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A degenerative familial disease with insomnia and autonomic disorders in a 53 years old man is reported. At post-mortem examination anterior and dorso-medial nuclei of the thalamus were atrophied.
Within the spectrum of Lewy body disease cognitive impairment occurs in PD with dementia (PDD) and dementia with Lewy bodies (DLB). Although neocortical cholinergic deficits are associated with cognitive impairments in PDD and DLB, no neurochemical study has been published describing the thalamic cholinergic activity whereas the thalamus plays a major role in modulating cortical activity. Choline acetyltransferase (ChAT) activity was analyzed in reticular (Re), mediodorsal (MD) and centromedian (CM) thalamic nuclei in series of nine controls, five DLB with parkinsonism (DLB + P), five DLB without parkinsonism (DLB - P), six PD without dementia and 14 PDD cases. Significant reductions in ChAT were apparent in PDD as follows: in Re and MD nuclei compared with controls; in MD and CM nuclei compared with DLB + P; and in MD compared with PD. Increased ChAT activity was found in CM nuclei in DLB + P compared with DLB - P. These findings show that significant thalamic presynaptic cholinergic deficits occur only in cases of combined cortical and subcortical neurodegeneration in which dementia developed after prolonged parkinsonism.
An autopsied case of membranous lipodystrophy (Nasu-Hakola disease, NHD) with thalamic degeneration was reported. A 34-year-old Japanese man was diagnosed as having NHD by bone biopsy prior to the onset of clinical symptoms. His maternal grandfather and paternal grandmother are cousins, but this family history is negative for NHD. He developed frontal lobe syndrome at the age of 35 with progressive dementia, and died of acute renal failure at the age of 46. Gross inspection of the brain detected atrophy and softening of the cerebral white matter, predominantly in the frontal lobe. Microscopically, numerous spheroids, predominant fibrillary gliosis with less prominent demyelination "dissociation glio-myélinique" and scanty sudanophilic lipid droplets were observed, indicating the sclerosing type of NHD. An unusual patholgoical finding in this case was selective involvement of the thalamic nuclei with preservation of the other gray matter except for focal cortical necrosis. The topography of the affected thalamic nuclei is similar to that of systemic thalamus degeneration. An association with thalamic degeneration in NHD has not been previously reported. The present case suggests that NHD also affects the thalamus.
Fatal Familial Insomnia (FFI) is an inherited disease characterized clinically by sleep, autonomic and motor disturbances and pathologically by marked atrophy of the anterior and dorsomedial nuclei of the thalamus. The neuropsychological study of three cases of FFI showed: (1) a progressive disturbance of attention and vigilance, (2) a memory deficit with lability of mnesic traces and difficulty in manipulation and ordering of events, suggesting an alteration of working memory and (3) a deficit of frontal abilities with impairment in planning and prevision of events but preservation of general intelligence.
We assessed the long-term effect of thalamic deep brain stimulation (DBS) on motor symptoms and progression of Parkinson's disease (PD) in PD patients treated for resting and postural/action tremor. Thalamic DBS was performed in 17 patients with treatment-resistant resting and postural/action tremor. Nine patients were available for follow-up examination a mean of 5.5 years after surgery. Three had tremor-dominant PD. DBS produced marked improvement in resting and postural/action tremor in target upper extremity in all 9 patients, which persisted unchanged at the time of the last follow-up visit 5.5 years after surgery. PD severity with DBS 'on' and 'off' 1 year after surgery was compared to PD severity at the last follow-up visit using UPDRS (Unified Parkinson's Disease Rating Scale) III motor scores and individual motor item subscores. Patients were tested while on medication. There was no significant worsening of tremor, rigidity, speech, postural stability, gait, or axial bradykinesia with DBS either on or off at the last follow-up visit compared to the 12-month visit. UPDRS III motor scores were unchanged. However, global assessment of PD progression and increased mean L-dopa dose and L-dopa equivalent daily dose at the time of last follow-up visit indicated that a progression of PD had occurred.
The Parkinson's disease (PD)-related inclusion body pathology comprises Lewy bodies (LBs) as well as Lewy neurites (LNs). The distribution and severity of this pathology were investigated in the thalamus of 12 autopsy cases with clinically diagnosed and neuropathologically confirmed PD. The LBs and LNs were visualized by immunoreactions against the protein alpha-synuclein. In the human thalamus during PD, a specific and highly stereotypical distribution pattern of LBs and LNs evolves. As in cortical and other subcortical regions, the components of human thalamus assigned to the limbic loop bear the brunt of the PD-related pathology. In contrast, the thalamic components integrated into the striatal and cerebellar loops as well as the primary sensory nuclei of the thalamus show at best a mildly developed pathology. Damage to the thalamic components of the limbic loop nuclei may contribute not only to the cognitive, emotional, and autonomic symptoms of PD but to the somatomotor and oculomotor dysfunctions as well.
A case of neuro-Behcet disease presenting as a thalamo-lenticular expanding lesion is reported. A 41-year-old female was admitted with mental deterioration and right hemiparesis. She had been suffering from recurrent oral and genital ulcers and erythema nodosum for several years before admission. Neurological examination on admission revealed poor mental activity, dysarthria and right hemiparesis. Lumbar puncture showed CSF pleocytosis. CT and MRI revealed a thalamo-lenticular expanding lesion. CT showed a homogeneous hypodense lesion of the left lenticulothalamic region, which was enhanced in the central part, exerting a mild mass effect. MRI also revealed the lesion better. The T2 weighted images showed a high intensity signal in the left thalamo-lenticular region and the left peduncle. The T1 weighted images showed a low signal in the corresponding areas with a central enhancement with Gd-DTPA. EEG, SPECT and angiography indicated normal findings. Histologic study of the brain biopsy tissue ruled out a tumor but did not show any specific diagnosis. The patient improved with steroid therapy. In conclusion, the clinical and radiological presentation of neuro-Behcet disease can mimic a cerebral tumor. In such cases, stereotactic biopsy is useful to exclude suspicion of a cerebral tumor.
MR imaging examinations of seven patients with a variety of claustral disorders are included in this study. The ages of the patients ranged from 4 to 65 years, and all of them were males. The lesions involving the claustrum comprised cases with asphyxia, Wilson's disease, ischemic white matter disease, thalamic arteriovenous malformation, MELAS syndrome, viral encephalitis and Parkinson's disease.
We report a 64-year-old Japanese woman who died one year after the onset of progressive gait disturbance and dementia. She noted a difficulty in holding a glass and hand tremor in June of 1996 when she was 63 years old. In July of 1996, she tended to lean toward left when she walked. She also noted truncal titubation. In November of 1996, she started to have visual hallucination and delusion in which she said "I see something is flying on the wall.", "Somebody has come into my room", and things like that. She was admitted to our service on November 22, 1996. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed disturbance in recent memory. Hasegawa's dementia rating scale was 22/30. She showed vivid visual hallucination with colors in which she saw faces of dwarfs and angels, a space ship, and others. Higher cerebral functions were normal. She showed left oculomotor palsy which was a sequel of an aneurysm and subarachnoid hemorrhage nine years before. Otherwise cranial nerves were unremarkable. She showed ataxic gait, limb ataxia, truncal titubation, and postural hand tremor. She had no weakness and no muscle atrophy. Deep tendon reflexes were within normal limits. Plantar response was flexor. Sensation was intact. Laboratory examination was also unremarkable. Complete survey for occult malignancy was negative. CSF was under a normal pressure and cell count was 1/microliter, total protein 27 mg/dl, and sugar 68 mg/dl. Cranial CT scan was unremarkable. MRI was not obtained because of the presence of an aneurysm clip in the left internal carotid-posterior communication artery junction. She showed progressive deterioration in her mental function. By January 1997, she became unable to stand or walk with marked dementia. Repeated CSF exams and cranial CT scans were unremarkable. She suffered from several episodes of aspiration pneumonia. A trial of three days methylprednisolone pulse therapy was given starting on March 7, 1997, which was of no effect on her neurologic status. On March 28, 1997, she was intubated because of acute respiratory distress syndrome. In April 2, her body temperature rose to 38 degrees C. On April 9, 1997, her blood pressure dropped and resuscitation was unsuccessful. She was pronounced dead on the same day. The patient was discussed in a neurologic CPC and the chief discussant arrived at the conclusion that the patient had primary leptomeningeal lymphoma. Other possibilities entertained among the audience included brain stem encephalitis of unknown type, carcinomatous cerebellar degeneration plus limbic encephalitis, Creutzfeldt-Jakob disease, thalamic degeneration, and progressive multifocal leukoencephalopathy. Post-mortem examination revealed thickening and clouding of the leptomeninges; Gram-positive diplococci were found in the leptomeninges. This meningitis appeared to have been an complication in the terminal stage of her illness. Microscopic examination revealed astrocytosis in the midbrain tegmentum. Cerebral cortices showed only mild astrtocytosis. No cerebellar atrophy was seen and Purkinje cells were retained which excluded paraneoplastic cerebellar degeneration. Neuropathologic diagnosis was bacterial meningitis, however, the presence of brain stem encephalitis prior to the onset of bacterial meningitis could not be excluded. It is interesting to note that the diagnosis of the primary neurologic disease of this patient was not easy even after autopsy. As autopsy permission was obtained only for the brain, it was not clear whether or not this patient had an occult malignancy somewhere in her body, however, there was no evidence to indicate paraneoplastic degeneration of the central nervous system. As the patient did not have meningeal signs until one month before her death, it is difficult to ascribe her entire neurologic problems to her meningitis. Finally, her visual hallucination was vivid and colorful; we thought this might have been
A patient had an ischemic infarction of the right medial thalamus, with a resultant contralateral neglect syndrome. We propose that the medial thalamic nuclei, particularly centromedian and parafascicularis (CMPF), are normally involved in the arousal-activation process by which an organism can be aroused by and respond to novel or important stimuli. Specifically, we propose that (1) the mesencephalic reticular formation subserves tonic arousal to novel or painful stimuli by inhibiting the nucleus reticularis thalami (NR) and (2) that selective attention is mediated by cortical input to NR. The CMPF is closely associated with motor systems (basal ganglia, ventrolateral nucleus of the thalamus [VL], and frontal lobes). A pathway involving CMPF, the frontal cortex, and the portion of NR associated with VL may be important in preparing the tonically aroused organism for action. Unilateral lesions of CMPF therefore induce an asymmetric hypokinesia, and bilateral lesions may induce akinetic mutism.
A patient with Kleine-Levin syndrome, typical except that onset was at 39 years of age, died during a symptomatic period. Autopsy disclosed recent and old lesions in the medial thalamus involving intralaminar, medial, and some dorsal nuclei as well as the pulvinar. Despite massive microglial infiltration, there was minimal neuronal loss. The hypothalamus was not involved. The findings suggest a viral cause for Kleine-Levin syndrome.
A patient complained of memory disturbance after a small left thalamic infarction. Neuropsychological testing revealed her memory to be normal provided that she was allowed to rehearse or use semantic encoding strategies. When these strategies were prevented, her performance was impaired. Mapping of the lesion demonstrated involvement of the caudal intralaminar nuclei (centre médian and parafascicular nuclei), and portions of the medial nuclei (medioventral [reuniens], centromedial, and the most inferior aspect of the mediodorsal nucleus). The majority of mediodorsal nucleus, the mammillary bodies, the mammillothalamic tract, and the anterior thalamic nuclei, were spared. A comparison among our patient's performances and those of alcoholic Korsakoff patients, patient NA, and amnestic patients with circumscribed diencephalic lesions suggests that there are two distinct behavioral and anatomic types of memory impairment associated with diencephalic lesions. The severe amnesia associated with damage to the mammillary bodies, midline nuclei, mammillothalamic tract, and/or dorsomedial nucleus of the thalamus (eg, Korsakoff and NA) is characterized by encoding deficits that never approximate normal performance. The memory disturbance associated with damage to the intralaminar and medial nuclei of the thalamus is milder and is characterized by severe distractibility.
Recent successes in developing an animal model of human amnesia in the monkey have made it feasible to try to identify with certainty the specific structures in the diencephalon and medial temporal region that cause amnesia when damaged. Monkeys with small lesions restricted largely to the posterior portion of the mediodorsal nucleus of the thalamus were given a test of memory sensitive to human amnesia and a second test that is analogous to the skill-based tasks performed normally by amnesic patients. The monkeys exhibited a marked impairment on the first test and performed normally on the second. The results show that circumscribed lesions of the mediodorsal nucleus can cause substantial amnesia.
Hemiballism has rarely been reported in the setting of multiple sclerosis. Review of clinical descriptions cast some doubt on the accuracy of the diagnosis of the movement disorder in several previous cases. We report a patient with definite multiple sclerosis, with videotape documentation of hemiballism, and demonstration of a plaque in the contralateral subthalamic nucleus on a magnetic resonance image.
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