Neurology Flashcards
- 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 )
- 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 )
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).
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) .
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) .
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) .
- 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
- 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. 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. 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 ) .
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
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 history
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
- 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).
- 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).
- 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
- 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) .
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).
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).
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).
- 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. 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. 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. 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. 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. 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. 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) .
- 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 ) .
31. penutupan sutur sagital pradini 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) .
32. Kepala datar pada dua sisi A. Trigonosepalus B. Skaposepalus C. Plagiosepalus D. Brakisepalus E. Oksisepalus F. Kleebattschädel G. Bukan, A -F
D
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) .
- Tn. X, 34 th dg HIV dibawa ke ruang gawat darurat dengan keluhan sakit kepala, pening, dan hemanopia homonimus kanan. MRI T1 dengan kontras menunjukkan lesi parietookipital kiri 3-4 cm yang tidak menyangat, yang menunjukkan naiknya sinyal pada citra-citra FLAR MRI. Diantara
luka-luka di bawah ini, luka manakah yang paling mungkin:
A. Infeksi measles yang menyebar ke otak
B. Serangan autoimun monophasik oleh sel-sel-T atas protein-protein dasar myelin
C. Peradangan perivenular dan demielinasi
D. Reaktivasi papovavirus menyerang oligodendrosit pada white matter subkortikal
E. Bukan salah satu dari A sampai dengan D
(D)
Progressive multifocal leukoencephalopathy (PML) i s a subacute demyelinating disease that results from reactivation of a papovavirus (JC virus) in immunocompromised patients. The reactivated JC virus infects oligodendrocytes, and results in multifocal areas of demyelination predominantly in the subcortical white mlltter. PML affects 4% of AIDS patients and has been associated with other illnesses characterized by defective cell-mediated immunity, such as lymphomas and leukemias. The lesions of PML are often parieto-occipital, nonenhancing, and exhibit increased signal on T2 and FLAIR MRI with no mass effect. Symptoms of PML depend on the location of lesions and include hemiparesis, visual field cuts, sensory changes, and eventually dementia. The diagnosis of PML is established by biopsy or PCR amplification of JC virus RNA in the CSF. The prognosis of PML is poor, and approximately 80% of patients die within 9 months of symptom onset. Choices A, B, and C are associated with subacute sclerosing panencephalitis (A) and acute disseminated encephalomyelitis ( B and C) (Merritt, pp. 151- 156).
- Pasien-pasien yang mengalami koreksi cepat hiponatremia berisiko terkena masalah di bawah ini yang dapat menimbulkan kejang, disartria, palsi pseudobulbar, koma dan/atau kematian:
A. Penyakit MARCHIAFAVA-BIGNAMI
B. Mielinolisis pontin sentral
C. Leukoenselopalopati dengan penyebaran akut
D. Penyakit KRABBE
E. Penyakit ALEXANDER
B
Central pontine myelinolysis (CPM) is an acute demyelinating condition that primarily affects the pons, although 10% of cases have concomitant extrapontine myelinolysis. CPl\1 most common!
Laki-laki, 16 tahun sedang melakukan uji coba tim sepak bola sekolahnya dan memperhatikan
adanya kram kaki yang parah dan tidak kunjung mereda. Meski pun dia pernah mengalami masalah ini di masa lalu selama latihan, kram yang dialaminya belum pernah separah ini. Hasil pemeriksaan neurologi tidak begitu jelas tetapi tampaknya dia menderita kekurangan mioposporilase enzim yang sangat parah.
35. Diagnosis mana yang paling mungkin? A. Penyakit VON GIERKE B. Oenyakit Pompe C. Penyakit McARDLE D. Penyakit CORI E. Distropi Duchene dengan onset lambat
C
Glycogen storage diseases a Fe primarily autosomal recessive disorders that result from deficiencies of the enzymes im•olved with the metabolism of glucose and glycogen. There are more than 12 different types of glycogenoses; they affect different tissues depending on the expression of the defective enzyme. Type I glycogenosis (Von Gierke’s disease) is characterized by deficiencies of glucose-6-phosphatase and primarily affects the liver. Patients with Von Gierke’s disease present with hepatomegaly and experience severe episodes of hypoglycemia. Type I I glycogenosis ( Pompe’s disease) results from deficiencies of the enzyme acid maltase a.-glucosidase and has three different forms. The infantile type of Pompe’s disease results in hYpotonia, macroglossia, hepatomegaly, cardiomegaly, and death by 2 years of age secondary to cardiac failure. The ju1•enile and adulthood variants of Pompe’s disease are less se1•ere and present with myopathy. Type I I I glycogenosis ( debranching enzyme deficiency or Cori’s disease) is rare and results in hepatomegaly, seizures, and growth retardation in children secondary to deficiencies of the debranching enzyme amylo-1 ,6-glucosidase. Type V glycogenosis
Laki-laki, 16 tahun sedang melakukan uji coba tim sepak bola sekolahnya dan memperhatikan
adanya kram kaki yang parah dan tidak kunjung mereda. Meski pun dia pernah mengalami masalah ini di masa lalu selama latihan, kram yang dialaminya belum pernah separah ini. Hasil pemeriksaan neurologi tidak begitu jelas tetapi tampaknya dia menderita kekurangan mioposporilase enzim yang sangat parah.
36. Apa yang mungkin tampak pada urinalisis pada pasien ini? A. Mioglobinuria B. Kelebihan garam C. Hiperglikemia D. Hiperkalsemia E. Hiperposfatemia
A
Glycogen storage diseases a Fe primarily autosomal recessive disorders that result from deficiencies of the enzymes im•olved with the metabolism of glucose and glycogen. There are more than 12 different types of glycogenoses; they affect different tissues depending on the expression of the defective enzyme. Type I glycogenosis (Von Gierke’s disease) is characterized by deficiencies of glucose-6-phosphatase and primarily affects the liver. Patients with Von Gierke’s disease present with hepatomegaly and experience severe episodes of hypoglycemia. Type I I glycogenosis ( Pompe’s disease) results from deficiencies of the enzyme acid maltase a.-glucosidase and has three different forms. The infantile type of Pompe’s disease results in hYpotonia, macroglossia, hepatomegaly, cardiomegaly, and death by 2 years of age secondary to cardiac failure. The ju1•enile and adulthood variants of Pompe’s disease are less se1•ere and present with myopathy. Type I I I glycogenosis ( debranching enzyme deficiency or Cori’s disease) is rare and results in hepatomegaly, seizures, and growth retardation in children secondary to deficiencies of the debranching enzyme amylo-1 ,6-glucosidase. Type V glycogenosis
Laki-laki, 16 tahun sedang melakukan uji coba tim sepak bola sekolahnya dan memperhatikan
adanya kram kaki yang parah dan tidak kunjung mereda. Meski pun dia pernah mengalami masalah ini di masa lalu selama latihan, kram yang dialaminya belum pernah separah ini. Hasil pemeriksaan neurologi tidak begitu jelas tetapi tampaknya dia menderita kekurangan mioposporilase enzim yang sangat parah.
37. Cara penularan proses penyakit ini adalah: A. Dominan autosomal B. Resesif autosomal C. Resesif rantai-X D. dominan rantai-X E. Penularan poligenetik
B
Glycogen storage diseases a Fe primarily autosomal recessive disorders that result from deficiencies of the enzymes im•olved with the metabolism of glucose and glycogen. There are more than 12 different types of glycogenoses; they affect different tissues depending on the expression of the defective enzyme. Type I glycogenosis (Von Gierke’s disease) is characterized by deficiencies of glucose-6-phosphatase and primarily affects the liver. Patients with Von Gierke’s disease present with hepatomegaly and experience severe episodes of hypoglycemia. Type I I glycogenosis ( Pompe’s disease) results from deficiencies of the enzyme acid maltase a.-glucosidase and has three different forms. The infantile type of Pompe’s disease results in hYpotonia, macroglossia, hepatomegaly, cardiomegaly, and death by 2 years of age secondary to cardiac failure. The ju1•enile and adulthood variants of Pompe’s disease are less se1•ere and present with myopathy. Type I I I glycogenosis ( debranching enzyme deficiency or Cori’s disease) is rare and results in hepatomegaly, seizures, and growth retardation in children secondary to deficiencies of the debranching enzyme amylo-1 ,6-glucosidase. Type V glycogenosis
- Semua ciri ataksia dari Friedrich di bawah ini adalah benar, KECUALI
A. merupakan gangguan repeat trinukleotida (GAA) resesif autosomal yang melibatkan gen frataksin
B. Gejala-gejala awalnya biasanya tampak pada usia 10 sampai dengan 15 tahun
C. Seringkali menyebabkan degenerasi kolom belakang dan saluran sinoserebelar
D. Perubahan-perubahan iskemik kortikal serebelar dan serebral seringkali dapat diasosiasikan dengan kardiomiopati yang menyertainya.
E. Dengan perawatan yang efektif, perkembangan penyakit yang berasosiasi dengan degenerasi neuronal dapat dihentikan sekitar 60% dari waktunya, meski pun gagal jantung biasanya bersifat refraktoris atas terapi.
E.
Friedreich’s ataxia (FA) is an autosomal recessive trinucleotide repeat ( GAA) disorder that involves repeat expansion in the frataxin gene, which encodes a mitochondrial protein and is located on chromosome 9q. The onset of symptoms with FA usually occurs by the age of 10 to 15 years; it is usually fatal by the fifth to sixth decades of life. FA results in degeneration of the posterior columns, spinocerebellar tracts, corticospinal tracts, and Clarke’s nucleus in the spinal cord. The medulla (vestibular, cochlear, gracile, and accessory cuneate nuclei), subthalamic nucleus, and pallidum also exhibit evidence of gliosis. Symptoms of FA include ataxic gait, dysarthria, areflexia, lower limb weakness, and loss of vibratory sense and proprioception. Some patients with FA also develop cardiomyopathy, which can lead to cerebellar and cerebral cortical ischemic changes. F.A is associated with the development of peripheral neuropathy as well, with concomitant degeneration of the dorsal root ganglia. Orthopedic deformities, such as hammertoes, kyphoscoliosis, and pes cavus are also common. There is no effective treatment for FA (Merritt, pp. 645-646).
39. Sindrom LESCH-NYHAN merupakan gangguan resesif rantai-X yang disebabkan kekurangan enzim yang mana? A. Protein transporter-1 Glukose B. Hidroksilase penilalanin C. Fosforibosiltransferase hiposantin- D. Sintase sistationin E. Dekarboksilase purin
C.
Lesch-Nyhan syndrome ( LNS) is an X-linked recessive disorder of purine metabolism that results from deficiencies of the enzyme hypoxanthine-guanine phosphoribosyltransferase ( HPR’f). Levels of uric acid are increased in LNS secondary to increased purine metabolism, and urate deposition can result in severe nephropathy and gout. Patients with LNS exhibit mental retardation, choreoathetosis, spasticity, self-mutilating behavior, and usually die from renal failure in the second or third decade. Glucose transporter protein syndrome (GTPS) results from deficiencies of the glucose transporter-! protein (GLUT-I ) , which is responsible for the facilitative transport of glucose across the blood-brain barrier. Patients with GTPS exhibit decreased CSF glucose levels (hypoglycorrhachia), seizures, developmental delay, microcephaly, ataxia, and hypotonia. Phenylketonuria is an autosomal recessil•e disorder resulting from phenylalanine hydroxylase deficiency, and cystathionine
40. Mungkin sifatnya sekunder, dengan kondisi utama kelemahan/lumpuh pada otot pratibial dan peroneal A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. Bukan, A-G
D
Patients with infarcts or trauma involving the corticospinal tracts on one side often develop a hem iplegic gaic characterized by a stiff leg that does not freely flex at the hip, knee, or ankle. With movement, the involved leg tends to rotate outward in a semicircle (circumduction). and the toe and outer side of the sole often become worn (less commonly also seen with steppage gait). The arm on the affected side is also usually weak, and is often carried in a tlexed position iree of any natural movement. Festinating gait is a term used to describe the involuntary acceleration or hastening that often accompanies Parkinson’s patients. Steppage or equine gait is caused by severe weakness or paralysis of the pretibial or peroneal muscles, with difficulty dorsiflexing and everting the foot. The steps are regular and even, but the advancing foot often hangs with the toes pointing down (footdrop) . Walking is accomplished by abnormally high elevation of the involved leg in order for the foot to clear the ground, often producing a characteristic slap as the foot strikes the floor. Waddling gait is characteristic of chronic or progressive muscular dystrophy (Wohlfart-Kugelberg-Welander disease) and congenital hip dislocation. Reeling gait is a term used to describe the gait of a severely intoxicated patient, which sways in many directions with no attempt to correct the staggering by watching the legs or trunk (as in cerebellar or sensory ataxia) . Toppling gait may be seen in patients with brainstem lesions, especially in the elderly with a recent stroke, where sudden lurches forward and frequent falls are a prominent feature. It has also been described as a feature of the lateral medullary syndrome (Wallenberg’s) and in certain patients with progressive supranuclear palsy and advanced Parkinson’s disease. Scissoring gait may result from lesions involving the thoracic spine, where abnormal gait may result from the combined effects of weakness and spasticity. The legs are usually maintained in an extended or slightly flexed position at the hips, and knees are often adducted at the hips. With severe spasticity, the legs may cross in front of each other during ambulation, causing a scissoring gait (Merritt, pp. 46-49; Adams, pp. 94-99) .
41. Penyakit Parkinson A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. Bukan, A-G
B
Patients with infarcts or trauma involving the corticospinal tracts on one side often develop a hem iplegic gaic characterized by a stiff leg that does not freely flex at the hip, knee, or ankle. With movement, the involved leg tends to rotate outward in a semicircle (circumduction). and the toe and outer side of the sole often become worn (less commonly also seen with steppage gait). The arm on the affected side is also usually weak, and is often carried in a tlexed position iree of any natural movement. Festinating gait is a term used to describe the involuntary acceleration or hastening that often accompanies Parkinson’s patients. Steppage or equine gait is caused by severe weakness or paralysis of the pretibial or peroneal muscles, with difficulty dorsiflexing and everting the foot. The steps are regular and even, but the advancing foot often hangs with the toes pointing down (footdrop) . Walking is accomplished by abnormally high elevation of the involved leg in order for the foot to clear the ground, often producing a characteristic slap as the foot strikes the floor. Waddling gait is characteristic of chronic or progressive muscular dystrophy (Wohlfart-Kugelberg-Welander disease) and congenital hip dislocation. Reeling gait is a term used to describe the gait of a severely intoxicated patient, which sways in many directions with no attempt to correct the staggering by watching the legs or trunk (as in cerebellar or sensory ataxia) . Toppling gait may be seen in patients with brainstem lesions, especially in the elderly with a recent stroke, where sudden lurches forward and frequent falls are a prominent feature. It has also been described as a feature of the lateral medullary syndrome (Wallenberg’s) and in certain patients with progressive supranuclear palsy and advanced Parkinson’s disease. Scissoring gait may result from lesions involving the thoracic spine, where abnormal gait may result from the combined effects of weakness and spasticity. The legs are usually maintained in an extended or slightly flexed position at the hips, and knees are often adducted at the hips. With severe spasticity, the legs may cross in front of each other during ambulation, causing a scissoring gait (Merritt, pp. 46-49; Adams, pp. 94-99) .
42. Berasosiasi dengan sirkumduksi pada kaki A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. Bukan, A-G
C
Patients with infarcts or trauma involving the corticospinal tracts on one side often develop a hem iplegic gaic characterized by a stiff leg that does not freely flex at the hip, knee, or ankle. With movement, the involved leg tends to rotate outward in a semicircle (circumduction). and the toe and outer side of the sole often become worn (less commonly also seen with steppage gait). The arm on the affected side is also usually weak, and is often carried in a tlexed position iree of any natural movement. Festinating gait is a term used to describe the involuntary acceleration or hastening that often accompanies Parkinson’s patients. Steppage or equine gait is caused by severe weakness or paralysis of the pretibial or peroneal muscles, with difficulty dorsiflexing and everting the foot. The steps are regular and even, but the advancing foot often hangs with the toes pointing down (footdrop) . Walking is accomplished by abnormally high elevation of the involved leg in order for the foot to clear the ground, often producing a characteristic slap as the foot strikes the floor. Waddling gait is characteristic of chronic or progressive muscular dystrophy (Wohlfart-Kugelberg-Welander disease) and congenital hip dislocation. Reeling gait is a term used to describe the gait of a severely intoxicated patient, which sways in many directions with no attempt to correct the staggering by watching the legs or trunk (as in cerebellar or sensory ataxia) . Toppling gait may be seen in patients with brainstem lesions, especially in the elderly with a recent stroke, where sudden lurches forward and frequent falls are a prominent feature. It has also been described as a feature of the lateral medullary syndrome (Wallenberg’s) and in certain patients with progressive supranuclear palsy and advanced Parkinson’s disease. Scissoring gait may result from lesions involving the thoracic spine, where abnormal gait may result from the combined effects of weakness and spasticity. The legs are usually maintained in an extended or slightly flexed position at the hips, and knees are often adducted at the hips. With severe spasticity, the legs may cross in front of each other during ambulation, causing a scissoring gait (Merritt, pp. 46-49; Adams, pp. 94-99) .
43. Sindrom WOHLFART-KUGELBERG-WELANDER kronis atau progresif A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. Bukan, A-G
E
Patients with infarcts or trauma involving the corticospinal tracts on one side often develop a hem iplegic gaic characterized by a stiff leg that does not freely flex at the hip, knee, or ankle. With movement, the involved leg tends to rotate outward in a semicircle (circumduction). and the toe and outer side of the sole often become worn (less commonly also seen with steppage gait). The arm on the affected side is also usually weak, and is often carried in a tlexed position iree of any natural movement. Festinating gait is a term used to describe the involuntary acceleration or hastening that often accompanies Parkinson’s patients. Steppage or equine gait is caused by severe weakness or paralysis of the pretibial or peroneal muscles, with difficulty dorsiflexing and everting the foot. The steps are regular and even, but the advancing foot often hangs with the toes pointing down (footdrop) . Walking is accomplished by abnormally high elevation of the involved leg in order for the foot to clear the ground, often producing a characteristic slap as the foot strikes the floor. Waddling gait is characteristic of chronic or progressive muscular dystrophy (Wohlfart-Kugelberg-Welander disease) and congenital hip dislocation. Reeling gait is a term used to describe the gait of a severely intoxicated patient, which sways in many directions with no attempt to correct the staggering by watching the legs or trunk (as in cerebellar or sensory ataxia) . Toppling gait may be seen in patients with brainstem lesions, especially in the elderly with a recent stroke, where sudden lurches forward and frequent falls are a prominent feature. It has also been described as a feature of the lateral medullary syndrome (Wallenberg’s) and in certain patients with progressive supranuclear palsy and advanced Parkinson’s disease. Scissoring gait may result from lesions involving the thoracic spine, where abnormal gait may result from the combined effects of weakness and spasticity. The legs are usually maintained in an extended or slightly flexed position at the hips, and knees are often adducted at the hips. With severe spasticity, the legs may cross in front of each other during ambulation, causing a scissoring gait (Merritt, pp. 46-49; Adams, pp. 94-99) .
44. Mungkin menyertai lesi pada spine thorak A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. Bukan, A-G
A
Patients with infarcts or trauma involving the corticospinal tracts on one side often develop a hem iplegic gaic characterized by a stiff leg that does not freely flex at the hip, knee, or ankle. With movement, the involved leg tends to rotate outward in a semicircle (circumduction). and the toe and outer side of the sole often become worn (less commonly also seen with steppage gait). The arm on the affected side is also usually weak, and is often carried in a tlexed position iree of any natural movement. Festinating gait is a term used to describe the involuntary acceleration or hastening that often accompanies Parkinson’s patients. Steppage or equine gait is caused by severe weakness or paralysis of the pretibial or peroneal muscles, with difficulty dorsiflexing and everting the foot. The steps are regular and even, but the advancing foot often hangs with the toes pointing down (footdrop) . Walking is accomplished by abnormally high elevation of the involved leg in order for the foot to clear the ground, often producing a characteristic slap as the foot strikes the floor. Waddling gait is characteristic of chronic or progressive muscular dystrophy (Wohlfart-Kugelberg-Welander disease) and congenital hip dislocation. Reeling gait is a term used to describe the gait of a severely intoxicated patient, which sways in many directions with no attempt to correct the staggering by watching the legs or trunk (as in cerebellar or sensory ataxia) . Toppling gait may be seen in patients with brainstem lesions, especially in the elderly with a recent stroke, where sudden lurches forward and frequent falls are a prominent feature. It has also been described as a feature of the lateral medullary syndrome (Wallenberg’s) and in certain patients with progressive supranuclear palsy and advanced Parkinson’s disease. Scissoring gait may result from lesions involving the thoracic spine, where abnormal gait may result from the combined effects of weakness and spasticity. The legs are usually maintained in an extended or slightly flexed position at the hips, and knees are often adducted at the hips. With severe spasticity, the legs may cross in front of each other during ambulation, causing a scissoring gait (Merritt, pp. 46-49; Adams, pp. 94-99) .
45. Seringkali tampak dengan foot drop A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. Bukan, A-G
D
Patients with infarcts or trauma involving the corticospinal tracts on one side often develop a hem iplegic gaic characterized by a stiff leg that does not freely flex at the hip, knee, or ankle. With movement, the involved leg tends to rotate outward in a semicircle (circumduction). and the toe and outer side of the sole often become worn (less commonly also seen with steppage gait). The arm on the affected side is also usually weak, and is often carried in a tlexed position iree of any natural movement. Festinating gait is a term used to describe the involuntary acceleration or hastening that often accompanies Parkinson’s patients. Steppage or equine gait is caused by severe weakness or paralysis of the pretibial or peroneal muscles, with difficulty dorsiflexing and everting the foot. The steps are regular and even, but the advancing foot often hangs with the toes pointing down (footdrop) . Walking is accomplished by abnormally high elevation of the involved leg in order for the foot to clear the ground, often producing a characteristic slap as the foot strikes the floor. Waddling gait is characteristic of chronic or progressive muscular dystrophy (Wohlfart-Kugelberg-Welander disease) and congenital hip dislocation. Reeling gait is a term used to describe the gait of a severely intoxicated patient, which sways in many directions with no attempt to correct the staggering by watching the legs or trunk (as in cerebellar or sensory ataxia) . Toppling gait may be seen in patients with brainstem lesions, especially in the elderly with a recent stroke, where sudden lurches forward and frequent falls are a prominent feature. It has also been described as a feature of the lateral medullary syndrome (Wallenberg’s) and in certain patients with progressive supranuclear palsy and advanced Parkinson’s disease. Scissoring gait may result from lesions involving the thoracic spine, where abnormal gait may result from the combined effects of weakness and spasticity. The legs are usually maintained in an extended or slightly flexed position at the hips, and knees are often adducted at the hips. With severe spasticity, the legs may cross in front of each other during ambulation, causing a scissoring gait (Merritt, pp. 46-49; Adams, pp. 94-99) .
46. Infark pada kapsul interna A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. Bukan, A-G
C
Patients with infarcts or trauma involving the corticospinal tracts on one side often develop a hem iplegic gaic characterized by a stiff leg that does not freely flex at the hip, knee, or ankle. With movement, the involved leg tends to rotate outward in a semicircle (circumduction). and the toe and outer side of the sole often become worn (less commonly also seen with steppage gait). The arm on the affected side is also usually weak, and is often carried in a tlexed position iree of any natural movement. Festinating gait is a term used to describe the involuntary acceleration or hastening that often accompanies Parkinson’s patients. Steppage or equine gait is caused by severe weakness or paralysis of the pretibial or peroneal muscles, with difficulty dorsiflexing and everting the foot. The steps are regular and even, but the advancing foot often hangs with the toes pointing down (footdrop) . Walking is accomplished by abnormally high elevation of the involved leg in order for the foot to clear the ground, often producing a characteristic slap as the foot strikes the floor. Waddling gait is characteristic of chronic or progressive muscular dystrophy (Wohlfart-Kugelberg-Welander disease) and congenital hip dislocation. Reeling gait is a term used to describe the gait of a severely intoxicated patient, which sways in many directions with no attempt to correct the staggering by watching the legs or trunk (as in cerebellar or sensory ataxia) . Toppling gait may be seen in patients with brainstem lesions, especially in the elderly with a recent stroke, where sudden lurches forward and frequent falls are a prominent feature. It has also been described as a feature of the lateral medullary syndrome (Wallenberg’s) and in certain patients with progressive supranuclear palsy and advanced Parkinson’s disease. Scissoring gait may result from lesions involving the thoracic spine, where abnormal gait may result from the combined effects of weakness and spasticity. The legs are usually maintained in an extended or slightly flexed position at the hips, and knees are often adducted at the hips. With severe spasticity, the legs may cross in front of each other during ambulation, causing a scissoring gait (Merritt, pp. 46-49; Adams, pp. 94-99) .
47. Batas anterior dan lateral sol sepatu seringkali rusak A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. Bukan, A-G
C
Patients with infarcts or trauma involving the corticospinal tracts on one side often develop a hem iplegic gaic characterized by a stiff leg that does not freely flex at the hip, knee, or ankle. With movement, the involved leg tends to rotate outward in a semicircle (circumduction). and the toe and outer side of the sole often become worn (less commonly also seen with steppage gait). The arm on the affected side is also usually weak, and is often carried in a tlexed position iree of any natural movement. Festinating gait is a term used to describe the involuntary acceleration or hastening that often accompanies Parkinson’s patients. Steppage or equine gait is caused by severe weakness or paralysis of the pretibial or peroneal muscles, with difficulty dorsiflexing and everting the foot. The steps are regular and even, but the advancing foot often hangs with the toes pointing down (footdrop) . Walking is accomplished by abnormally high elevation of the involved leg in order for the foot to clear the ground, often producing a characteristic slap as the foot strikes the floor. Waddling gait is characteristic of chronic or progressive muscular dystrophy (Wohlfart-Kugelberg-Welander disease) and congenital hip dislocation. Reeling gait is a term used to describe the gait of a severely intoxicated patient, which sways in many directions with no attempt to correct the staggering by watching the legs or trunk (as in cerebellar or sensory ataxia) . Toppling gait may be seen in patients with brainstem lesions, especially in the elderly with a recent stroke, where sudden lurches forward and frequent falls are a prominent feature. It has also been described as a feature of the lateral medullary syndrome (Wallenberg’s) and in certain patients with progressive supranuclear palsy and advanced Parkinson’s disease. Scissoring gait may result from lesions involving the thoracic spine, where abnormal gait may result from the combined effects of weakness and spasticity. The legs are usually maintained in an extended or slightly flexed position at the hips, and knees are often adducted at the hips. With severe spasticity, the legs may cross in front of each other during ambulation, causing a scissoring gait (Merritt, pp. 46-49; Adams, pp. 94-99) .
Perempuan, 34 tahun tiba-tiba mengalami hemiparesis kiri. Dia menderita trombosis venous yang dalam pada kaki kirinya sekitar 4 tahun sebelumnya.
48. Diantara penyebab-penyebab defisit dari pasien di bawah ini, penyebab manakah yang paling mungkin? A. Fibrilasi atrial B. Tumor otak metastatis C. Sklerosis majemuk D. Antikoagulan lupus E. Cacad septal artrial
D
The lupus inhibitor can be found in up to one-third of systemic lupus erythematosus (SLE) patients . In SLE patients, the associated thrombosis risk can be as high as 10 to 1 5%, while the risk is less in patients without SLE but still in excess of normal. This disease process is associated with deep venous thrombosis of the legs, ischemic strokes in the brain, and miscarriages (placental dysfunction) . Abnormal bleeding does not typically occur, although laboratory findings include prolonged PT and PTT that does not reverse when the patient’s plasma is mixed with normal plasma. Diagnosis can be confirmed by the thromboplastin inhibition test (TTI) and antiphospholipid antibody assays. Treatment to prevent thrombosis may include corticosteroids, antiplatelet drugs, and anticoagulation; plasmapheresis is not typically used. Anticoagulation appears to be the best therapy, with a goal of keeping the INR at least 3 . 0 . Atrial fibrillation and atrial-septal defects are common causes of stroke in certain patient populations, but there is no reason to suspect a rhythm or congenital heart problem in this patient. Multiple sclerosis and metastatic brain tumors are not typically associated with deep venous ‘thrombosis unless the patient is immobilized for a prolonged time ( Merritt, p. 245).
Perempuan, 34 tahun tiba-tiba mengalami hemiparesis kiri. Dia menderita trombosis venous yang dalam pada kaki kirinya sekitar 4 tahun sebelumnya.
- Ciri laboatoris non-spesifik dari gangguan ini mungkin meliputi :
A. PT dan PTT menahun yang tidak pulih
ketika plasma pasien tercampur dengan plasma normal
B. Pita oligoklional pada CSF
C. Kelainan gen distropia
D. Elevasi antibodi lgE pada serum
E. bukan salah satu dari A sampai dengan D
A
The lupus inhibitor can be found in up to one-third of systemic lupus erythematosus (SLE) patients . In SLE patients, the associated thrombosis risk can be as high as 10 to 1 5%, while the risk is less in patients without SLE but still in excess of normal. This disease process is associated with deep venous thrombosis of the legs, ischemic strokes in the brain, and miscarriages (placental dysfunction) . Abnormal bleeding does not typically occur, although laboratory findings include prolonged PT and PTT that does not reverse when the patient’s plasma is mixed with normal plasma. Diagnosis can be confirmed by the thromboplastin inhibition test (TTI) and antiphospholipid antibody assays. Treatment to prevent thrombosis may include corticosteroids, antiplatelet drugs, and anticoagulation; plasmapheresis is not typically used. Anticoagulation appears to be the best therapy, with a goal of keeping the INR at least 3 . 0 . Atrial fibrillation and atrial-septal defects are common causes of stroke in certain patient populations, but there is no reason to suspect a rhythm or congenital heart problem in this patient. Multiple sclerosis and metastatic brain tumors are not typically associated with deep venous ‘thrombosis unless the patient is immobilized for a prolonged time ( Merritt, p. 245).
Perempuan, 34 tahun tiba-tiba mengalami hemiparesis kiri. Dia menderita trombosis venous yang dalam pada kaki kirinya sekitar 4 tahun sebelumnya.
- Yang dapat memperkuat Diagnosisnya adalah:
A. Membuktikan adanya reaksi-reaksi penghambatan di dalam sistem penggumpalan dengan uji hambatan trombioplastin (TTI) dan uji venom biper dari Russell
B. Penguatan adanya cacad pada faktor V
C. Luka majemuk pada materi putih dalam dengan MRI
D. Mencatat adanya cacad septal atrial dengan menggunakan ekokardiografi
E. Pendarahan yang berlebihan setelah mencukur
A
The lupus inhibitor can be found in up to one-third of systemic lupus erythematosus (SLE) patients . In SLE patients, the associated thrombosis risk can be as high as 10 to 1 5%, while the risk is less in patients without SLE but still in excess of normal. This disease process is associated with deep venous thrombosis of the legs, ischemic strokes in the brain, and miscarriages (placental dysfunction) . Abnormal bleeding does not typically occur, although laboratory findings include prolonged PT and PTT that does not reverse when the patient’s plasma is mixed with normal plasma. Diagnosis can be confirmed by the thromboplastin inhibition test (TTI) and antiphospholipid antibody assays. Treatment to prevent thrombosis may include corticosteroids, antiplatelet drugs, and anticoagulation; plasmapheresis is not typically used. Anticoagulation appears to be the best therapy, with a goal of keeping the INR at least 3 . 0 . Atrial fibrillation and atrial-septal defects are common causes of stroke in certain patient populations, but there is no reason to suspect a rhythm or congenital heart problem in this patient. Multiple sclerosis and metastatic brain tumors are not typically associated with deep venous ‘thrombosis unless the patient is immobilized for a prolonged time ( Merritt, p. 245).
Perempuan, 34 tahun tiba-tiba mengalami hemiparesis kiri. Dia menderita trombosis venous yang dalam pada kaki kirinya sekitar 4 tahun sebelumnya.
- Terapi optimal untuk penyakit ini bisa mencakup:
- Mempertahankan rasio normalisasi internasional (INR) pada ambang minimal 3.0 dari warfarin
- Kortikosteroid
- Agen-agen antiplatelet
- Plasmaperesis
A
The lupus inhibitor can be found in up to one-third of systemic lupus erythematosus (SLE) patients . In SLE patients, the associated thrombosis risk can be as high as 10 to 1 5%, while the risk is less in patients without SLE but still in excess of normal. This disease process is associated with deep venous thrombosis of the legs, ischemic strokes in the brain, and miscarriages (placental dysfunction) . Abnormal bleeding does not typically occur, although laboratory findings include prolonged PT and PTT that does not reverse when the patient’s plasma is mixed with normal plasma. Diagnosis can be confirmed by the thromboplastin inhibition test (TTI) and antiphospholipid antibody assays. Treatment to prevent thrombosis may include corticosteroids, antiplatelet drugs, and anticoagulation; plasmapheresis is not typically used. Anticoagulation appears to be the best therapy, with a goal of keeping the INR at least 3 . 0 . Atrial fibrillation and atrial-septal defects are common causes of stroke in certain patient populations, but there is no reason to suspect a rhythm or congenital heart problem in this patient. Multiple sclerosis and metastatic brain tumors are not typically associated with deep venous ‘thrombosis unless the patient is immobilized for a prolonged time ( Merritt, p. 245).
Ny. X, 72 tahun yang sebelumnya sehat menderita halusinasi pendengaran dan depresi. Pasien mengalami kesulitan menjelaskan detilnya tapi dia yakin bahwa anak laki-lakinya memang sedang berbicara kepadanya. Selama wawancara, pasien tidak kooperatif, tetapi pemeriksaan fisik menunjukkan hasil yang tidak jelas. Setiap hari pasien mengkonsumsi multivitamin dan aspitrin
52. Diantara diagnosis-diagnosis di bawah ini, diagnosis manakah yang paling mungkin? A. Hipertiroidisme B. Kejang parsial kompleks C. Penyakit Alzheimer D. Demensia (pikun) multi-infract E. Hiperparatiroidisme
C
Alzheimer’s disease (AD) is a rare cause of new-onset auditory hallucinations in elderly patients. Typically, other forms of cognitive impairment are present, but hallucinations may occasionally precede other manifestations of this disease process. Low doses of haloperidol are often effective in treating the hallucinations, while medications such as tacrine and donepezil may slow the cognitive decline modestly early in the disease course. Several structures in the brain are affected in AD, including the hippocampus (especially CAl ) , mesial temporal lobe, frontotemporoparietal association areas, and the nucleus basalis of i\leynert (n.b.M) . The degeneration of cells in the n.b.M (and other basal forebrain nuclei) results in decreased•cortical cholinergic input and is associated with decreased levels of ACh and choline acetyltransferase in the hippocampus and neocortex. Patients with AD also exhibit decreased CNS levels of serotonin, norepinephrine, glutamate, and substance P. Ventriculomegaly accompanies the diffuse cerebral neocortical atrophy. Several genes are involved in the pathogenesis of AD including amyloid precursor protein (chromosome 2 1 ) , presenilin 1 (chromosome 14), and presenilin 2 (chromosome 1). The £4 allele of apolipoprotein E (chromosome 19) is associated with an increased risk of developing AD and is associated with 25 to 40% of all cases of AD. In any patient with suspected AD, treatable causes of dementia must be ruled out initially with a good history, physical examination, laboratory data, and imaging studies. Patients with AD are most often diagnosed with a detailed history and physical examination (Merritt, pp. 633 - 637 ) .
Ny. X, 72 tahun yang sebelumnya sehat menderita halusinasi pendengaran dan depresi. Pasien mengalami kesulitan menjelaskan detilnya tapi dia yakin bahwa anak laki-lakinya memang sedang berbicara kepadanya. Selama wawancara, pasien tidak kooperatif, tetapi pemeriksaan fisik menunjukkan hasil yang tidak jelas. Setiap hari pasien mengkonsumsi multivitamin dan aspitrin
- Cara yang paling lazim untuk memperoleh diagnosis ini mencakup:
A. Ambil kadar hormon perangsang tiroid (TSH)
B. Elektroensepalografi
C. Riwayat detil dan pemeriksaan fisik
D. Biopsi otak
E. Ambil kadar enzim pengubah angiotensin
C
Alzheimer’s disease (AD) is a rare cause of new-onset auditory hallucinations in elderly patients. Typically, other forms of cognitive impairment are present, but hallucinations may occasionally precede other manifestations of this disease process. Low doses of haloperidol are often effective in treating the hallucinations, while medications such as tacrine and donepezil may slow the cognitive decline modestly early in the disease course. Several structures in the brain are affected in AD, including the hippocampus (especially CAl ) , mesial temporal lobe, frontotemporoparietal association areas, and the nucleus basalis of i\leynert (n.b.M) . The degeneration of cells in the n.b.M (and other basal forebrain nuclei) results in decreased•cortical cholinergic input and is associated with decreased levels of ACh and choline acetyltransferase in the hippocampus and neocortex. Patients with AD also exhibit decreased CNS levels of serotonin, norepinephrine, glutamate, and substance P. Ventriculomegaly accompanies the diffuse cerebral neocortical atrophy. Several genes are involved in the pathogenesis of AD including amyloid precursor protein (chromosome 2 1 ) , presenilin 1 (chromosome 14), and presenilin 2 (chromosome 1). The £4 allele of apolipoprotein E (chromosome 19) is associated with an increased risk of developing AD and is associated with 25 to 40% of all cases of AD. In any patient with suspected AD, treatable causes of dementia must be ruled out initially with a good history, physical examination, laboratory data, and imaging studies. Patients with AD are most often diagnosed with a detailed history and physical examination (Merritt, pp. 633 - 637 ) .
Ny. X, 72 tahun yang sebelumnya sehat menderita halusinasi pendengaran dan depresi. Pasien mengalami kesulitan menjelaskan detilnya tapi dia yakin bahwa anak laki-lakinya memang sedang berbicara kepadanya. Selama wawancara, pasien tidak kooperatif, tetapi pemeriksaan fisik menunjukkan hasil yang tidak jelas. Setiap hari pasien mengkonsumsi multivitamin dan aspitrin
54. Rezim perawatan untuk halusinasi pendengaran ini dapat meliputi: A. Takrin B. Donepezil C. Vitamin E D. Haloperidol E. A, B, C dan D
D
Alzheimer’s disease (AD) is a rare cause of new-onset auditory hallucinations in elderly patients. Typically, other forms of cognitive impairment are present, but hallucinations may occasionally precede other manifestations of this disease process. Low doses of haloperidol are often effective in treating the hallucinations, while medications such as tacrine and donepezil may slow the cognitive decline modestly early in the disease course. Several structures in the brain are affected in AD, including the hippocampus (especially CAl ) , mesial temporal lobe, frontotemporoparietal association areas, and the nucleus basalis of i\leynert (n.b.M) . The degeneration of cells in the n.b.M (and other basal forebrain nuclei) results in decreased•cortical cholinergic input and is associated with decreased levels of ACh and choline acetyltransferase in the hippocampus and neocortex. Patients with AD also exhibit decreased CNS levels of serotonin, norepinephrine, glutamate, and substance P. Ventriculomegaly accompanies the diffuse cerebral neocortical atrophy. Several genes are involved in the pathogenesis of AD including amyloid precursor protein (chromosome 2 1 ) , presenilin 1 (chromosome 14), and presenilin 2 (chromosome 1). The £4 allele of apolipoprotein E (chromosome 19) is associated with an increased risk of developing AD and is associated with 25 to 40% of all cases of AD. In any patient with suspected AD, treatable causes of dementia must be ruled out initially with a good history, physical examination, laboratory data, and imaging studies. Patients with AD are most often diagnosed with a detailed history and physical examination (Merritt, pp. 633 - 637 ) .
- Seorang pengacara berusia 45 tahun mengeluh kesulitan memegang dan menggunakan pena. Dia mencatat bahwa dia mengalami kejang-kejang pada lengan kanan dan tangannya selama tiga bulan sebelumnya hanya pada saat menulis. Pemeriksaan fisik kurang begitu
menggembirakan,. Diantara etiologi-etiologi di bawah ini, etiologi manakah yang paling mungkin berasosiasi dengan tanda-tanda /gejala pada pasien?
A. Penyakit Parkinson dini
B. Atetosis
C. Radikulopati C7
D. Distonia
E. Sindrom saluran Carpal
D .
Writer’s cramp i s a focal dystonia o f unknown etiology. Patients develop sustained muscle contractions that yield abnormal postures or twisting movements when attempting to write. It differs from athetosis by the persistent nature of the abnormal posture or movement. It can be focal (writer’s cramp), segmental (e.g. , face), or rarely multifocal (e.g., face and leg) . Dystonia can also be either primary (inherited) or secondary (stroke, toxin, medication, etc. ) . The medical treatment is variable, often empiric, and can include local botulinum toxin injections, anticholinergics, benzodiazepines, anticonvulsants, lithium , reserpine, baclofen, and levodopa. Micrographia can be seen with Parkinson’s disease but is often accompanied by signs of rigidity, tremor, and bradykinesia. Carpal tunnel syndrome is clue to pressure on the median nerve and is asso.ciated with numbness. weakness, and pain. A cervical radiculopathy rarely if ever presents with the findings seen in this patient; instead. there is pain, weakness, numbness, and ret1ex changes in the distribution of the affected nerve ( Merritt, pp. 669- 6 7 7 )
56. Antibodi yang diisolasi dengan karsinoma paru-paru sel kecil, neuropati sensoris, ensepalomielitis A. Antibodi anti-Hu B. Antibodi anti-Ri C. Antibodi anti-Jo D. Antibodi anti-Yo E. Antibodi anti-VGCC F. Antibodi anti-Tr G. Antibodi anti-Ta H. Bukan, A-G
A
Refer to Table 3 . 56-3.63A (Merritt, pp. 767, 894-895; Rolak. pp. 237-239 ) .
57. Sindrom miastenik LAMBERT-EATON A. Antibodi anti-Hu B. Antibodi anti-Ri C. Antibodi anti-Jo D. Antibodi anti-Yo E. Antibodi anti-VGCC F. Antibodi anti-Tr G. Antibodi anti-Ta H. Bukan, A-G
E
Refer to Table 3 . 56-3.63A (Merritt, pp. 767, 894-895; Rolak. pp. 237-239 ) .
58. Sindroma Opsoklonus-mioklonus A. Antibodi anti-Hu B. Antibodi anti-Ri C. Antibodi anti-Jo D. Antibodi anti-Yo E. Antibodi anti-VGCC F. Antibodi anti-Tr G. Antibodi anti-Ta H. Bukan, A-G
B
Refer to Table 3 . 56-3.63A (Merritt, pp. 767, 894-895; Rolak. pp. 237-239 ) .
59. Bereaksi terhadap del-sel Punkinje dari serebelum A. Antibodi anti-Hu B. Antibodi anti-Ri C. Antibodi anti-Jo D. Antibodi anti-Yo E. Antibodi anti-VGCC F. Antibodi anti-Tr G. Antibodi anti-Ta H. Bukan, A-G
F
Refer to Table 3 . 56-3.63A (Merritt, pp. 767, 894-895; Rolak. pp. 237-239 ) .
60. Polimiositis A. Antibodi anti-Hu B. Antibodi anti-Ri C. Antibodi anti-Jo D. Antibodi anti-Yo E. Antibodi anti-VGCC F. Antibodi anti-Tr G. Antibodi anti-Ta H. Bukan, A-G
C
Refer to Table 3 . 56-3.63A (Merritt, pp. 767, 894-895; Rolak. pp. 237-239 ) .
61. Penyakit HODGKIN A. Antibodi anti-Hu B. Antibodi anti-Ri C. Antibodi anti-Jo D. Antibodi anti-Yo E. Antibodi anti-VGCC F. Antibodi anti-Tr G. Antibodi anti-Ta H. Bukan, A-G
F
Refer to Table 3 . 56-3.63A (Merritt, pp. 767, 894-895; Rolak. pp. 237-239 ) .
62. Antibodi yang terutama ditemukan pada pasien dengan kanker testikular A. Antibodi anti-Hu B. Antibodi anti-Ri C. Antibodi anti-Jo D. Antibodi anti-Yo E. Antibodi anti-VGCC F. Antibodi anti-Tr G. Antibodi anti-Ta H. Bukan, A-G
G
Refer to Table 3 . 56-3.63A (Merritt, pp. 767, 894-895; Rolak. pp. 237-239 ) .
63. Degenerasi retinal A. Antibodi anti-Hu B. Antibodi anti-Ri C. Antibodi anti-Jo D. Antibodi anti-Yo E. Antibodi anti-VGCC F. Antibodi anti-Tr G. Antibodi anti-Ta H. Bukan, A-G
B
Refer to Table 3 . 56-3.63A (Merritt, pp. 767, 894-895; Rolak. pp. 237-239 ) .
- Seorang ibu membawa bayi laki-lakinya yang berumur 4 bulan ke kantor anda setelah menyaksikan satu episode yang ditandai oleh kejang-kejang ekstensor yang tiba-tiba, dan melibatkan kepala, tangkai dan anggota badan. Yang paling mungkin ditunjukkan oleh EEG adalah:
A. Pengosongan gelombang-percik 3-Hz
B. Pengosongan epileptiform dengan lateralisasi berkala (PLEDs)
C. Hipsaritmia
D. Pelambatan yang semakin lama semakin umum
E. gelombang kejut 4 – 6 Hz
C.
Refer to Table 3 .64A ( Merritt, pp. 813-836)
- Peningkatan latensi refleks-H bersama-sama dengan latensi gelombang F melokalisasi luka pada lokasi yang mana?
A. Pertemuan neuromuscular
B. sel-sel tanduk depan dari spinal cord
C. Akar-akar dorsal
D. Akar-akar ventral
E. Bukan salah satu dari A sampai dengan D
C.
The F wave and H reflex evaluate certain aspects of nerve conduction. Whereas sensory nerve action potentials (SNAP) and compound muscle action potentials (Ct–1AP) are best at evaluating distal nerves, the F wave and H reflex are the two most commonly used methods in evaluating the proximal portions of nerves. The F wa1•e (F response) measures the entire length of the nerve, including the ventral root. It results from supramaximal stimulus of distal motor nerves with impulse propagation in an antidromic direction. The stimulus tra1•els proximally up the motor nerve and stimulates the anterior horn motor neurons. The variable backfiring of nonreiractory anterior horn cells then results in impulse propagation back down the ventral root and motor nerve to the distal electrode. F-wave latencies are most sensitive for disorders causing generalized or multifocal demyelination (GBS or extensive plexopathies). This is because any focal conduction slowing is diluted by the normal conduction velocity over most of the F-11•ave pathway. The H retlex is the electrical equi1•alent of the stretch retlex and is obtained with submaximal stimulation of the median and tibial nerves. The H reflex therefore involves an afferent (sensory) limb and an efferent (motor) limb, similar to monosynaptic reflex arcs. The H reflex exhibits increased latency in proximal neuropathies and radiculopathies (i.e., C6, C7, or Sl root lesions). Increased H-reflex latencies in conjunction with normal F-wm•e latencies localize lesions to the dorsal roots (Adams, pp. 1022-1025; Merritt, pp. 73-7 6 ) .
- 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 )
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).
- Semua karakteristik di bawah ini adalah karakteristik neuropati demielinasi pada pemeriksaan-pemeriksaan konduksi saraf motorik, KECUALI
A. Latensi distal menahun
B. Menurunnya kecepatan segmental
C. Amplitudo respons yang jika dipicu tetap normal atau hanya menurun sedikit
D. Penurunan gelombang F
E. Dispersi temporal
D.
Nerve conduction studies (NCSs) vary in conditions that result in either demyelination or axonal degeneration. Conduction velocity. amplitude of evoked response, latency (latency from the stimulus to recording electrodes) , and duration of response all provide information about the integrity of motor and sensory nerves. NCSs of the sensory nerves generate a sensory nerve action potential (SNAP), while NCSs of motor nerves generate a compound muscle action potential
67. Semua karakteristik di bawah ini adalah karakteristik potensi unit-motorik-tunggal normal pada EMG, KECUALI A. Durasi 5-15 milidetik B. Dua sampai empat fase C. 0,5 – 3 mV D. Potensi fibrilasi E. Aktivitas insersional
D.
Normal muscle potentials appear as waveforms with a duration of 5 to 1 5 ms, 2 to 4 phases, and an amplitude of 0 .
pic 68. Spindel tidur dan kompleks K A. Tahap 1 B. Tahap 2 C. Tahap 3 D. Tahap 4 E. Rapid Eye Movement (REM) F. Bukan salah satu dari A sampai dengan F
B,C
Refer t o Table 3 . 68- 3 . 73A ( Merritt, p. 64).
pic 69. Gelombang delta > 50% A. Tahap 1 B. Tahap 2 C. Tahap 3 D. Tahap 4 E. Rapid Eye Movement (REM) F. Bukan salah satu dari A sampai dengan F
D
Refer t o Table 3 . 68- 3 . 73A ( Merritt, p. 64).
pic 70. Transisi dari gelombang α ke aktivitas voltase-rendah lambat A. Tahap 1 B. Tahap 2 C. Tahap 3 D. Tahap 4 E. Rapid Eye Movement (REM) F. Bukan salah satu dari A sampai dengan F
A
Refer t o Table 3 . 68- 3 . 73A ( Merritt, p. 64)
