A 49-year-old man presents to the Emergency Department complaining of visual disturbance. Examination reveals a right incongruous homonymous hemianopia.
Where is the lesion most likely to be?
Correct Answer A:
Visual field defects: The main points for the exam are:
A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric. Please see the link for an excellent diagram.
Homonymous hemianopia:
Homonymous quadrantanopias*:
Bitemporal hemianopia:
*this is very much the 'exam answer'. Actual studies suggest that the majority of quadrantanopias are caused by occipital lobe lesions. Please see the following link for more details: http://www.ncbi.nlm.nih.gov/pubmed/9109741
Each one of the following is associated with Friedreich's ataxia, except:
Friedreich's ataxia:
Friedreich's ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterized by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich's ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipation.
The typical age of onset is 10-15 years old. Gait ataxia is the most common presenting feature.
Neurological features:
Other features:
A 61-year-old woman presents with bilateral tinnitus. She reports no change in her hearing or other ear-related symptoms. Ear and cranial nerve examination is unremarkable.
Which medication is she most likely to have recently started?
Correct Answer D:
Tinnitus:
Causes of tinnitus include:
Other causes include:
*10% of patients may have a 'flamingo tinge', caused by hyperaemia.
What is the most common clinical pattern seen in motor neuron disease?
Correct Answer E:
Motor neuron disease: types:
Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognized including amyotrophic lateral sclerosis, primary lateral sclerosis, progressive muscular atrophy and progressive bulbar palsy. In some patients however, there is a combination of clinical patterns.
Amyotrophic lateral sclerosis (50% of patients):
Primary lateral sclerosis:
Progressive muscular atrophy:
Progressive bulbar palsy:
Each one of the following is associated with ataxic telangiectasia, except:
Correct Answer C:
Ataxic telangiectasia:
Basics:
Features:
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