamus. After damage to the subthalamic nucleus, the thalamic motor neurons are disinhibited and provide excessive activation of the motor cortex. The dopaminergic projections, howeYer, remain intact. pro,•iding a constant and unbalanced acti,•ation of motor neurons throughout the basal ganglia. This is the basis for using neuroleptic agents, including dopamine receptor blockers (haloperidol and perphenazine) and presynaptic dopamine depletors (reserpine and tetrabenazine ) to treat this disease process (Tarsy, p. 9; Pritchard, pp. 330-331).

23

Tn. X, 74 tahun dengan penyakit vaskular peripheral dibawa ke ruang gawat darurat dengan lengan kiri terus terayun-ayun tanpa terkendali. Pencitraan resonansi magnetik (MRI) atas otak mengungkapkan adanya ventrikulomegali, atropi kortikal difusi, dan infark lakunar multiple

23. Perawatannya bisa mencakup semua perawatan di bawah ini, KECUALI
A. Haloperidol
B. Perpenazin
C. Reserpin
D. Tetrabenazin
E. L-DOPA

E
Wild, involuntary flinging of an extremity may be secondary to a cerebrovascular accident affecting the subthalamic nucleus and is commonly referred to as hemiballismus. Normally, glutamatergic projections from the subthalamic nucleus to the Gpi and SNr suppress the motor nuclei of the thalamus. After damage to the subthalamic nucleus, the thalamic motor neurons are disinhibited and provide excessive activation of the motor cortex. The dopaminergic projections, howeYer, remain intact. pro,•iding a constant and unbalanced acti,•ation of motor neurons throughout the basal ganglia. This is the basis for using neuroleptic agents, including dopamine receptor blockers (haloperidol and perphenazine) and presynaptic dopamine depletors (reserpine and tetrabenazine ) to treat this disease process (Tarsy, p. 9; Pritchard, pp. 330-331).

24

24. Diantara pernyataan-pernyataan mengenai penyakit Huntington di bawah ini, pernyataan manakah yang paling benar?
A. Merupakan gangguan berulang trinukeotida (CAG) yang menumpuk pada kromosom 4
B. Merupakan kondisi resesif autosomal dengan penetrasi tidak tuntas
C. Terutama merupakan penyakit yang menyerang proyeksi-proyeksi GABA/enkepalin dari amigdala ke stratum
D. Lebih lazim di kalangan perempuan dibandingkan dengan kalangan laki-laki
E. Memiliki onset lebih lambat pada generasi-generasi sesudahnya

A
HD is an autosomal dominant condition with complete penetrance that Yaries in symptom onset from ju,•eniles to late adulthood. \Yith a\•erage onset between 35 and 40 years of age. Sporadic cases of HD are rare. HD, a CHAPTER 3 Neurology Answers 67 trinucleotide repeat (CAG) disorder that localizes to chromosome 4, is more common in males than females and exhibits anticipation (earlier onset in successive generations). The abnormal gene product results in protein conformational changes that lead to aggregation in the cytosol and eventually cellular apoptosis. HD primarily affects the GABNenkephalin projections from the striatum to the external segment of the globus pallidus (indirect pathway) , resulting in thalamic facilitation of motor cortical areas and hyperkinesia. Haloperidol may be effective in suppressing abnormal movements early in the disease course, but the disease is inevitably progressive and usually results in death within 20 years from symptom onset (Merritt, pp. 659-664, 696-699).

25

25. Tengkorak cloverleaf-shape
A. Trigonosepalus
B. Skaposepalus
C. Plagiosepalus
D. Brakisepalus
E. Oksisepalus
F. Kleebattschädel
G. Bukan, A -F

G
Craniosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally flattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491-492) .

26

26. Tengkorak tower
A. Trigonosepalus
B. Skaposepalus
C. Plagiosepalus
D. Brakisepalus
E. Oksisepalus
F. Kleebattschädel
G. Bukan, A -F

E
Craniosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally flattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491-492) .

27

27. Malformasi Chiarri
A. Trigonosepalus
B. Skaposepalus
C. Plagiosepalus
D. Brakisepalus
E. Oksisepalus
F. Kleebattschädel
G. Bukan, A -F

F
Craniosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally flattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491-492) .

28

28. Sinostosis metopik
A. Trigonosepalus
B. Skaposepalus
C. Plagiosepalus
D. Brakisepalus
E. Oksisepalus
F. Kleebattschädel
G. Bukan, A -F

A
Craniosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally flattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491-492) .

29

29. Kepala datar pada satu sisi
A. Trigonosepalus
B. Skaposepalus
C. Plagiosepalus
D. Brakisepalus
E. Oksisepalus
F. Kleebattschädel
G. Bukan, A -F

C
Craniosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally flattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491-492) .

30

30. Tengkorak boat-shape
A. Trigonosepalus
B. Skaposepalus
C. Plagiosepalus
D. Brakisepalus
E. Oksisepalus
F. Kleebattschädel
G. Bukan, A -F

B
Craniosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally flattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491-492) .