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Neurology (Expert Forum)
Are Symptoms Consistant With BCGD?
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Are Symptoms Consistant With BCGD?
by Archbishop, Aug 31, 2006 12:00AM
FOLLOW-UP TO: "Slow onset of left foot 'Sticking' to floor' - archbishop: 08/02/2006"
When I asked my mom's neuro to perform EMG & Lumbar MRI, she said that signs & symptoms doesn't fit a "possible L5 radiculopathy" at all, but ordered a Brain SPECT instead for this 78 yr female with history of progressive left lower extremity stiffness & weakness. Here's FINDINGS: Tomographic images demonstrate decreased perfusion in the frontal lobes extending to the frontal parietal region with relative sparing of the prefrontal region. There is somewhat decreased perfusion of the basal ganglia bilaterally. The ventricles and other CSF containing spaces are diffusely symmetrically promnent consistent with diffuse brain atrophy.
IMPRESSION: Bilateral hypoperfusion in a pattern suggestive of either frontal or corticobasal degenerative process.
My mother's neurologist tells us that this means EITHER frontal dementia (ruled out as mom is sharp as a tack) or BCGD "even though Mom DOES NOT have vast majority of BCGD symptoms, it's my gut feeling she has BCGD", Neuro said.
NOW, Mom can move her left leg extremely well while in bed or sitting in chair. BUT, once standing, leg "stiffens and turns to cement." WHY can she move it freely in bed/sitting, but not standing and is this part of BCGD? Exercise/limbering up in AM helps her for most of day, then turns to stone after much standing. Is this BCGD? Can this be anything else? Should we get other tests? Get 2nd opinion? What's BCGD Prognosis in general? IF BCGD, what else can we do to help? PLEASE HELP ME TO HELP MY MOM! Thanx!
When I asked my mom's neuro to perform EMG & Lumbar MRI, she said that signs & symptoms doesn't fit a "possible L5 radiculopathy" at all, but ordered a Brain SPECT instead for this 78 yr female with history of progressive left lower extremity stiffness & weakness. Here's FINDINGS: Tomographic images demonstrate decreased perfusion in the frontal lobes extending to the frontal parietal region with relative sparing of the prefrontal region. There is somewhat decreased perfusion of the basal ganglia bilaterally. The ventricles and other CSF containing spaces are diffusely symmetrically promnent consistent with diffuse brain atrophy.
IMPRESSION: Bilateral hypoperfusion in a pattern suggestive of either frontal or corticobasal degenerative process.
My mother's neurologist tells us that this means EITHER frontal dementia (ruled out as mom is sharp as a tack) or BCGD "even though Mom DOES NOT have vast majority of BCGD symptoms, it's my gut feeling she has BCGD", Neuro said.
NOW, Mom can move her left leg extremely well while in bed or sitting in chair. BUT, once standing, leg "stiffens and turns to cement." WHY can she move it freely in bed/sitting, but not standing and is this part of BCGD? Exercise/limbering up in AM helps her for most of day, then turns to stone after much standing. Is this BCGD? Can this be anything else? Should we get other tests? Get 2nd opinion? What's BCGD Prognosis in general? IF BCGD, what else can we do to help? PLEASE HELP ME TO HELP MY MOM! Thanx!
Doctor's Answer
by CCF-Neuro-M.D.-SH, Sep 01, 2006 12:00AM
First of all, keep in mind that I am unable to diagnose you because I am unable to examine you, this forum is for educational purposes.
The symptoms you describe could suggest dystonic posturing of the left lower leg, that seems to be at least sometimes action induced. This could the early onset of a condition such as Cortical Basal Ganglionic Degeneration (CBGD), Parkinsons plus syndrome, or a primary dystonia. The SPECT scan is not usually the first step in the process of sorting this out, but the findings of your SPECT are suggestive of possible Cortical Basal Ganglionic Degeneration (CBGD). I would recommend getting an MRI/MRA of the brain, to evaluate for alternative causes, for example stroke, etc. Screening blood tests should include B12, RPR, TSH, Ceruloplasmin, ESR, CBC, electrolytes and copper/zinc. You may also consider formal neuro-psych testing, they have tests that specifically evaluate for frontal lobe dysfunction, that we would not recognize in everyday interactions. The typical approach to her dystonia would also include a trial on sinemet (levadopa/carbidopa). An EMG and lumbar puncture would also be reasonable tests if the above is not helpful. If you need to seek a second opinion to get the required tests to rule out other disorders besides CBGD, than do so, otherwise your neurologist will likely be willing to help. The prognosis of CBGD is not good, with a progressive loss of motor and cognitive skills over 3-6 years, with death usually occuring due to pneumonia or other secondary cause (Which is why we look for other more treatable diseases than CBGD). If you do seek a second opinion, I would see a neurologist that specializes in movement disorders.
I hope this has been helpful.
The symptoms you describe could suggest dystonic posturing of the left lower leg, that seems to be at least sometimes action induced. This could the early onset of a condition such as Cortical Basal Ganglionic Degeneration (CBGD), Parkinsons plus syndrome, or a primary dystonia. The SPECT scan is not usually the first step in the process of sorting this out, but the findings of your SPECT are suggestive of possible Cortical Basal Ganglionic Degeneration (CBGD). I would recommend getting an MRI/MRA of the brain, to evaluate for alternative causes, for example stroke, etc. Screening blood tests should include B12, RPR, TSH, Ceruloplasmin, ESR, CBC, electrolytes and copper/zinc. You may also consider formal neuro-psych testing, they have tests that specifically evaluate for frontal lobe dysfunction, that we would not recognize in everyday interactions. The typical approach to her dystonia would also include a trial on sinemet (levadopa/carbidopa). An EMG and lumbar puncture would also be reasonable tests if the above is not helpful. If you need to seek a second opinion to get the required tests to rule out other disorders besides CBGD, than do so, otherwise your neurologist will likely be willing to help. The prognosis of CBGD is not good, with a progressive loss of motor and cognitive skills over 3-6 years, with death usually occuring due to pneumonia or other secondary cause (Which is why we look for other more treatable diseases than CBGD). If you do seek a second opinion, I would see a neurologist that specializes in movement disorders.
I hope this has been helpful.
Though CBD (the new name for CGBD)is difficult to diagnose clinically and the known symptomes (the major or minor groups)are not very sensitive, but what you are describing is not any thing like regidity,apraxia, or what we could call alien limb were the foot would act on its own in a weird way.
These are the symptomes/sign of CBD
Major
• Asymmetric parkinsonism
• Asymmetric cortical signs (dysphasia, apraxia, astereognosis, impaired graphesthesia)
• Asymmetric dystonia/action and postural tremor/myoclonus
• “Alien limb” phenomenon
Minor
• Intellectual decline
• Frontal release signs
• Supranuclear gaze problems
• Dysphagia and dysarthria
• Pyramidal signs
So I think what you are describing could be a stiff limb syndrome which is a sub category of a stiff person syndrome.
Regards,
Bob
I would hope your original neuro did rule this out. I am still not sure why your original neuro did not do the emg? This I would think would completely at least rule in or out a radiculopathy or compression issue vs. "something higher up". The common peroneal nerve gets easily compressed when one with edema starts walking. The anterior leg muscles swell and tighten up on the nerve. The leg becomes very weak and the foot will drop. You can check for tenderness of this nerve when she is standing and look for a Hoffman tinels sign when the nerve is tapped. (tingling down leg or in top of foot).
good luck