PRESENTING COMPLAINT
67 year-old gentleman with PMH of T2 diabetes, HTN, OA, bilateral knee replacement (2009) is referred to neurology services for evaluation of difficulty walking and slow progress in mobility after his knee surgery.
HISTORY OF PRESENTING COMPLAINT
The patient had 2 falls in the past 2 months. Progress with physios is very slow. Referred to his GP because of slow movement and slurred speech and symptoms of urinary frequency. GP prescribed antifungal treatment (no idea why? apparently his GP doesn't know that neurology exists;)
A month later, the patient experienced rapid decline of his mobility needing a walking stick initially and using a wheelchair currently. In his letter to neurology, the GP describes that patient is feeling his foot is glued to the floor and has enormous difficulty lifting it off.
Patient has occasional choking on bread and weight loss of 1 Kg over 6 months. But no smell, visual or taste problems. No problems with chewing, sleep disturbance. Upon further questioning, the patient also denies experiencing postural dizziness and there is no sudden bouts of uncontrollable laughter or crying (what do neurologists call this symptom? -Hint: common in patients with stroke)
OTHER HISTORY
Drug History - aspirin, amlodipine, omerprazole, insulin homologue
Family History - irrelevant
Social History - ex-smoker, drinks no alcohol
ON EXAMINATION
The patient appeared relaxed and slightly underdressed. Despite a room full of neurologists, he was very unnerved, and was joking and laughing. Dealing with audience questions, he would answer the question and then say 'Next'!!
His speech is slurred and dysarthric, better described as strangulated speech with words produced from the back of the mouth. In addition, there is an element of staccato speech when asked to say 'British constitution' or 'West Register Street'.
Gait- magnetic with a degree of stamping and difficulty of initiation. Stride is really slow at start, but improved slightly with 3rd and 4th step with increasing speed. Postural instability with stooped posture. Normal base.
CRANIAL NERVES
Normal range of eye movements. No restriction of upward gaze. No diplopia, squint or ptosis. Saccadic eye movement is normal. facial and mastication muscles are not weak or wasted. Absent jaw jerk. No wasting, fasciculation or weakness of the tongue. Rest of exam is also normal.
UPPER AND LOWER LIMB NEURO EXAM
Wasting and fasciculation of right wrist extensors. Tone is increased in upper and lower limbs - it is velocity dependent with a spastic catch. There is mild cogwheel rigidity in upper limbs. Global hyperreflexia (more predominant on R>L upper limbs). Positive Hoffman's reflex. No clonus and upper plantars are equivocal.
Finger-thumb test showed time-dependent reduction of velocity and amplitude of movement with 'fatiguability'.
No dysmetria, intention or resting tremors, dysdiadokokinesia or nystagmus.
INVESTIGATIONS
A number of investigations were performed. Results of investigations are provided upon your request.
What is the diagnosis? and Why?
Hint: Start by naming pathways in the nervous system, the involvement of which may account for this patients signs. Findings from the neuro exam should allow you to localize the lesion.
