Corticobasal degeneration preceded by cognitive impairment and apathy: An autopsy case report.

Abstract

Background

Corticobasal degeneration (CBD) presents with a range of clinical phenotypes, making diagnosis challenging. We report the clinical course of a patient with autopsy‐confirmed CBD who initially presented with cognitive impairment and apathy, without motor symptoms.

Case Presentation

A 51‐year‐old man presented to our hospital with decreased motivation, amnesia, and executive dysfunction. Memory disturbance and frontal lobe dysfunction were evident on examination. No motor symptoms such as Parkinsonism, apraxia, or oculomotor abnormalities were apparent. Magnetic resonance imaging and brain perfusion imaging revealed mild atrophy and hypoperfusion in the left frontal lobe. Based on these findings, he was initially diagnosed with behavioral variant frontotemporal dementia. However, due to the presence of memory impairment, Alzheimer's disease was also considered in the differential diagnosis. At the age of 53 years, he began experiencing frequent falls, followed by rapid progression of extrapyramidal symptoms. Dopamine‐transporter (DAT) imaging revealed a marked reduction in DAT availability in the striatum. The patient died of aspiration pneumonia at the age of 55 years. Neuropathological examination confirmed the diagnosis of CBD, showing abundant tau pathology in the frontal lobes and basal ganglia.

Conclusion

In this case, abundant tau pathology in the frontal lobe corresponded with early cognitive impairment, including frontal lobe dysfunction, whereas abundant tau pathology in the basal ganglia corresponded with rapid exacerbation of extrapyramidal symptoms and marked reduction in DAT availability. Clinicians should be aware that patients with CBD without motor symptoms may seek treatment at outpatient clinics specializing in dementia.

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Keywords: Alzheimer's disease, cognitive dysfunction, corticobasal degeneration, dopaminergic imaging, frontotemporal dementia

Article notes

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Revised 2025 Jun 30; Received 2025 Jun 11; Accepted 2025 Jul 23; Collection date 2025 Sep.

Author notes

BACKGROUND

Corticobasal degeneration (CBD) is a neurodegenerative disease characterized by the accumulation of 4‐repeat tau‐positive inclusions, including astrocytic plaques, in the neurons and glial cells of the cerebral cortex and basal ganglia. ¹ Traditionally, the concept of CBD encompassed progressive asymmetric muscle rigidity, apraxia, cortical sensory impairment, myoclonus, and dystonia. However, CBD is now recognized to manifest with highly diverse clinical phenotypes. ¹ The current diagnostic criteria for CBD include several clinical phenotypes: corticobasal syndrome (CBS), which features the classical motor symptoms; frontal behavioral spatial syndrome, characterized by frontal lobe dysfunction and visuospatial cognitive deficits; and progressive supranuclear palsy syndrome (PSPS), which involves vertical gaze palsy, axial rigidity, and postural instability. ¹ Moreover, a subset of patients with pathologically confirmed CBD presents primarily with cognitive impairment without evident motor symptoms. In such cases, the initial clinical diagnosis may be frontotemporal dementia (FTD) or Alzheimer's dementia (AD), complicating the clinical identification of CBD. ² , ³

Recently, anti‐amyloid β antibody therapies have been introduced for AD, increasing the importance of expeditious and accurate dementia diagnosis. ⁴ A study reported that approximately 20% of patients clinically diagnosed with AD do not show pathological evidence of the disease. ⁵ Therefore, improving the diagnostic accuracy for disorders that need to be distinguished from AD in clinical practice is essential.

Herein, we present the clinical course of a patient who initially developed executive dysfunction, apathy, and memory impairment and was pathologically confirmed to have CBD. We also discuss the challenges of differentiating CBD from other dementias in outpatient dementia clinics.

CASE PRESENTATION

The patient was a 51‐year‐old right‐handed Japanese man with a medical history of diabetes but no family history of dementia or psychiatric disorders. After graduating from university at the age of 22, he worked as a civil servant, remained unmarried, and lived alone. At the age of 50 years, he was transferred to a different department at work, after which his motivation declined significantly. By age 51, he was making frequent mistakes at work and required his colleagues’ assistance. He also started getting lost in familiar areas and exhibited impaired judgment, such as paying off a friend's debts on his behalf and renting two additional apartment rooms without clear reasoning. He was unable to organize his belongings, and the two rented rooms became cluttered with hoarded items. He first visited our hospital at age 51, upon the recommendation of his supervisors and colleagues.

The patient displayed an indifferent attitude and provided superficial responses to questions about his job and hobbies, stating, “I don't remember.” However, spontaneous speech was fluent, and language disturbances, such as dysarthria or apraxia of speech, were absent. His Mini‐Mental State Examination (MMSE) score was 25/30 (delayed recall subscore: 1/3), and the Frontal Assessment Battery (FAB) score was 11/18, indicating temporal disorientation, memory impairment, and frontal lobe dysfunction. No signs of Parkinsonism, aphasia, apraxia, or oculomotor abnormalities were noted. Laboratory test results were unremarkable. Magnetic resonance imaging (MRI) showed slight atrophy in the left frontal lobe (Figure 1a). Moreover, mild asymmetric atrophy was observed in the frontal operculum (Figure 1a). On the other hand, there was no apparent atrophy of the amygdala or midbrain (Figure 1b,c). Technetium‐99m ethyl cysteinate dimer [(^99mTc)ECD] single‐photon emission computed tomography (SPECT) depicted left‐dominant frontal lobe and mild parietal lobe hypoperfusion (Figure 1d). Based on symptoms such as apathy, mild disinhibition, and lack of empathy, the patient was diagnosed with behavioral‐variant frontotemporal dementia (bvFTD) according to Rascovsky's criteria. ⁶ However, due to concurrent memory impairment and parietal hypoperfusion, the behavioral/dysexecutive variant of AD (bvAD) was also considered in the differential diagnosis. ⁷ He continued working with a reduced workload. At a follow‐up visit 1 year later (patient age: 52 years), a marked decline in spontaneous speech, worsening of apathy, and more prominent memory and constructional impairments were apparent. At this juncture, the patient's MMSE and FAB scores were 22/30 and 12/18, respectively. However, Parkinsonism or dysarthria was absent. MRI showed slight progression of left frontal lobe atrophy and mild enlargement of the left Sylvian fissure (Figure 1e). Amygdala and midbrain volumes remained preserved (Figure 1f,g). [^99mTc]ECD SPECT showed exacerbation of left frontal lobe hypoperfusion (Figure 1i). At age 53, the patient retired. He became prone to falls and was hospitalized with a rib fracture. Following hospitalization, his functional independence declined significantly, requiring continuous supervision and assistance. He rapidly developed extrapyramidal symptoms, including bradykinesia, rigidity, and dysphagia, without exposure to antipsychotics. Dopamine‐transporter (DAT) SPECT revealed marked reduction in striatal DAT availability (Figure 1h). The clinical diagnosis of “suggestive of PSP” was made based on the tendency to fall, Parkinsonism, and frontal lobe dysfunction. ⁸ Six months later, he was bedridden and subsequently died of aspiration pneumonia at the age of 55 years. The total disease duration was approximately 5 years.

Neuroradiological findings. (a) Magnetic resonance fluid‐attenuated inversion recovery imaging (MRI FLAIR) (patient age: 51 years) showing slight atrophy of the left frontal lobe. In addition, mild asymmetric atrophy can be observed in the frontal operculum. (b, c) T1‐weighted MRI (patient age: 51 years) showing no atrophy of the amygdala or midbrain. (d) Brain perfusion single‐photon emission computed tomography (SPECT) easy Z‐score imaging system (eZIS) analysis (PDRadiopharma Inc.) (patient age: 51 years) showing hypoperfusion in the left‐dominant frontal lobe and mild parietal lobe. The color scale for the Z score is shown in the right part of the figure. Colored areas indicate a Z score >1. (e) MRI FLAIR images (patient age: 52 years) showing mild enlargement of the left Sylvian fissure, in addition to left frontal lobe atrophy. (f, g) T1‐weighted MRI (patient age: 52 years) showing no atrophy of the amygdala and midbrain. (h) 123I‐N‐omega‐fluoropropyl‐2‐beta‐carbomethoxy‐3‐beta‐(4‐iodophenyl) nortropane SPECT (patient age: 53 years) depicting a marked reduction in dopamine transporter (DAT) availability in the whole striatum bilaterally on visual assessment. (i) Brain perfusion SPECT eZIS analysis (PDRadiopharma Inc.) (patient age: 52 years) showing worsening of hypoperfusion in the left frontal lobe. The color scale for the Z score is shown in the right part of the figure. Colored areas indicate a Z score >1. — Figure 1Neuroradiological findings. (a) Magnetic resonance fluid‐attenuated inversion recovery imaging (MRI FLAIR) (patient age: 51 years) showing slight atrophy of the left frontal lobe. In addition, mild asymmetric atrophy can be observed in the frontal operculum. (b, c) T1‐weighted MRI (patient age: 51 years) showing no atrophy of the amygdala or midbrain. (d) Brain perfusion single‐photon emission computed tomography (SPECT) easy Z‐score imaging system (eZIS) analysis (PDRadiopharma Inc.) (patient age: 51 years) showing hypoperfusion in the left‐dominant frontal lobe and mild parietal lobe. The color scale for the Z score is shown in the right part of the figure. Colored areas indicate a Z score >1. (e) MRI FLAIR images (patient age: 52 years) showing mild enlargement of the left Sylvian fissure, in addition to left frontal lobe atrophy. (f, g) T1‐weighted MRI (patient age: 52 years) showing no atrophy of the amygdala and midbrain. (h) ¹²³I‐N‐omega‐fluoropropyl‐2‐beta‐carbomethoxy‐3‐beta‐(4‐iodophenyl) nortropane SPECT (patient age: 53 years) depicting a marked reduction in dopamine transporter (DAT) availability in the whole striatum bilaterally on visual assessment. (i) Brain perfusion SPECT eZIS analysis (PDRadiopharma Inc.) (patient age: 52 years) showing worsening of hypoperfusion in the left frontal lobe. The color scale for the Z score is shown in the right part of the figure. Colored areas indicate a Z score >1.

Neuropathological findings

On autopsy, the brain weighed 1080 g before fixation. Atrophy of the frontal cerebral cortices was evident, while the motor cortex was preserved (Figure 2a,b). In the coronal sections, widening of the sulci in the frontal cortex, brownish changes in the pallidum and putamen, and atrophy of the inferior temporal cortex were observed, whereas the amygdala, hippocampus, and the parieto‐occipital cortices were preserved (Figure 2c–f). Gallyas–Braak (GB) silver staining revealed tau pathology with abundant threads in the frontal cortex, amygdala, entorhinal cortex, inferior temporal cortex, pallidum, and hippocampus; however, the tau pathology was relatively mild in the parieto‐occipital cortex (Figure 2g–j). Threads were more abundant in the frontal cortex than in the parietal cortex; they were abundant in both the gray and white matter, and particularly prominent in the latter (Figure 3a–c,f–h). Astrocytic plaques were observed in the frontal and parietal cortices, which were positive for phosphorylated tau (AT‐8) and 4‐repeat tau (RD4) immunostaining (Figure 3d,e) but were negative for 3‐repeat tau (RD3) immunostaining, confirming the diagnosis of CBD. Threads were prominent in the pencil fibers in the caudate and putamen (Figure 4a,b,e). Astrocytic plaques were also observed in the caudate nucleus (Figure 4d). The subthalamic nuclei were relatively preserved, whereas numerous threads and neuronal inclusions were visible on GB silver staining (Figure 4c,f). Tau pathology was severe in the tegmentum of the midbrain, pons, and medulla oblongata (Figure 5a,b,d,e,g,h). Severe neuronal loss and abundant threads were observed in the substantia nigra (Figure 5c). Mild neuronal loss and tau pathology, including threads and intraneuronal inclusions, were evident in the locus coeruleus and olivary nucleus (Figure 5f,i). The pyramidal tract at the level of the medulla oblongata did not show degeneration (Figure 5j,k). The distribution and extent of severity of the neuropathological findings, evaluated according to Dickson et al.'s method, ⁹ , ¹⁰ are summarized in Table 1. Although tau or GB‐positive threads were widespread throughout the cerebral cortex and white matter, brain stem, and dentate nucleus of the cerebellum, neuronal loss was most severe in the frontal cortex and substantia nigra.

Figure 2Macroscopic and semi‐macroscopic findings. Focal atrophy is evident in the frontal cortices (a) and medial frontal cortices of the cerebrum (b). Moderate atrophy of the frontal cortices (c) and mild atrophy of the temporal cortices can be visualized in the coronal sections of the cerebrum (d, e). The motor (e), parietal, and occipital cortices (f) are intact. Gallyas‐Braak silver staining reveals severe tau pathology in the superior frontal cortices (g), amygdala and entorhinal cortices (h), inferior temporal cortices, hippocampus, and pallidum (i). Tau pathology is relatively mild in the parietal cortices (j). Gallyas‐Braak silver staining (g–j). Scale bars: 10 mm (a–f), 5 mm (g–j).

Figure 3Microscopic findings of the cerebrum. Abundant threads appear in the gray and white matter of the superior frontal lobe (a). The threads are more abundant in the white matter (c) than in the gray matter (b). Astrocytic plaques are seen in the gray matter (b), which are positive for phosphorylated tau (AT‐8) (d), and four‐repeat tau (RD4) (e). The inferior parietal lobes show less abundant neuropil threads (f) than in the frontal lobes. Astrocytic plaques are also seen in the parietal cortex. Threads in the white matter are also more abundant than in the gray matter (h). Gallyas‐Braak silver staining (a–c, f–h), phosphorylated tau (AT‐8) immunostaining (d), and four‐repeat tau (RD4) immunostaining (e). Scale bars: 500 μm (a, f), 25 μm (b–e, g–h).

Figure 4Microscopic findings of the basal ganglia. Numerous threads are visible in the caudate nuclei and internal capsule (a). Astrocytic plaques are seen in the caudate nuclei (d). Abundant neuropil threads are also seen in the globus pallidum and putamen (b). Many threads can be seen in Wilson's pencil fibers (e). The subthalamic nuclei are relatively spared (c) with a fair number of threads and pleomorphic neuronal inclusions. Gallyas‐Braak silver staining (a–f). Scale bars: 500 μm (a–c), 25 μm (d–f).

Figure 5Macroscopic, semi‐macroscopic, and microscopic findings of the midbrain, pons, and medulla oblongata. Depigmentation is apparent in the substantia nigra (a). Threads are abundant in the midbrain tegmentum as well as the substantia nigra (b). Neuronal loss with threads is observed in the substantia nigra (c). The locus coeruleus shows mild depigmentation (d) with some threads and neurofibrillary tangles (f). Numerous threads are present in the pontine tegmentum and fibers of the pontine base (e). The tegmentum of the medulla oblongata shows brownish degenerative changes (g), where threads are also abundant (h). The olivary nuclei contain many threads and some neurofibrillary tangles (h, i). The pyramidal tract at the level of the medulla oblongata does not show degeneration (j, k). Gallyas‐Braak silver staining (b, c, e, f, h, i) and Klüver‐Barrera staining (j, k). Scale bars: 500 μm (a,b, d, e, g, h, j), 25 μm (c, f, i), 250 μm (k).

Table 1Distribution and severity of major histopathologic findings.

	Neuronal loss and gliosis	Ballooned neurons	Tau‐ or Gallyas‐ positive neurons	Tau‐ or Gallyas‐ positive glia	Tau‐ or Gallyas‐ positive threads
Cerebral cortex
Frontal	2	2	2	2	2
Motor(peri‐Rolandic)	1	2	2	2	2
Cerebral white matter				2	2
Parietal	1	1	1	1	2
Temporal	1	1	2	2	2
Entorhinal	1	2	2	2	2
Occipital	0	0	0	1	1
Subcortical areas
Hippocampus	0		0	2	2
Amygdala	1	2	2	1	2
Basal nucleus of Meynert	0		2	1	1
Caudate and putamen	1		1	2	2
Globus pallidus	1		2	2	2
Internal capsule				2	2
Thalamus	1		2	2	2
Subthalamic nucleus	1		2	2	2
Brain stem
Midbrain tectum (colliculi)	2		2	2	2
Red nucleus	1		2	2	2
Substantia nigra	2		2	2	2
Cerebral peduncle	0		0	1	1
Locus ceruleus	0		2	1	2
Pontine tegmentum	1		2	2	2
Fibers in the pontine base				2	2
Inferior olivary nucleus	0		2	2	2
Cerebellum
Dentate nucleus	0		1	2	2
Cerebellar white matter				1	1

Aβ or neuritic plaques were not observed in any region. Immunohistochemistry revealed Thal's phase 0 for Aβ plaques; the neuritic plaque score determined by the Consortium to Establish a Registry for AD was C0. Neurofibrillary degeneration was graded as Braak stage I. AD neuropathologic changes, evaluated using the ABC score, ¹¹ were graded as A0B1C0, indicating “Not AD.” Numerous argyrophilic grains were observed in the amygdala, hippocampus, and temporal and frontal cortices, whose distribution corresponded to Saito stage III. ¹² No Lewy pathology nor TDP‐43 pathology was found in any region. Arteriosclerotic changes and vascular lesions in the brain were absent.

DISCUSSION

This patient initially presented with cognitive impairment, apathy, and mild disinhibition, leading to a diagnosis of bvFTD or bvAD. Thereafter, extrapyramidal symptoms developed rapidly, and the patient was finally clinically diagnosed with “suggestive of PSP,” and the pathological diagnosis was CBD. A Japanese clinicopathological study reported that the most common initial symptom of CBD was gait disturbance (74%), followed by bradykinesia (64%), although memory impairment (44%) and behavioral abnormalities (22%) were also observed. ³ In addition, corresponding to the presence of memory impairment and behavioral abnormalities, the initial diagnosis of CBD was AD in 13% and FTD in 9% of cases. ³ This is consistent with the clinical course of this case, where the initial symptoms were memory impairment and frontal dysfunction, and the diagnosis of bvFTD or bvAD was considered. Notably, until midway through the course, this patient did not exhibit the motor symptoms that are the hallmark initial symptoms of CBD, but instead exhibited memory impairment, apathy, and executive dysfunction. CBD, in which cognitive dysfunction is more dominant than motor symptoms, is characterized by more frequent executive dysfunction and apathy compared with CBD, in which motor symptoms are dominant. ² In addition, patients with CBD in whom psychiatric symptoms and behavioral abnormalities appear first without motor symptoms may not even exhibit motor disorders, not only in the early stages of the course but also in the final stages. ¹³ Therefore, clinicians need to be aware of the existence of a patient subset in whom motor symptoms are absent in the early stages, even in those with underlying CBD, as in this patient.

In this patient, abundant tau pathology was evident in the frontal lobe and basal ganglia, such as the substantia nigra, globus pallidum, caudate, and putamen. CBD, in which cognitive impairment is predominant over motor symptoms, reportedly harbors more tau pathology in the superior frontal cortex and inferior temporal cortex and less tau pathology in the motor cortex. ² Similarly, the abundance of tau pathology in the frontal lobe may have influenced this patient's clinical course, which was marked by executive dysfunction and apathy. Abundant tau pathology in the basal ganglia and severe neuronal loss may have contributed to the rapid deterioration of motor symptoms. However, CBD, in which cognitive impairment predominates over motor symptoms, has been reported to have milder neuronal loss in the substantia nigra and subthalamic nucleus compared to CBD, in which motor symptoms predominate. ² , ¹³ The relationship between severe tau pathology and neuronal loss in the substantia nigra and the lack of motor symptoms in the early stages of the disease in this patient is unclear, but a discrepancy between motor symptoms and DAT imaging findings reflecting degeneration of the substantia nigra is often seen in dementia with Lewy bodies and FTD. ¹⁴ , ¹⁵

The pathologies that underlie bvFTD are mainly divided into frontotemporal lobar degeneration (FTLD)‐tau, such as Pick's disease, CBD, and PSP; FTLD‐transactive response DNA‐binding protein 43 kDa (TDP43), which is classified into types A to D according to the accumulation pattern of TDP43‐positive structures; and FTLD‐fused sarcoma. ¹⁶ , ¹⁷ Abnormal findings on DAT‐SPECT in patients with bvFTD may suggest that the underlying pathology is CBD, PSP, or FTLD‐TDP type B. ¹⁵ , ¹⁸ In addition, abnormal findings may be detected on DAT‐SPECT before the onset of motor symptoms in these diseases. ¹⁵ In this case, DAT‐SPECT was performed after the motor symptoms became apparent. However, if DAT‐SPECT had been performed at the initial consultation, abnormal findings may have been detected, and the later appearance of motor symptoms could have been predicted. This patient exhibited severe tau pathology not only in the substantia nigra but also in the globus pallidum and pencil fibers in the caudate and putamen. These pathological findings may have contributed to the significant reduction in DAT. In cases of bvFTD with underlying pathology, such as CBD, PSP, or FTLD‐TDP type B, atrophy of the frontal and temporal lobes is likely to be mild. ¹⁹ Consistently, in this patient, atrophy of the frontal and temporal lobes was very mild on MRI. Therefore, if the patient presents with clinical features suggestive of bvFTD, and atrophy of the frontal and temporal lobes is mild, even in the absence of motor symptoms, considering DAT‐SPECT may be useful in determining the patient's underlying pathology.

This study has some limitations. First, genetic analysis was not performed. Although the patient had no family history, the age of onset was younger than previously reported autopsy cases of CBD. ³ Therefore, important information pertaining to the presence or absence of gene mutations, such as microtubule‐associated protein tau, is missing. ²⁰ Second, biochemical analysis was not performed. Therefore, important information supporting the diagnosis of CBD is missing. ⁹ Finally, the significant decrease in spontaneous speech over the disease course may have led to underestimation of the language disturbances, such as apraxia of speech, which may occur in CBD.

CONCLUSION

Clinicians should be aware that patients with cognitive‐predominant CBD with less prominent motor symptoms may seek treatment at dementia specialist outpatient clinics. In addition, performing DAT‐SPECT in patients with frontal dysfunction without motor symptoms may contribute to an accurate clinical diagnosis.

AUTHOR CONTRIBUTIONS

Shoma Hirose analyzed the clinical data and drafted the manuscript. Ryota Kobayashi analyzed the clinical and pathological data and drafted the manuscript. Shoma Hirose examined the patient and revised the manuscript. Shinobu Kawakatsu analyzed the neuropathological data and drafted the manuscript. Akihito Suzuki and Chiaki Kawanishi analyzed the data and revised the manuscript. Kumiko Utsumi examined the patient, analyzed the data, and revised the manuscript. All the authors have read and approved the final version of this manuscript.

CONFLICT OF INTEREST STATEMENT

R.K. has received honoraria for lectures from PDR Pharma and Nihon Medi‐Physics, as well as research support from Nihon Medi‐Physics. The other authors declare no conflicts of interest.

ETHICS APPROVAL STATEMENT

This study was approved by the Ethical Review Committee of Sunagawa City Hospital.

PATIENT CONSENT STATEMENT

Written informed consent was obtained from the patient and his family for participation in this study and publication of the data. Written consent for the publication of the anonymized case details was obtained from the patient's family.

CLINICAL TRIAL REGISTRATION

N/A.

ACKNOWLEDGMENTS

The authors would like to thank Editage (www.editage.jp) for English language editing. This study was supported by a Grant‐in‐Aid from the Ministry of Health, Labour, and Welfare Research on Dementia Program (Grant Numbers 20GB1002 and 23GB1003).

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Hirose S, Kobayashi R, Hatakeyama S, Kawakatsu S, Suzuki A, Kawanishi C, et al. Corticobasal degeneration preceded by cognitive impairment and apathy: an autopsy case report. Psychiatry Clin Neurosci Rep. 2025;4:e70174. 10.1002/pcn5.70174

Contributor Information

Ryota Kobayashi, Email: ryo.kobayashi@med.id.yamagata-u.ac.jp.

Chiaki Kawanishi, Email: chiaki.kawanishi@gmail.com.

DATA AVAILABILITY STATEMENT

The data that support the findings of this study are available from the corresponding author upon reasonable request.

REFERENCES

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Data Availability Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.

Corticobasal degeneration preceded by cognitive impairment and apathy: An autopsy case report.

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Corticobasal degeneration preceded by cognitive impairment and apathy: An autopsy case report

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BACKGROUND

CASE PRESENTATION

Neuropathological findings

DISCUSSION

CONCLUSION

AUTHOR CONTRIBUTIONS

CONFLICT OF INTEREST STATEMENT

ETHICS APPROVAL STATEMENT

PATIENT CONSENT STATEMENT

CLINICAL TRIAL REGISTRATION

ACKNOWLEDGMENTS

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DATA AVAILABILITY STATEMENT

REFERENCES

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References

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