Assistant Professor in Neurology Question Paper and Answer Key

Question Paper Code: 24/2024/OL
Category Code: 336/2023
Exam: Assistant Professor in Neurology
Date of Test 16-03-2024
Department Medical Education

Question1:-In muscle spindle, the term annulospiral endings is given for
        A:-Group la fibers
        B:-Group II endings
        C:-Dynamic gamma motor neuron
        D:-Static gamma motor neuron
        Correct Answer:- Option-A

Question2:-Which nucleus of amygdala has connections with hypothalamus?
        A:-Superficial corticomedial
        D:-None of these
        Correct Answer:- Option-B

Question3:-Neural groove is formed on Day
        Correct Answer:- Option-B

Question4:-34 year old male presents with progressive distal Right upper limb weakness in the distribution of median and ulnar nerves with no sensory
symptoms. What is the likely diagnosis?
        A:-Carpal tunnel syndrome
        B:-Mononeuritis multiplex
        C:-Multifocal motor neuropathy
        D:-Cubital s'fossa syndrome
        Correct Answer:- Option-C

Question5:-A 45 year old male presents with weakness in left arm abduction and sensory loss over the lateral surface of the left upper arm and shoulder. Damage to
which of the following structures of the brachial plexus may cause this presentation?
        A:-Anterior division of lower trunk
        B:-C7 root
        C:-Posterior cord
        D:-Middle trunk
        Correct Answer:- Option-C

Question6:-A 62-year-old man is seen for subacute onset of generalized weakness and gait changes with diffuse decreased reflexes and CIDP is suspected. Which of
the following clinical and laboratory findings is concerning for the diagnosis of POEMS syndrome as opposed to CIDP?
        A:-Absence of pain
        C:-Elevated CSF protein level
        D:-Presence of lambda light chain
        Correct Answer:- Option-D

Question7:-A 44-year old man presents for evaluation of weakness. He reports the onset of difficulty in swallowing and weakness in his arms 3 year prior, which was
initially attributed to statin therapy but has continued to progress off the statin. He reports progression of weakness and is detected to be diabetic. He has tongue and
peri oral fasciculations, bilateral deltoid and iliopsoas weakness with atrophy and fasciculations. Absent ankle jerk; trace reflexes and flexor plantar and intact
sensation. Which of the following additional features would be expected on examination?
        A:-Bitemporal hemianopia
        B:-Cerebellar ataxia
        Correct Answer:- Option-C

Question8:-A 13-year old boy presents with muscle pain and stiffness which is much worse in cold weather. In fact, few hours after exposure to cold, he felt quite weak
as well as stiff. He had percussion myotonia at the thenar eminence and eye closure myotoria. Mutation in which of the following channels is mots likely responsible for his presentation?
        Correct Answer:- Option-D

Question9:-20-year-old male presents for evaluation of abnormal movements. He initially developed dystonia of his left leg at 10 year of age, associated with walking
and running. No response to levodopa, and trihexyphenidyl. He was successfully treated with Botulinum injections. However, he progressed to have dystonia in his
right arm. His 57 year old father has Write's cramp and his younger sister has recently developed suspected dystonia in her legs. Mutation in which of the following genes is the most likely cause of the dystonia?
        A:-Beta tubulin
        B:-GTP- cyclohydrolase I
        C:-TATA - binding protein - associated factor gene
        D:-Torsin A
        Correct Answer:- Option-D

Question10:-Which of the following is not a structure involved in the formation of new memories within the circuit of paper?
        B:-Mammilary bodies
        C:-Mediodorsal nucleus of the thalamus
        Correct Answer:- Option-D

Question11:-Associative visual agnosia is caused by a lesion in the
        A:-Posterior parietal cortex
        B:-Occipital cortex
        C:-Inferotemporal cortex
        D:-Association area of the frontal cortex
        Correct Answer:- Option-A

Question12:-All the following statements are correct regarding Foville syndrome except:
        A:-Presence of Ipsilateral V, VI, VII, VIII Horner syndrome and contralateral ataxia
        B:-Presence Ipsilateral VI, VII and contralateral ataxia
        C:-Presence of Ipsilateral VI, VII Horner syndrome and contralateral ataxia
        D:-Presence of Ipsilateral VI, VII, IX, X and contralateral ataxia
        Correct Answer:- Option-D

Question13:-Which of the following correlates with decorlicate rigidity?
        A:-It is always associated with pinpoint pupils
        B:-Lesion below the vestibular nucleus with facilitation of the ruprospinal tract
        C:-Lesion below the level of the red nucleus with facilitation of the vestibulospinal tract
        D:-Disinlibition of the red nucleus with facilitation of the rubrospinal tracts
        Correct Answer:- Option-D

Question14:-A 30-year-old lady is evaluated for recurrent episodes of severe, repetitive stabbing pain in the right forehead and temple that is associated with ipsilateral conjunctival injection, tearing and photophobia. The attacks last between1 and 2 minutes and occur atleast 100 times daily. Which of the following preventive measure is most likely to be effective in this patient?
        Correct Answer:- Option-C

Question15:-A 16 year old girl has 6 months history of early night-time episodes characterized by sudden arousal from sleep, lifting her head and dystonic posturing
of the extremities sometimes followed by getting out of bed and ambulating around the room for 1 to 2 minutes. There is a history of similar symptoms in her brother,
father and paternal grandmother. Mutation of which of the following genes is most likely to be present in this patient?
        A:-Cystatin B
        B:-GABA subunit
        C:-Leucine - rich glioma inactivated
        D:-Neuronal nicotinic acetylcholine receptor
        Correct Answer:- Option-D

Question16:-A 64-year-old man presents with a 1 year history of progressive memory loss and more recent onset of abnormal movements of his arms, legs and
trunk. Then he has obstructive sleep apnoea. His wife, who accompanies him describes odd movements during sleep in which he appears to be performing his
previous work activities, such as threading wires. On examination, he has a vertical gaze palsy. What is the most likely diagnosis?
        A:-Anti-CASPR2 encephalitis
        B:-Anti-EgLON 5 disease
        C:-Dentatorubrul - Pallidolusyian atrophy
        D:-Pantothenate kinase - associated neurodegeneration
        Correct Answer:- Option-B

Question17:-A girl presenting with DMD phenotype is most likely to be
        Correct Answer:- Option-B

Question18:-Which is a CSF synaptic biomarker for Alzheimer's disease?
        B:-A beta-42
        D:-Neurofilament light chain
        Correct Answer:- Option-B

Question19:-In which stage of intracerebral haemorrhage, both T1 and T2 MRI sequences are hyperintense?
        C:-Early subacute
        D:-Late subacute
        Correct Answer:- Option-D

Question20:-Which of the following is one among the earliest electrophysiological finding in Guillain Barre syndrome?
        A:-F wave impersistence
        B:-Conduction block
        C:-Prolonged distal latency
        D:-Absent SNAPs
        Correct Answer:- Option-A

Question21:-Ear of lynx sign is seen in
        A:-HSP type 3
        B:-HSP type 4
        C:-HSP type 11
        D:-HSP type 17
        Correct Answer:- Option-C

Question22:-False regarding QSART
        A:-Tests preganglionic sympathetic fibres
        B:-Stimulus is iontophoresis of acetylcholine
        C:-Response is sweat response
        D:-It is an axon reflex
        Correct Answer:- Option-A

Question23:-Changing the filter settings on the EEG allows for all of the following except:
        A:-Passing of signals above a certain frequency
        B:-Exclusion of signals below a certain frequency
        C:-Stopping a narrow band of frequencies
        D:-Preferential amplification of epiletiform discharges
        Correct Answer:- Option-D

Question24:-The intracellualr pathophysiological mechanism of an epiletiporm discharge is
        A:-Action potential
        D:-Paroxysmal depolarising shift
        Correct Answer:- Option-D

Question25:-Insular Lobe seizures are characterized by all except
        A:-Perioral warmth feeling
        B:-Hyper salivation
        C:-Laryngeal constriction
        D:-Complex gestural automatisms
        Correct Answer:- Option-D

Question26:-In Transient Global Aphasia (TGA), the following are correct except
        A:-Impairment in state of consciousness
        B:-No other signs of confusion
        C:-No overt seizure activity
        D:-Personal identification intact
        Correct Answer:- Option-A

Question27:-The anatomic structures of central importance in memory functions are the following except?
        A:-Entorhinal cortex
        C:-Midline nuclei of thalamus
        D:-Red nucleus
        Correct Answer:- Option-D

Question28:-The following is an example of implicit memory except?
        A:-Classic conditioning
        B:-Acquisition of new skills
        C:-Learning new material
        D:-Playing tennis
        Correct Answer:- Option-C

Question29:-The following are related to features of depression except?
        A:-Emotional blocking
        B:-Persecutory delusions
        C:-Agitation with pacing
        D:-Qualitative changes in intellectual function
        Correct Answer:- Option-D

Question30:-The following are examples of subcortical dementias except?
        A:-Progressive supranuclear palsy
        B:-Huntington's chorea
        C:-Parkinson's disease
        D:-Alzheimer's disease
        Correct Answer:- Option-D

Question31:-Following Hemtopoietic Stem Cell Transplantation (HSCT) the following neurological complication can occur except?
        A:-Herpes simplex encephalitis
        B:-Progressive multifocal leukoencephalopathy
        C:-Intra cranial haemorrage
        D:-Subarachnoid haemorrage
        Correct Answer:- Option-D

Question32:-The following drugs will produce cerebellar syndrome
        Correct Answer:- Option-D

Question33:-Botulinum toxin A bind to following synaptic vesicular protein
        A:-SNAP 25
        D:-Snare proteins
        Correct Answer:- Option-A

Question34:-Clinical improvement after the use of Edrophonium are seen in the following conditions except?
        A:-Botulinum intoxication
        B:-Snake bite
        C:-Organophosphate intoxication
        D:-Motor neuron disease
        Correct Answer:- Option-D

Question35:-Therapeutic options for ADEM are except
        B:-Plasma exchange
        C:-Intravenous immunoglobulin
        D:-Bone marrow transplantation
        Correct Answer:- Option-D

Question36:-Reye Syndrome is characterized by following except
        A:-Normal CSF
        B:-High serum concentration of liver enzymes
        C:-High serum ammonia
        D:-Oligo clonal bands
        Correct Answer:- Option-D

Question37:-Monoclonal antibodies are used in Multiple Sclerosis are the following except
        Correct Answer:- Option-D

Question38:-Evoked Potentials used in early stages of Multiple Sclerosis or spinal form of multiple sclerosis are following except
        A:-Readiness potential
        D:-Brain stem Auditory Evoked Response (BAER)
        Correct Answer:- Option-A

Question39:-Multiple Sclerosis in conjunction with peripheral neuropathy indicative of following except
        A:-Associated vitamin deficiency
        B:-Due to primary disease
        C:-Due to underlying disease such as AIDS
        D:-Due to plexopathy
        Correct Answer:- Option-B

Question40:-Recurrent myelitis at one level of spinal cord without any other signs of demyelinating disease can be found in except?
        A:-Multiple sclerosis
        B:-Behcet disease
        C:-Lupus erythematosus
        Correct Answer:- Option-B

Question41:-JC Virus causing Progressive Multifocal Leukoencephalopathy by attacking which cells of brain?
        B:-Glial cells
        C:-Ependymal cells
        D:-Stellate cells
        Correct Answer:- Option-B

Question42:-In SSPE histopathological changes are not observed in following part of brain?
        B:-Subcortical area
        C:-Brain stem
        Correct Answer:- Option-D

Question43:-Infections that follow neurosurgery or the insertion of cranial appliances are usually due to
        C:-Listeria Monocytogenes
        D:-H. Influenza
        Correct Answer:- Option-B

Question44:-Eosinophilic meningitis is caused by following except
        A:-Cholesterol emboli
        B:-Meningeal Hodgkin disease
        D:-Angiostrongylus cantonensis
        Correct Answer:- Option-C

Question45:-Drugs used for treating CNS toxoplasmosis are except
        A:-Oral Sulfadiazine
        Correct Answer:- Option-D

Question46:-CNS Aspergillosis can occur as a complication of the following except?
        A:-Chronic sinusitis
        B:-Skull base osteomyelitis
        Correct Answer:- Option-D

Question47:-Risk of stroke is implicated with polymorphism of which chromosome?
        Correct Answer:- Option-B

Question48:-CHADS2 and CHA2DS2-VASC are scoring system to predict the risk of stroke following
        A:-Atrial Fibrillation
        Correct Answer:- Option-A

Question49:-Hollenhorst plaque is get in which organ?
        A:-Internal carotid artery
        C:-Basilar artery
        Correct Answer:- Option-B

Question50:-Blood supply of Globus Pallidum is by
        A:-Anterior Choroidal artery
        B:-Posterior Choroidal artery
        C:-Middle Cerebral artery
        D:-Posterior cerebral artery
        Correct Answer:- Option-A

Question51:-Transmantle sign is seen in the Magnetic Resonance imaging of which of the following conditions causing epilepsy?
        A:-Focal Cortical Dysplasia Type I
        B:-Focal Cortical Dysplasia Type IIa
        C:-Focal Cortical Dysplasia Type IIb
        D:-Focal Cortical Dysplasia Type III
        Correct Answer:- Option-C

Question52:-Which among the following statements is/are true regardingresponsive Neurostimulation (RNS) treatment in epilepsy?
I.   It is synonymous with adaptive Deep Brain Stimulation
II. Epilepsy arising from loci in the eloquent cortex is the major contra-indication for RNS
III. It is a form of closed-loop neurostimulation
IV. Useful in those with poorly localised or multi-focal epilepsy in whom resective surgery is contra-indicated
        A:-Only statement III is correct
        B:-I and IV are correct
        C:-II and III are correct
        D:-III and IV are correct
        Correct Answer:- Option-A

Question53:-Synaptic Vesicle Protein 2A (SV2A) mediates the anticonvulsant effects of which of the following anti-seizure medications?
I.   Lacosamide
II. Levetiracetam
III. Rufinamide
IV. Stiripentol
        A:-I and III
        B:-II and IV
        C:-Only I
        D:-Only II
        Correct Answer:- Option-D

Question54:-Which among the following statements is true regarding Gastaut Syndrome?
        A:-Characterized by polymorphic and refractory seizures
        B:-Typical age of seizure onset is at 7-10 years of age
        C:-Callosotomy is the treatment of choice
        D:-Intellectual subnormality is seen in the majority of affected patients
        Correct Answer:- Option-B

Question55:-Epileptogenic Zone is most accurately defined as
        A:-The area of the cerebral cortex that is generating seizures
        B:-The zone that generates interictal epileptiform discharges
        C:-Zone whose resection is necessary and sufficient to eliminate seizures
        D:-The zone of the cortex that produces the seizure manifestations
        Correct Answer:- Option-C

Question56:-Mutations in which among the following genes is related to Autosomal Dominant Epilepsy with Auditory Features (ADEAF)?
        A:-GABA Receptor GABRA1 gene
        B:-GABA Receptor GABRG2 gene
        C:-Voltage Gated Potassium Channel KCNQ2 gene
        D:-Leucine-Rich Glioma Inactivated-1 (LG/1) gene
        Correct Answer:- Option-D

Question57:-Select the correct statement/statements regarding the physiology of sleep and wakefulness.
I.    Cholinergic neurons located close to mesopontine junction are involved in maintenance of wakefulness.
II.   The major neurotransmitter for sleep-wake cycle, hypocretin, comes from neurons in the tuberomammillary nucleus.
III. Neurons in the median preoptic nucleus are involved in the initiation of sleep.
IV.   Neurons in the lateral hypothalamus form the major source of histamine in the physiology of sleep
        A:-II and IV are correct
        B:-I and III are correct
        C:-Only II is correct
        D:-Only IV is correct
        Correct Answer:- Option-B

Question58:-Which among the following is classified as a parasomnia in the international classification of Sleep Disorders?
        A:-REM Sleep Behaviour Disorder
        B:-Restless Leg Syndrome
        C:-Narcolepsy Type 2
        D:-Kleine Levin Syndrome
        Correct Answer:- Option-A

Question59:-The "3P Model" or the Spielman model is the most widely accepted theoretical model of
        D:-None of the above
        Correct Answer:- Option-A

Question60:-The neurotoxin beta-N-oxalylamino-L-alanine (L-BOAA), responsible for lathyrism, is a:
        A:-NMDA Glutamate receptor agonist
        B:-GABA-A Receptor agonist
        C:-AMPA Glutamate Receptor agonist
        D:-GABA-B Receptor agonist
        Correct Answer:- Option-C

Question61:-Skewed Lyonization impacts the clinical manifestations of which among the following diseases?
        A:-Werdnig Hoffmann disease
        B:-Kennedy disease
        C:-Huntington's disease
        D:-Nijmegen breakage syndrome
        Correct Answer:- Option-B

Question62:-Which among the following statements regarding Upper Motor Neuron (UMN) Disorders is correct?
I.    Spastic Paraplegia 2 due to PLP1 mutations is inherited as an autosomal recessive condition.
II.   Konzo is a myelopathy predominantly seen in Tanzania and results from Human T Lymphotropic Virus Type 2 infection.
III. Primary Lateral Sclerosis is a diagnosis of exclusion.
IV.   Magnetic Resonance Spectroscopy shows reduction in choline relative to N-acetyl aspartate in patients with UMN syndrome in motor neuron disease.
        A:-I and II are correct
        B:-I and III are correct
        C:-Only IV is correct
        D:-Only III is correct
        Correct Answer:- Option-D

Question63:-Tofersen is a novel drug being tried in the treatment of Amyotrophic Lateral Sclerosis resulting from mutation in which among the following genes?
        Correct Answer:- Option-D

Question64:-Which among the following medications modulates the splicing of the Survival Motor Neuron 2 (SMN2) pre-mRNA?
        A:-Onasemnogene abeparvovec
        Correct Answer:- Option-C

Question65:-A 65-year-old lady comes to you with frequent headaches which are exclusively nocturnal. The headaches wake her up from sleep almost every night
around midnight. The headache may last from 20-30 minutes, sometimes up to 2 hours. No medical comorbidities were reported. No abnormalities were found on
clinical examination and baseline laboratory tests and MR imaging of brain were reported normal. Which among the following medications is likely to help this patient?
        A:-Calcitonin gene-related peptide antagonist
        D:-Calcitonin gene-related peptide agonist
        Correct Answer:- Option-B

Question66:-Which among the following neurons in the cerebellum are glutaminergic and excitatory?
        A:-Granule cells
        B:-Purkinje cells
        C:-Golgi cells
        D:-Basket cells
        Correct Answer:- Option-A

Question67:-All the following Spinocerebellar Ataxias (SCAs) are caused by polyglutamine-coding CAG repeat expansions, EXCEPT
        Correct Answer:- Option-C

Question68:-Select the correct statement/statements regarding autosomal recessive ataxias.
I.   In Ataxia Telangiectasia Like Disorder, serum Alphafeto Protein (AFP) levels are typically normal.
II. Serum AFP levels are elevated in Ataxia with Oculomotor Apraxia Type 2.
III. Lymphoid cancer risk in Ataxia with Oculomotor Apraxia Type 2 is even higher than Ataxia Telangiectasia.
IV. Ataxia with Oculomotor Apraxia Type 1 is associated with immunodeficiency.
        A:-Only II is correct
        B:-II and III are correct
        C:-III and IV are correct
        D:-I and II are correct
        Correct Answer:- Option-D

Question69:-The delayed-start study design is used to:
        A:-Evaluate synergistic effects of a study drug when combined with an established treatment
        B:-Separate disease-modifying effects of study-drug, from its effects on symptoms
        C:-Explore the long-term safety of medications which have been found to have beneficial effects in phase II trials
        D:-Test the efficacy of the study-drug in the pre-symptomatic phase of neurodegenerative diseases
        Correct Answer:- Option-B

Question70:-Which among the following "apraxias" is most likely to occur as a treatment-related complication in patients with Parkinson's diease undergoing bilateral subthalamic nucleus deep brain stimulation?
        A:-Oculomotor Apraxia
        B:-Apraxia of eye lid opening
        C:-Gait Apraxia
        D:-Melokinetic Apraxia
        Correct Answer:- Option-B

Question71:-Which among the following is a 3 Repeat (3-R) tauopathy?
        A:-Progressive Supranuclear Palsy
        B:-Corticobasal degeneration
        C:-Pick's disease
        D:-Globular Glial tauopathy
        Correct Answer:- Option-C

Question72:-The landmark clinical trial, PD-MED, whose results were published in 2014, compared which of the following therapies in Parkinson's disease?
        A:-Levodopa, Dopamine agonists and Monoamine Oxidase-B inhibitors
        B:-Levodopa with Entacapone Vs Levodopa without Entacapone
        C:-Best Medical Therapy Vs Deep Brain Stimulation
        D:-Best Medical Therapy Vs Apomorphine Pump
        Correct Answer:- Option-A

Question73:-What is the approximate penetrance in DYT-TOR1A (TOR-1A Associated dystonia)
        A:-Around 90%
        B:-Around 70%
        C:-Around 50%
        D:- Around 30%
        Correct Answer:- Option-D

Question74:-Which among the following is the most characteristic histological features linking the different types of Multiple System Atrophy?
        A:-Lewy Neurites
        B:-Tufted astrocytes
        C:-Globose neurofibrillary tangles
        D:-Glial cytoplasmic inclusions
        Correct Answer:- Option-D

Question75:-Ambroxol, which has been tried as a candidate drug for repurposing for the treatment of Parkinson's disease, acts on which of the following mechanisms?
        A:-Leucine-rich Repeat Kinase 2 (LRRK2) pathway
        B:-Glucocerebrosidase pathway
        C:-Post-translational modification of alpha synuclein
        D:-Mono-amine Oxidase pathway
        Correct Answer:- Option-B

Question76:-Identify the correct statement
        A:-The most common symptomatic peripheral nerve viral infection is with the neurotropic VZV
        B:-Recurrent cold-sensitive purpura, cutaneous vasculitis and mononeuritis multiplex could suggest chronic hepatitis C infection
        C:-Electrophysiology in Acute flaccid syndrome of West Nile Virus infection could mimic AMAN
        D:-All are correct
        Correct Answer:- Option-D

Question77:-Identify the incorrect combination.
        A:-Metronidazole - dose dependent painful, predominantly sensory polyneuropathy
        B:-Nitrofurantoin - sensorimotor distal axonal polyneuropathy
        C:-High dose Pyridoxine - sensory polyganglionopathy
        D:-Rapid acetylators - high risk for INH polyneuropathy
        Correct Answer:- Option-D

Question78:-Predominant upper limb motor involvement is the typical pattern in all except
        A:-Lewis- Sumner syndrome
        B:-Lead neuropathy
        C:-Multifocal Motor Neuropathy with Conduction Blocks
        D:-Familial Amyloid neuropathy
        Correct Answer:- Option-D

Question79:-A 32-year, premorbidly normal lady, developed gait unsteadiness followed by double vision and drooping of eyelids. She had mild sore throat a week
ago and clinical examination revealed bilateral external ophthalmoplegia, subtle bifacial weakness and ataxia. The most likely diagnosis.
        A:-Post viral cerebellitis
        B:-Miller Fisher syndrome
        C:-Bickerstaff Brain stem encephalitis
        D:-Myasthenia gravis
        Correct Answer:- Option-B

Question80:-In Hereditary Neuropathy with Liability to Pressure Palsies (HNPP), the most frequently affected never is
        A:-Fibular nerve
        B:-Ulnar nerve
        C:-Radial nerve
        D:-Median nerves
        Correct Answer:- Option-A

Question81:-Features of Musk-Antibody Myasthenia Gravis include all except
        A:-Predominant weakness in cranio-bulbar muscles, frequently with marked atrophy
        B:-Restricted distribution of electro-physiological abnormalities
        C:-Many MUSK-MG patients do not improve with cholinesterase inhibitors
        D:-Have prominent thymic changes
        Correct Answer:- Option-D

Question82:-"Ice pack test" in Myasthenia Gravis
        A:-Has high sensitivity and specificity
        B:-Has uncertain sensitivity and specificity
        C:-Low sensitivity and High specificity
        D:-Unreliable test in suspected MG
        Correct Answer:- Option-A

Question83:-Thymectomy for Myasthenia Gravis (MG)
        A:-Not recommended now in non-Thymoma MG due to better other treatment options
        B:-Recommended only in Thymoma associated MG
        C:-Better response in MUSK-MG, if done early
        D:-Thymectomy should be considered in all early-onset AChR MG patients
        Correct Answer:- Option-D

Question84:-Which of the following statement is wrong about Lambert-Eaton Myasthenia (LEM)?
        A:-The weakness in LEM is not usually life-threatening
        B:-SOX1 antibodies are a marker for underlying cancer in LEM patients
        C:-Anti VGCC Antibodies are usually negative Non-Cancer Associated LEM
        D:-All the statements are correct
        Correct Answer:- Option-C

Question85:-Incorrect statement about Congenital Myasthenic Syndrome
        A:-Most have Autosomal recessive inheritance
        B:-Ophthalmoparesis, ptosis and mild facial paresis are present in most cases during infancy
        C:-Skeletal deformities are common
        D:-Episodic respiratory crises occur only in choline acetylcholinesterase (ChAT) deficiency
        Correct Answer:- Option-D

Question86:-In the treatment of Duchenne Muscular Dystrophy (DMD) which of the following is incorrect?
        A:-Deflazacort delays progression of the disease
        B:-Consensus recommendations suggest initiating an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker by the age of 10
        C:-Eteplirsen offer curative therapy
        D:-Eteplirsen acts by inducing exon 51 skipping
        Correct Answer:- Option-C

Question87:-Sudden death secondary to fatal arrhythmias is common in
        A:-LGMD 1A
        B:-LGMD 1B
        C:-LGMD 1C
        D:-LGMD 1D
        Correct Answer:- Option-B

Question88:-Asymmetric weakness, deltoid sparing and Positive Beevor sign seen in
        B:-LGMD 1A
        C:-Myotonic Dystrophy Type 1
        D:-Miyoshi Myopathy
        Correct Answer:- Option-A

Question89:-Incorrect about Immune mediated Myopathy associated with AntiHMGCoA Reductase antibodies
        A:-Has LGMD phenotype
        B:-Rapid progression
        C:-Can occur as paraneoplastic myopathy
        D:-Immunotherapy is ineffective
        Correct Answer:- Option-D

Question90:-Muscle infiltration with aberrant large granular T lymphocytes may be seen in which of the following?
        A:-Inclusion Body Myositis
        D:-Immune mediated Necrotising Myopathy
        Correct Answer:- Option-A

Question91:-Which of the following is not known to produce 'secondary pseudotumor cerebri'?
Superior vena cava syndrome
        D:-Sulfa drugs
        Correct Answer:- Option-C

Question92:-Low cerebellar tonsils, crowding of the posterior fossa, small prepontine cistern and inferior displacement of the optic chiasm are seen in
        A:-Dandy Walker syndrome
        C:-Spontaneous spinal CSF leaks
        D:-All of the above
        Correct Answer:- Option-C

Question93:-Which of the following is not an anti-migraine agent?
        Correct Answer:- Option-C

Question94:-Identify the incorrect statement about SUNCT and SUNA.
        A:-Indomethacin effective in 50% of patients
        B:-Lamotrigine can be used as first line medication
        C:-SUNCT symptoms usually occur in V1 distribution
        D:-High flow oxygen inhalation cannot abort SUNA
        Correct Answer:- Option-A

Question95:-Pain felt deep in the auditory canal is suggestive of
        A:-Glossopahryngeal neuralgia
        B:-Geniculate neuralgia
        C:-Occipital neuralgia
        D:-Temporal arteritis
        Correct Answer:- Option-B

Question96:-Treatment for self-multilation in Autistic Spectrum Disorders include all of the following except
        Correct Answer:- Option-D

Question97:-Which of the following is not an atypical features in Dyslexia?
        A:-Male gender
        B:-Left handedness
        C:-Absence of family history
        Correct Answer:- Option-A

Question98:-Which of the following could be permitted for use in patients with Porphyria?
        D:-Methyl Dopa
        Correct Answer:- Option-B

Question99:-A 16-year-old was operated for vestibular schwannoma on both sides within a span of 2 years. What is ophthalmological finding most commonly encountered?
        B:-Retinal AV malformation
        C:-Posterior subcapsular cataracts
        D:-Lisch Nodules
        Correct Answer:- Option-C

Question100:-Which of the following is atypical for Panayiotopoulos Syndrome?
        A:-Autonomic manifestations
        B:-Prominent visual phenomena
        C:-Eye adversion
        D:-Generally good outcome
        Correct Answer:- Option-B

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