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A nine-year-old Somalian boy was treated in Denmark for a posterior fossa tumor, which proved to be a tuberculoma. This disease is virtually unknown in our highly industrialized country, which emphasizes the fact that due to increased international traveling, knowledge of endemic diseases in other parts of the world is necessary. The treatment of tuberculoma and their diagnostic features are reviewed.
Brain insulin-like growth factor I (IGF-I) and its related molecules may be involved in neurodegenerative processes in which IGF-I-containing pathways are compromised. Since IGF-I is present in the olivocerebellar circuitry, two types of late-onset cerebellar ataxias (olivopontocerebellar and idiopathic cerebellar cortical atrophy) were chosen to test this hypothesis. The following significant changes in the peripheral IGF-I system of these patients were found: low IGF-I levels, and high IGF-binding protein 1 (BP-1), and BP-3 affinity for IGF-1. Sixty percent of the patients also had significantly low insulin levels. Patients suffering from other neurological diseases with cerebellar dysfunction and ataxia not involving the olivocerebellar pathway also had low IGF-I levels, while IGFBPs and insulin levels were normal. Our data indicate that degeneration of an IGF-I-containing neuronal pathway produces significant changes in the peripheral IGF system. This suggests strongly that the endocrine (bloodborne) and the paracrine/autocrine (brain) IGF systems are linked functionally. We propose that alterations in the blood IGF-I system may constitute a marker of some cerebellar diseases.
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Patients with paraneoplastic cerebellar degeneration and Hodgkin's disease develop autoantibodies (Tr-Ab) that immunoreact with the cytoplasm of the Purkinje cells and produce a characteristic punctate pattern in the molecular layer of the cerebellum. In the present study, we analyzed the structures of the adult rat cerebellar cortex identified by Tr-Ab and the expression of the antigen recognized by Tr-Ab in the developing rat brain. By laser confocal microscopy and immunoelectron microscopy, Tr-Ab immunoreactivity was found localized in the cytosol and outer surface of the endoplasmic reticulum of the perikarya of neurons of the molecular layer and the cell body and dendrites of Purkinje cells without a particular concentration in dendritic spines. Tr-Ab reactivity was more widespread in the developing rat brain. Tr-Ab labeling of Purkinje cells was already observed at P0 (day of birth). The staining of the molecular layer followed the development of the dendritic tree. The internal and inner level of the external granule cell layer were labeled with Tr-Ab with a dotted pattern that became almost negative by the 2nd postnatal week. The staining probably corresponded to granule cells as suggested by the positive Tr-Ab labeling of cultures of embryonic granule neurons. The present findings suggest that the antigen recognized by Tr-Ab appears early and is widely expressed in the developing rat brain. In the adult cerebellum, the antigen is localized in the cell body and dendrites of the Purkinje cells but is not concentrated in the dendritic spines.
The corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (the so-called "Hashimoto's Encephalopathy") is a rare disorder with multiple symptomatology, breaking out with an acute or subacute onset and having a relapsing course, not correlated to thyroid hormone levels, with autoimmune pathogenesis, and usually associated with Hashimoto's thyroiditis. In this paper, we report on a case study regarding a 46 year-old woman showing a subacute course cerebellar syndrome, associated with Hashimoto's thyroiditis, diagnosed as "Hashimoto's encephalopathy". The possible pathogenesis and the major aspects of the differential diagnostic sector are discussed with particular reference to an ataxic syndrome caused by a progressive non-familial adult onset cerebellar degeneration (PNACD), associated with the thyroid disease itself.
Two cases of Vogt-Koyanagi-Harada disease with rare neurological complications were reported, which are one case with vestibular and cerebellar ataxia, and one with multiple cranial nerve palsies. The first case, a 32-year-old man, was admitted to our ophthalmologic department because of visual impairment. Two weeks later, he complained of gait disturbance. Limb and truncal ataxia, vestibular dysfunction and perceptive deafness were observed. Ophthalmologic examination revealed typical findings of Vogt-Koyanagi-Harada disease. CSF showed elevated protein and mononuclear pleocytosis. After treatment with oral steroid therapy, vestibular and cerebellar ataxia, and visual impairment improved. The second case, a 22-years-old man, showed the VIIth, VIIIth, IXth, and Xth cranial nerve palsies with visual impairment, and he was diagnosed to have cranial nerve palsies associated with Vogt-Koyanagi-Harada disease. After steroid therapy, there was a marked clinical improvement. Vogt-Koyanagi-Harada disease is an autoimmune disease against the melanocytes and involving tissues containing melanocytes such as uvea, meningea, skin and inner ear, etc.. Neurological complications except for meningitis and perceptive deafness have been rarely reported in Japan. Related to the present cases, possible pathogenesis was discussed.
Cerebellar dentate and interpositus nuclei pathology was studied morphometrically in 3 patients with Joseph disease compared to 3 control subjects. Size of neurons, number of small neurons (cell body area: 50-199 microns 2) and large neurons (cell body area: 200 microns 2 or greater) at the rostral, medial and caudal levels, neuronal cell density, total volume of the gray bands, and total neuronal cell number were evaluated in the dentate, emboliform, and globose nuclei, using an image analyzer, after making horizontal serial 20-microns thick sections of a unilateral cerebellar hemisphere embedded in celloidin. The number of large neurons in Joseph disease was around 20% of that in the controls at each level in the individual nucleus (P less than 0.05 or P less than 0.01). In contrast, the number of small neurons was significantly reduced only at the caudal level of the dentate nucleus (P less than 0.05). The neuronal cell density was decreased within the nuclei. The total volume of the gray bands was reduced to about 70% within the nuclei (P less than 0.05 or P less than 0.01). The total number of neurons was decreased to about a third, a half, and a third within the dentate (P less than 0.001), emboliform (P less than 0.01), and globose nuclei (P less than 0.001), respectively. The principal pathologic change of the dentate and interpositus nuclei in Joseph disease was severe loss of neurons, with significant loss of the large neurons, indicating that Joseph disease is a type of cerebellar efferent system disorder.
A 59 year old woman developed pronounced thirst, increased water intake, and increased urinary output followed by slowly progressive cerebellar symptoms. Brain MRI showed abnormal hyperintensity on T2 weighted studies in the region of both dentate nuclei without atrophy of the cerebellum or the brainstem. A 99mTC diphosphonate bone scan showed bone lesions in the distal parts of both femurs as well as distal and proximal parts of both tibias. The diagnosis of Erdheim-Chester disease was made by bone biopsy. This is the first case of Erdheim-Chester disease presenting as a slowly progressive cerebellar syndrome and diabetes insipidus, and also showing high signal lesions in deep cerebellar nuclei on MRI. Skeletal surveys are indicated for patients with otherwise unexplained slowly progressive cerebellar symptoms.
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This morphometric and morphological study demonstrates 3 categories (types A, B and C) of degenerative feature in the cerebellar dentate nucleus. Type A is characterized by neuronal loss, astrocytosis and granular and/or amorphous argyrophilic change around the neurons and neuronal processes, and this type was thought to be synonymous with the so-called grumose degeneration of the DN. Type B is characterized by extensive neuronal loss and astrocytosis without argyrophilic change, and it was considered that many diverse factors were responsible for type B. Type C features marked swelling of the neurons without neuronal loss, astrocytosis or argyrophilic change. The Purkinje cells were not involved in type A and C, but severely damaged in type B. Clinically, type A was observed in progressive supranuclear palsy and dentatorubropallidoluysian atrophy, type B extensively in many diseases including anoxic, toxic and infectious disorders, and type C in tardive dyskinesia manifesting with oral hyperkinesia. Types A and C may be more or less specific signs of degeneration of the dentate nucleus, whereas type B appears to be non-specific.
Postural instability was quantitatively studied in patients with lesions in the 1) pontine tegmentum, 2) deep cerebellar nuclei, or 3) cerebellar hemisphere. As compared to controls, patients with each lesion demonstrated a characteristic 3 Hertz (Hz) body oscillation. Cross-correlation functions revealed a strong correlation between the body sway and activities in the lower leg muscles. Based on these findings, we conclude that the 3 Hz body oscillation may be the result of any disturbance in the loop of long-latency reflexes mediated by the cerebellum.
A case of neuro-Behçet's disease manifested as chronic progressive cerebellar ataxia is reported. A 56-year-old woman had suffered from recurrent oral aphthous ulcers, genital ulcers and polyarthritis since her late twenties. At age 53, she noticed small stepped-gait; at age 55, she developed scanning speech, a wide-based gait and memory disturbance. On admission she had oral aphthous ulcers, scarring of genital ulcers and polyarthralgia. A neurological examination revealed memory disturbance, saccadic eye movement, scanning speech, a slow tongue wiggle, moderate limb and truncal ataxia and moderate hyperreflexia in four limbs without pathological reflexes. Relevant laboratory examination data showed a positive HLA-B51. The cerebrospinal fluid (CSF) had a mild elevation of the cell counts and the amounts of protein. Brain magnetic resonance imaging showed mild atrophy of the cerebellum and brainstem. Fluoro-2-deoxyglucose-positron emission tomography showed a decreased glucose metabolism in the cerebellum and brainstem. Methylprednisolone pulse therapy (1 g x 3 days) followed by oral corticosteroids (50 mg/day) with gradual tapering markedly alleviated the cerebellar ataxia. The presence of oral and genital ulcers and CSF pleocytosis as well as effectiveness of corticosteroids in relieving the neurologic symptoms suggested neuro-Behçet's disease. We propose the existence of a new subtype of neuro-Behçet's disease characterized by chronic progressive cerebellar involvement possibly due to microvasculitis for which corticosteroids may be effective.
Pick's disease chiefly is characterized by progressive degeneration of specific telencephalic cortical areas and associated subcortical nuclei. Components of the cerebellum also are affected. Immunoreactions for abnormally hyperphosphorylated tau protein, indicating the development of cytoskeletal anomalies in a few susceptible neuroectodermal cell types, permit visualization and identification of the pathology. Initially, accumulations of nonargyrophilic material appear in the perikarya and cellular processes of susceptible nerve cells. In some neuronal types, the abnormal deposits are transformed into more condensed inclusions, so-called Pick bodies in perikarya and Pick neurites in cellular processes, some of which become argyrophilic in the course of the disease. This study employs silver techniques and immunoreactions to draw attention to Pick's disease-associated lesions in the cerebellar cortex and cerebellar nuclei. Immunoreactive rosettes, which correspond to the terminal synaptic boutons of mossy fibers, frequently are encountered in the cerebellar granule cell layer. Some cases of Pick's disease also exhibit afflicted monodendritic brush cells in this layer. Single immunopositive Purkinje cells occasionally are seen as well. The brunt of the alterations is borne by cerebellar subdivisions receiving dense input from the telencephalic cortex through the pontocerebellar pathway (neocerebellum). The dentate nucleus shows immunoreactive axons with numerous varicose thickenings which remain confined to the reaches of this band-like nuclear gray and probably represent collaterals of altered mossy fibers. A large number of the dentate projection cells also contain the abnormal material in the perikarya, as well as in all of the neuronal processes. Many of these cells develop spherical nonargyrophilic condensations of this material. Output of the neocerebellum is conveyed to extended territories of the telencephalic cortex via the dentate nucleus and thalamus. Therefore, all of the cerebellar territories which receive major input from and generate output chiefly to the telencephalic cortex (pontocerebellum or neocerebellum) are notably afflicted in Pick's disease. Other subdivisions with preponderant input from the spinal cord and/or other noncortical sources remain intact or else are only minimally involved. It is concluded that the pattern of cerebellar involvement reflects Pick's disease-associated neocortical destruction.
In 31 patients with completed stroke (n = 30) or PRIND (n = 1) a brain SPECT with 123I-labeled amphetamines was performed. In 14 (= 45%) of the respective patients--suffering from long-lasting hemiplegia--crossed cerebellar diaschisis was present. The interval between onset of the disease and time of examination varied between 1 week and 7 years. On the other hand, patients without crossed cerebellar diaschisis did not, with one exception, suffer from hemiplegia. It is likely that this phenomenon is caused by the reduction of spino-cerebellar stimuli due to the paresis of the respective extremities.
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We used positron emission tomography with [18F]fluorodeoxyglucose to study local cerebral metabolic rates for glucose (ICMRglc) in patients with multiple system atrophy (MSA), sporadic olivopontocerebellar atrophy (sOPCA), and dominantly inherited olivopontocerebellar atrophy (dOPCA) in comparison with normal control subjects. IN MSA, absolute lCMRglc was significantly decreased in the brainstem, cerebellum, putamen, thalamus, and cerebral cortex. In sOPCA, absolute lCMRglc was significantly decreased in the brainstem, cerebellum, putamen, thalamus, and cerebral cortex. In dOPCA, absolute lCMRglc was significantly decreased in the brainstem and cerebellum but not in the other structures. Examination of lCMRglc normalized to the cerebral cortex in comparison with normal controls revealed in MSA significant decreases in the brainstem, cerebellum, and putamen but, in both sOPCA and dOPCA, significant decreases only in the brainstem and cerebellum. The findings indicate that these three disorders all show a marked decrease of lCMRglc in the brainstem and cerebellum but differ in the degree of hypometabolism in forebrain and cerebral cortical structures. The results are consistent with the possibility that, in many cases, sOPCA will evolve into MSA. Moreover, positron emission tomography may provide helpful diagnostic information in these neurodegenerative diseases.
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