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Corticobasal Degeneration Substantiated by Imaging Studies
LETTER Corticobasal Degeneration Substantiated by Imaging Studies
T
O THE EDITOR: Corticobasal degeneration (CBD) is a slowly progressive disorder, characterized by initially unilateral akinesia, rigidity, apraxia, cortical sensory impairment, postural instability, and the alien hand syndrome.1–3 The diagnosis can be challenging, given the variable presentations that reflect which brain region is affected first. Neuropathology is said to provide a definitive diagnosis, with neuronal achromasia and cortical atrophy of the frontoparietal region.1,3 SPECT can show asymmetrical parietal hypoperfusion,2,4,5 and focal atrophy can be seen on MRI.6 We describe a case of CBD in FIGURE 1.
which the patient presented with few and mild symptoms, and where, over time, imaging studies were helpful diagnostic adjuncts. A 67-year-old right-handed man presented in late 2001 with a 2-year history of gradual functional decline. His difficulties started with pronouncing words (“words wouldn’t come out”), but without other language problems. Later, he avoided talking to strangers and answering the phone, and was increasingly impaired in fine motor movements of both hands (especially the right) and by a fine motor tremor. He was unable to button his shirt or write, although he could still type. His medical history included antiphospholipid syndrome, esophageal stricture, and spinal stenosis. On examination, he had oral-buccal apraxia and apraxia of fine finger movements,
with mild cogwheel rigidity and tremor. A December 2000 MRI brain scan had shown mild, periventricular white-matter ischemic changes and mild global atrophy. Neuropsychological testing (2002) revealed only mild apraxia. A July 2003 brain SPECT perfusion scan, using 99m-Technetium ethylene cysteine, demonstrated moderately decreased perfusion in the left parietal lobe (Figure 1). In November 2003, MRI showed bilateral (left ⬎ right) temporo-parietal lobe atrophy. Subsequently, his disease showed more typical CBD progression, with more apraxia (worse on the right) and worse parkinsonism, including a disabling tremor. This case differed from “classic” CBD, with, initially, mild and predominantly cortical features. This presentation was puzzling, and al-
Nuclear Medicine Brain SPECT Scan Obtained 4 Months Before the Follow-Up MRI (A) Axial and (B) Coronal Images
A
B
Note: There is a region of moderately decreased perfusion involving the left parietal lobe (arrows).
Am J Geriatr Psychiatry 13:4, April 2005
333
Erratum though the combination of fine motor and oral apraxia suggested CBD, SPECT imaging was especially helpful in substantiating a clinical diagnosis that has now become more evident. SPECT findings in CBD are variable, but include asymmetric fronto-temporal perfusion and variable prefrontal involvement.1,4,5 Here, the presence of isolated, asymmetric hypoperfusion contralateral to clinical findings and the subsequently neurodegenerative (compared with ischemic) course are highly suggestive of CBD. MRI findings in CBD include asymmetric parietal or fronto-parietal atrophy, but there is considerable overlap between MRI profiles of CBD and related “atypical” parkinsonian syndromes.6 Here, after only 3 years, the MRI showed atrophy in the left temporo-parietal region, corresponding to earlier SPECT findings. Our findings must be interpreted with caution. In addition to the
usual limitations of case reports, our patient is still alive, so that neuropathological confirmation is not available. Of note, however, is the fact that even patients with a typical course do not always meet neuropathological criteria, which has led to the proposal that “CBD syndrome” is a better term,3 and suggests that the diagnosis might best be confirmed by careful follow-up, which, in this case, will include further anatomic and functional-imaging studies.
Jennifer Martin, M.D. Gilbert Matte, Ph.D. David Barnes, M.D. Steven Burrell, M.D. Department of Radiology Dalhousie University Halifax, Nova Scotia, Canada Sultan Darvesh, M.D., Ph.D. Kenneth Rockwood, M.D. Department of Medicine Divisions of Geriatric Medicine and Neurology
Dalhousie University Halifax, Nova Scotia, Canada
References 1. Mathuranath PS, Xuereb JH, Bak T, et al: Corticobasal ganglionic degeneration and/or frontotemporal dementia? J Neurol Neurosurg Psychiatry 2000; 68:304– 312 2. Graham NL, Bak TH, Hodges JR: Corticobasal degeneration as a cognitive disorder. Mov Disord 2003; 18:1224–1232 3. Doran M, du Plessis DG, Enevoldson TP, et al: Pathological heterogeneity of clinically diagnosed corticobasal degeneration. J Neurol Sci 2003; 216:127–134 4. Slawek J, Lass P, Derejko M, et al: Cerebral blood flow SPECT may be helpful in establishing the diagnosis of progressive supranuclear palsy and corticobasal degeneration. Nuclear Medicine Review Central Eastern Europe 2001; 4:73–76 5. Zhang L, Murata Y, Ishida R, et al: Differentiating between progressive supranuclear palsy and corticobasal degeneration by brain perfusion SPECT. Nucl Med Commun 2001; 22:767–772 6. Yekhlef F, Ballan G, Macia F, et al: Routine MRI for the differential diagnosis of Parkinson’s disease, MSA, PSP, and CBD. J Neural Transm 2003; 110:151–169
ERRATUM
I
n the November–December 2004 issue of the Journal (12:613–620), the article “Hippocampal Volume Measurement in Older Adults With Bipolar Disorder,” by John L. Beyer et al., cited an article by Noga JT et al., stating that the authors had found increased
334
hippocampal volume size in bipolar patients. The authors, in fact, found decreased right-hippocampal volumes in the monozygotic twin affected with bipolar disorder. Dr. Beyer regrets this error and appreciates Dr. Noga’s bringing it to our attention.
Am J Geriatr Psychiatry 13:4, April 2005
T
O THE EDITOR: Corticobasal degeneration (CBD) is a slowly progressive disorder, characterized by initially unilateral akinesia, rigidity, apraxia, cortical sensory impairment, postural instability, and the alien hand syndrome.1–3 The diagnosis can be challenging, given the variable presentations that reflect which brain region is affected first. Neuropathology is said to provide a definitive diagnosis, with neuronal achromasia and cortical atrophy of the frontoparietal region.1,3 SPECT can show asymmetrical parietal hypoperfusion,2,4,5 and focal atrophy can be seen on MRI.6 We describe a case of CBD in FIGURE 1.
which the patient presented with few and mild symptoms, and where, over time, imaging studies were helpful diagnostic adjuncts. A 67-year-old right-handed man presented in late 2001 with a 2-year history of gradual functional decline. His difficulties started with pronouncing words (“words wouldn’t come out”), but without other language problems. Later, he avoided talking to strangers and answering the phone, and was increasingly impaired in fine motor movements of both hands (especially the right) and by a fine motor tremor. He was unable to button his shirt or write, although he could still type. His medical history included antiphospholipid syndrome, esophageal stricture, and spinal stenosis. On examination, he had oral-buccal apraxia and apraxia of fine finger movements,
with mild cogwheel rigidity and tremor. A December 2000 MRI brain scan had shown mild, periventricular white-matter ischemic changes and mild global atrophy. Neuropsychological testing (2002) revealed only mild apraxia. A July 2003 brain SPECT perfusion scan, using 99m-Technetium ethylene cysteine, demonstrated moderately decreased perfusion in the left parietal lobe (Figure 1). In November 2003, MRI showed bilateral (left ⬎ right) temporo-parietal lobe atrophy. Subsequently, his disease showed more typical CBD progression, with more apraxia (worse on the right) and worse parkinsonism, including a disabling tremor. This case differed from “classic” CBD, with, initially, mild and predominantly cortical features. This presentation was puzzling, and al-
Nuclear Medicine Brain SPECT Scan Obtained 4 Months Before the Follow-Up MRI (A) Axial and (B) Coronal Images
A
B
Note: There is a region of moderately decreased perfusion involving the left parietal lobe (arrows).
Am J Geriatr Psychiatry 13:4, April 2005
333
Erratum though the combination of fine motor and oral apraxia suggested CBD, SPECT imaging was especially helpful in substantiating a clinical diagnosis that has now become more evident. SPECT findings in CBD are variable, but include asymmetric fronto-temporal perfusion and variable prefrontal involvement.1,4,5 Here, the presence of isolated, asymmetric hypoperfusion contralateral to clinical findings and the subsequently neurodegenerative (compared with ischemic) course are highly suggestive of CBD. MRI findings in CBD include asymmetric parietal or fronto-parietal atrophy, but there is considerable overlap between MRI profiles of CBD and related “atypical” parkinsonian syndromes.6 Here, after only 3 years, the MRI showed atrophy in the left temporo-parietal region, corresponding to earlier SPECT findings. Our findings must be interpreted with caution. In addition to the
usual limitations of case reports, our patient is still alive, so that neuropathological confirmation is not available. Of note, however, is the fact that even patients with a typical course do not always meet neuropathological criteria, which has led to the proposal that “CBD syndrome” is a better term,3 and suggests that the diagnosis might best be confirmed by careful follow-up, which, in this case, will include further anatomic and functional-imaging studies.
Jennifer Martin, M.D. Gilbert Matte, Ph.D. David Barnes, M.D. Steven Burrell, M.D. Department of Radiology Dalhousie University Halifax, Nova Scotia, Canada Sultan Darvesh, M.D., Ph.D. Kenneth Rockwood, M.D. Department of Medicine Divisions of Geriatric Medicine and Neurology
Dalhousie University Halifax, Nova Scotia, Canada
References 1. Mathuranath PS, Xuereb JH, Bak T, et al: Corticobasal ganglionic degeneration and/or frontotemporal dementia? J Neurol Neurosurg Psychiatry 2000; 68:304– 312 2. Graham NL, Bak TH, Hodges JR: Corticobasal degeneration as a cognitive disorder. Mov Disord 2003; 18:1224–1232 3. Doran M, du Plessis DG, Enevoldson TP, et al: Pathological heterogeneity of clinically diagnosed corticobasal degeneration. J Neurol Sci 2003; 216:127–134 4. Slawek J, Lass P, Derejko M, et al: Cerebral blood flow SPECT may be helpful in establishing the diagnosis of progressive supranuclear palsy and corticobasal degeneration. Nuclear Medicine Review Central Eastern Europe 2001; 4:73–76 5. Zhang L, Murata Y, Ishida R, et al: Differentiating between progressive supranuclear palsy and corticobasal degeneration by brain perfusion SPECT. Nucl Med Commun 2001; 22:767–772 6. Yekhlef F, Ballan G, Macia F, et al: Routine MRI for the differential diagnosis of Parkinson’s disease, MSA, PSP, and CBD. J Neural Transm 2003; 110:151–169
ERRATUM
I
n the November–December 2004 issue of the Journal (12:613–620), the article “Hippocampal Volume Measurement in Older Adults With Bipolar Disorder,” by John L. Beyer et al., cited an article by Noga JT et al., stating that the authors had found increased
334
hippocampal volume size in bipolar patients. The authors, in fact, found decreased right-hippocampal volumes in the monozygotic twin affected with bipolar disorder. Dr. Beyer regrets this error and appreciates Dr. Noga’s bringing it to our attention.
Am J Geriatr Psychiatry 13:4, April 2005