Fulminant corticobasal degeneration: Agrypnia excitata in corticobasal syndrome

Abstract

Corticobasal degeneration (CBD) may be expressed as an atypical parkinsonism with a mean disease survival of about 7 years, 1 the shortest reported survival being 24 months. 2 We report a patient with corticobasal syndrome (CBS) and pathology-confirmed CBD whose 10-month course was manifested as a frontal alien hand evolving into agrypnia excitata.Case report. A 60-year-old man without family history of neurologic illnesses developed falls and worsening dexterity of his right arm over 4 months, followed by severe insomnia and episodes of intermittent truncal tremor triggered by limb movement. By 7 months, he had developed an asymmetric parkinsonism with marked rigidity, high-frequency jerky hand tremor, and hyperreflexia. He had right-hand ideomotor apraxia but no cortical sensory loss. He exhibited picking movements with his right hand, which he did not perceive as alien (video on the Neurology ® Web site at Neurology.org).His medical history included hepatitis C-associated liver cirrhosis, diagnosed 3 years previously, and ribavirin-induced peripheral neuropathy. Brain MRI showed only minimal atrophy of the left posterior frontal and anterotemporal lobes (figure, A). PET with fluorodeoxyglucose showed mild decreased metabolic activity in the posterior parietal lobes and the bilateral thalami (figure, B). Routine CSF studies had normal results, including 14-3-3 protein. Levodopa yielded no benefits. By 8 months, he was bedbound, was unable to fall or remain asleep, and appeared to be in a constant dream-like state (detailed observations by his daughter during this period are available online as supplemental correspondence). He also manifested paranoid ideation and hallucinations. He died within 10 months after symptom onset.Postmortem examination. The brain weighed 1,275 g. The left hemisphere showed mild frontal and parietal atrophy. There was moderate to severe neuronal cell loss and gliosis in the neocortex, basal ganglia, thalamus, and midbrain. Microvacuolization (spongiform change) was present within the superficial neocortex, most prominent in the middle frontal gyrus, cingulate gyrus, and inferior parietal lobule. No Lewy bodies were identified in the brain with hematoxylin & eosin staining. a-Synuclein staining was negative in the brainstem. 4R tau-immunoreactive neurons and astroglia, thread-like deposits, astrocytic plaques, and coiled bodies were found throughout the cerebral cortex, basal ganglia, subthalamic nucleus, cerebellar dentate nucleus, midbrain, pons, and medulla ( figure, C). Within the thalamus, there was severe neuronal and glial tau deposition within the ventral lateral and reticular nuclei, as well as the zona incerta. There was moderate involvement of the dorsomedial nucleus, but relatively sparse involvement of the anterior (anteroventral) nucleus with only a few tau-immunoreactive neurons. Swollen achromatic balloon-like neurons were also documented. There was no associated b-amyloid staining. Tissue submitted to the National Prion Disease Pathology Surveillance Center showed no e...

Myoclonus‐Dominant Corticobasal Degeneration

“…“Undefined” tremors are also mentioned in CBD cases . In a recent report of a CBD‐CBS case with a 10‐month course, episodes of intermittent truncal tremor triggered by limb movement and high‐frequency jerky hand tremor were described . In the present case, what initially resembled high‐frequency action tremor showed, in fact, clinical and neurophysiological features of action myoclonus.…”

Fulminant corticobasal degeneration: a distinct variant with predominant neuronal tau aggregates

“…Autopsy-confirmed CBD cases with disease duration of 3 years or less are rare but have been described in the literature. A report described a CBD patient who presented with a 10-month history of rapidly progressive corticobasal syndrome described as 'fulminant' CBD [55]. In a small series of 14 CBD cases, one patient had a disease duration of 2.5 years [65], while another series of 15 CBD cases reported a patient who succumbed to the illness after 2 years [48].…”

Dream Enactment Behavior Disorder Associated with Pallido‐Nigro‐Luysian Degeneration and Tau Proteinopathy

“…Nevertheless, patients with agrypnia excitata usually have a very rapid progression of symptoms, as well as additional features lacking in our patient, including autonomic hyperactivity, severe insomnia and oneiric stupor. 17 Finally, we cannot exclude that the subtle level of inflammatory cell infiltration represents a terminal phenomenon, however, it is possible that the lymphocytic inflammation as seen here, could contribute to the pathogenesis of neurodegenerative diseases without detectable antibodies.…”