In a road traffic accident, a 34-year-old man sustains crush injury of the spine. One half of his spinal cord is damaged severely at the level of the tenth thoracic vertebra.
Which of the following is a feature of hemisection of the spinal cord?
B. Brown–Sequard syndrome is the result of hemisection of the spinal cord. This consists of: (1) loss of contralateral pain and temperature due to interruption of the crossed spinothalamic tract and (2) loss of ipsilateral proprioception below the level of the lesion due to involvement of the ascending fi bres in the posterior columns. It is also associated with ipsilateral spastic weakness due to involvement of the descending corticospinal tract. The ‘sensory level’ is usually one or two segments below the level of the lesion.
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A patient presents in an agitated state with increased sweating and tremors. On examination she has signs of Grave’s disease.
Which is the commonest sign noted in thyroid ophthalmopathy?
B. Lid retraction is the most common clinical feature of Grave’s ophthalmopathy. The associated extraocular myopathy is attributed to inflammation and fibrosis of muscles. Inferior rectus is most commonly involved and lateral rectus is the least involved of all extraocular muscles. Diplopia may be especially worse early in the day. Other signs include orbital congestion, lid lag on looking down, proptosis, conjunctival injection, and optic neuropathy due to compression of the optic nerve by enlarged extraocular muscles in the orbital apex.
Headache associated with ipsilateral nasal congestion, rhinorrhea, lacrimation, redness of the eye is characteristic of which of the following?
C. Cluster headache is considered as a vascular headache syndrome. It is usually episodic in nature and is characterized by attacks of acute, periorbital pain. This pain is often deep and excruciating but rarely pulsatile. It occurs almost every day over a 4–8 week period, followed by a pain-free interval that averages a year. Attacks last from 30 minutes to 2 hours. It is often associated with lacrimation, reddening of the eye, nasal stuffiness, lid ptosis, and nausea. It is common in men aged 20 to 50 years. Propranolol and amitriptyline are ineffective. Lithium is beneficial for cluster headache though ineffective for migraine.
Epilepsy associated with learning disability, shagreen patches, and ash leaf macules are seen in which of the following?
B. Tuberous sclerosis is a congenital disease where hyperplasia of ectodermal and mesodermal cells leads to various lesions in the skin, nervous system, heart, kidney and other organs. It is characterized clinically by the triad of adenoma sebaceum, epilepsy, and mental retardation. Hypomelanotic skin macules (ash-leaf lesions) and subepidermal fibroses (shagreen patches) are other associated skin lesions noted in tuberous sclerosis. Neurofibromatosis is a hereditary neoplastic syndrome where benign tumours of the skin, nervous system, bones, and endocrine organs are seen. Café au lait spots are characteristic, coffee-coloured skin patches seen in neurofibromatosis. In Sturge–Weber syndrome (encephalotrigeminal angiomatosis), facial port wine stain associated with cerebral angiomatosis is seen. Fabry’s disease is a glycogen storage disease.
Parkinsonian features associated with downward gaze palsy and pseudobulbar dysarthria is characteristic of which of the following conditions?
D. Progressive supranuclear palsy is a degenerative neurological disease with parkinsonian symptoms as a prominent clinical feature. It is characterized by axial akinetic rigidity, dizziness, unsteadiness, falls, and pseudobulbar dysarthria. Eye movement abnormalities affecting down gaze occur first, followed by variable limitations of upward and horizontal eye movement. Doll’s eye movements are preserved as the brain stem is intact. Upper motor neurone signs and occasionally cerebellar signs may be present. Dementia is a common sequel.
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