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An illness presenting as rapidly developing dementia, pyramidal and extrapyramidal motor findings, and myoclonus in a 60-year-old male patient is likely to be: (PGI)

1. Wilson’s disease

2. Normal pressure hydrocephalus

3. Acute intermittent porphyria

4. Creutzfeldt-Jakob disease

Clinically, Creutzfeldt-Jakob disease presents with a rapidly progressive dementia (most affected patients die within one year from clinical onset) associated with myoclonus of the limbs, cerebellar ataxia, focal cortical signs, and rigidity. It is incurable and invariably fatal.

Normal pressure hydrocephalus presents with features like gait ataxia and urinary incontinence, in addition to dementia. Acute intermittent porphyria presents with abdominal pain and peripheral neuropathy, in addition to psychiatric symptoms. Wilson’s disease manifests as neurological or psychiatric symptoms and liver disease.

Prusiner S.B.: Shattuck lecture–neurodegenerative diseases and prions. N Engl J Med. 344:1516-1526 2001.

Seeley WW, Miller BL. Chapter 371. Dementia. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 18^th edition; 2012.

A 45-year-old man presents with history of frequent falls and difficulty in looking down. What is the most probable diagnosis? (AIIMS)

1. Normal pressure hydrocephalus

2. Parkinson’s disease

3. Alzheimer’s disease

4. Progressive supranuclear palsy

Progressive supranuclear palsy is a progressive neurodegenerative disease frequently associated with impaired downward (vertical) gaze and dystonia in extension. These features make patients susceptible to frequent falls.

Parkinson’s disease is characterized by a combination of tremor, rigidity, and bradykinesia. Alzheimer’s disease is characterized by progressive memory loss. A triad of dementia, urinary incontinence, and gait ataxia characterizes normal pressure hydrocephalus.

Ropper AH, Samuels MA, Klein JP. Chapter 39. Degenerative Diseases of the Nervous System. In: Ropper AH, Samuels MA, Klein JP. eds. Adams & Victor’s Principles of Neurology, 10^th edition; 2014.

Jankovic J. Chapter 71. Movement Disorders. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC. eds. Bradley’s Neurology in Clinical Practice, 6^th edition; 2012.

The preferred therapy for a 40-year-old patient with suspected community-acquired pyogenic meningitis is: (PGI)

1. Ceftriaxone plus vancomycin

2. Ampicillin plus gentamycin

3. Ceftriaxone plus gentamycin

4. Ampicillin plus vancomycin

Commonly observed pathogens at this age include Streptococcus pneumonia and Neisseria meningitidis. The recommended therapy for this patient is vancomycin plus ceftriaxone or cefotaxime or cefepime.

Chaudhuri A, Martinez-Martin P, Kennedy PG, et al. EFNS guideline on the management of community-acquired bacterial meningitis: report of an EFNS Task Force on acute bacterial meningitis in older children and adults. Eur J Neurol. Jul 2008;15(7):649-59.

Koshy A, Roos K. Chapter 53C. Infections of the Nervous System: Bacterial and Fungal. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC. eds. Bradley’s Neurology in Clinical Practice, 6th edition; 2012.

When speech is nonfluent, dysarthric, laborious, agrammatical and telegraphic, the lesion is in the: (AIIMS)

1. Posterior superior part of temporal lobe

2. Posterior inferior frontal gyrus

3. Parietal lobe

4. Anterior part of occipital lobe

The three main types of aphasias are Broca’s aphasia, Wernicke’s aphasia, and conduction aphasia. Of these, Broca’s aphasia is characterized by impaired fluency, impaired repetition, and relatively preserved comprehension. The patient with Broca’s aphasia often uses the principal meaning-containing nouns and verbs but without small grammatical words. This pattern is called agrammatism or ‘telegraphic speech.’ The lesion responsible for Broca’s aphasia usually includes the Broca area in the posterior part of inferior frontal gyrus.

Howard S. Kirshner H S. Chapter 12A. Language and Speech Disorders: Aphasia and Aphasic Syndromes. In: Daroff RB, Fenichel GM, Jankovic J, John C. Mazziotta JC. eds. Bradley’s Neurology in Clinical Practice, 6^th edition; 2012.

Ropper AH, Samuels MA, Klein JP. Chapter 23. Disorders of Speech and Language. In: Ropper AH, Samuels MA, Klein JP. eds. Adams & Victor’s Principles of Neurology, 10^th edition; 2014.

Dementia, prominent Parkinsonian features, visual hallucinations, fluctuation in sensorium, and behavior are typically seen in: (AIIMS)

1. Alzheimer’s disease

2. Frontotemporal dementia

3. Huntington’s disease

4. Lewy body dementia

Dementia with Lewy bodies (DLB) is a progressive, degenerative dementia of unknown etiology. DLB is characterized by fluctuating cognition and alertness, recurrent visual hallucinations (observed in 75% of patients), and motor features of Parkinsonism. Other symptoms include REM sleep behavior disorder (also known as RBD) and sensitivity to neuroleptic medications.

McKeith IG, Ballard CG, Perry RH, et al. Prospective validation of consensus criteria for the diagnosis of dementia with Lewy bodies. Neurology. Mar 14 2000;54(5):1050-8.

Seeley WW, Miller BL. Chapter 371. Dementia. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 18^th edition; 2012.

A 40-year-old patient had hemiparesis, hemisensory loss, and homonymous hemianopia but does not describe any deficit or rather denies it. He is most likely to have lesion in the: (AIIMS)

1. Right hemisphere

2. Left hemisphere

3. Both hemispheres

4. Basal ganglia

This patient is suffering from anosognosia. It is a condition in which a person who suffers a certain disability seems unaware of the existence of his or her disability. It results from damage to the parietal lobe in the right (non-dominant) hemisphere.

Vallar, Giuseppe; Ronchi, Roberta (2006). "Anosognosia for motor and sensory deficits after unilateral brain damage: A review". Restorative Neurology and Neuroscience 24 (4–6): 247–57.

Ropper AH, Samuels MA, Klein JP. Chapter 22. Neurologic Disorders Caused by Lesions in Specific Parts of the Cerebrum. In: Ropper AH, Samuels MA, Klein JP. eds. Adams & Victor’s Principles of Neurology, 10^th edition; 2014.

A 15-year-old female patient develops loss of appetite and dry cough for 10 days, followed by neck rigidity. CSF examination shows glucose 40 mg%, protein 150 mg%, and chloride 52 meq/L, with an increased number of lymphocytes. The likely diagnosis is: (AIIMS)

1. Tubercular meningitis

2. Brain abscess

3. Bacterial meningitis

4. Viral encephalitis

Tubercular meningitis shows lymphocytic pleocytosis, high protein, and low sugar on CSF examination. Loss of appetite and cough favor the diagnosis of tuberculosis.

Typical CSF findings of pyogenic bacterial meningitis are low sugar, high proteins, and polymorphonuclear pleocytosis. Viral encephalitis often produces signs of altered sensorium, while brain abscess often produces focal neurological deficits.

Thwaites GE. Advances in the diagnosis and treatment of tuberculous meningitis. Curr Opin Neurol. Mar 12 2013

Ropper AH, Samuels MA, Klein JP. Chapter 32. Infections of the Nervous System (Bacterial, Fungal, Spirochetal, Parasitic) and Sarcoidosis. In: Ropper AH, Samuels MA, Klein JP. eds. Adams & Victor’s Principles of Neurology, 10^th edition; 2014.

A condition with adenoma of face, seizures, and dementia, usually beginning in childhood is: (AIIMS)

1. Obstructive hydrocephalus

2. Bromism

3. Porphyria

4. Tuberous sclerosis

Tuberous sclerosis is characterized by the presence of seizures, mental retardation, and skin changes. Typical skin changes include adenoma sebaceum (small erythematous papules on the nose and cheeks of a child, representing angiofibromata), ash leaf spot, shagreen patch, and periungual fibromas. Other features include cortical tubers, subependymal giant cell astrocytomas, renal angiomyolipoma etc.

Crino P, Nathanson K, Henske E (2006). "The Tuberous Sclerosis Complex". New England Journal of Medicine 355 (13): 1345–56.

Islam MP, Roach ES. Chapter 65. Neurocutaneous Syndromes. In: Daroff RB, Fenichel GM, Jankovic J, John C. Mazziotta JC. eds. Bradley’s Neurology in Clinical Practice, 6^th edition; 2012.

Which one of the following is an example of a trinucleotide repeat disorder? (PGI)

1. Huntington disease

2. Ataxia telengiectasia

3. Wiskott Aldrich syndrome

4. Tuberous sclerosis

Important causes of trinucleotide repeat disorders include myotonic dystrophy type 1, fragile X syndrome, spinocerebellar ataxia (type 1, 2, 3, 6, 7 and 12), Huntington’s disease, Friedreich’s ataxia, and dentorubral pallidoluysian atrophy.

Mirkin SM. Expandable DNA repeats and human disease. Nature. 2007;447:932–940.

Jameson J, Kopp P. Chapter 61. Principles of Human Genetics. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 18^th edition; 2012.

Which of the following is the treatment of choice in bacterial brain abscess? (AIIMS)

1. Antibiotics alone

2. Antibiotics with steroids

3. Surgery alone

4. Antibiotics with surgery

A brain abscess is a focal, suppurative infection within the brain parenchyma, typically surrounded by a vascularized capsule. The classical presentation consists of a triad of fever, headache, and focal neurological deficit. Surgical excision or drainage, combined with prolonged antibiotics (usually about 2 months), remains the treatment of choice. The use of steroids in bacterial abscess is controversial, and they are usually avoided.

Muzumdar D, Jhawar S, Goel A. Brain abscess: an overview. Int J Surg. 2011;9(2):136-44.

Roos KL, Tyler KL. Chapter 381. Meningitis, Encephalitis, Brain Abscess, and Empyema. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 18^th edition; 2012.

Choreiform movements, dementia in adult life, and similar symptoms in family members make the diagnosis of: (AIIMS)

1. Sydenham’s chorea

2. Habit spasms

3. Huntington’s chorea

4. Dystonia musculorum deformans

Huntington’s chorea is characterized by the presence of movement disorders, dementia, and psychiatric disturbances. This neurodegenerative genetic disorder is progressive and shows autosomal dominant pattern of inheritance.

Sydenham’s chorea is rheumatic chorea (autoimmune), while habit spasms are also known as tics. Dystonia musculorum deformans has prominent dystonia and is seen during childhood.

Ropper AH, Samuels MA, Klein JP. Chapter 39. Degenerative Diseases of the Nervous System. In: Ropper AH, Samuels MA, Klein JP. eds. Adams & Victor’s Principles of Neurology, 10^th edition; 2014.

Jankovic J. Chapter 71. Movement Disorders. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC. eds. Bradley’s Neurology in Clinical Practice, 6^th edition; 2012.

The preferred therapy for a 70-year-old patient with suspected community-acquired pyogenic meningitis is: (PGI)

1. Ceftriaxone plus vancomycin plus ampicillin

2. Ampicillin plus gentamycin plus ceftriaxone

3. Ceftriaxone plus gentamycin

4. Ampicillin plus vancomycin

Commonly observed pathogens in elderly patients include S. pneumoniae, N. meningitidis, L. monocytogenes, and aerobic gram-negative rods. The recommended therapy is vancomycin plus ceftriaxone or cefotaxime or cefepime. Further, ampicillin is added to provide adequate coverage against listeria infection.

Rapid, repetitive, coordinated, and stereotyped movements, most of which can be mimicked, are known as: (AIIMS)

1. Chorea

2. Ballismus

3. Athetosis

4. Tics