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PubMed1994

A case of Pick's disease with unusual neuronal inclusions.

An autopsy case of unusual Pick's disease in a 61-year-old male is described. Findings included severe atrophy of the frontal and temporal lobes, pyramidal tracts and basal ganglia accompanied by numerous intraneuronal argyrophilic hyaline inclusions. His neurological symptoms were constantly progressive during the 12-year course, characterized by akinesia and emotional incontinence. The inclusions were round, well-demarcated, slightly eosinophilic and intensely argyrophilic bodies in the perikarya, and distributed mainly in the subiculum and Sommer's sector of the hippocampus, amygdala and affected gyri. Immunocytochemically, they contain antigenic determinants of both phosphorylated and nonphosphorylated neurofilaments, but were negative for ubiquitin. Ultrastructurally, they were composed primarily of skeins of neurofilaments intermingled with cell organelles. Tubular profiles studded with granular substances, previously reported as a feature of the generalized variant of Pick's disease, and Hirano body-like lattice structures were occasionally observed in the inclusions. This case represents a slowly progressive neurodegenerative disorder characterized by fronto-temporal lobar atrophy and might by categorized as a variant of Pick's disease. However, some unusual properties of neuronal inclusions may suggest a different pathogenesis from that in classical Pick's disease.

Dementia↗
PubMed1991

Peripheral and central distal axonopathy of suspected inherited origin in Birman cats.

Three female cats, littermates born from clinically normal parents, were examined at 8 to 10 weeks of age because of a slowly progressive posterior ataxia. Another cat from a previous litter from the same parents suffered from similar neurological symptoms. Histopathological examination of the nervous tissues of these animals revealed degeneration of axons and myelinopathy in a distal distribution pattern. Both peripheral nerves and central nervous system were involved. The central nervous system lesions were most prominent in the lateral pyramidal tracts of the spinal cord, the fasciculi gracili of the dorsal column in the cervical spinal cord and the cerebellar vermian white matter. In the PNS numerous degenerating nerve fibers were found in the sciatic nerves but not in the spinal nerve roots. Our findings show that these cats were suffering from a hereditary multisystem degeneration with a distribution pattern of the lesions suggestive of a distal axonopathy.

Animals↗
PubMed1995

Clinical and neuropathological features of a neurodegenerative disorder in the central nervous system with progressive head drooping (Kubisagari).

The clinical and neuropathological features of a case of a neurodegenerative disorder with pronounced and progressive head drooping, in Japanese Kubisagari, are reported. This female patient died at the age of 72 years after an approximately 20-year history of peculiar posture with progressive head drooping (Kubisagari) and lordosis (bowed posture), parkinsonism, dysphonia and slight muscle wasting of the face, tongue, neck, and distal portions of the upper extremities. She did not display mental deterioration until the terminal stage of the illness. A simple macroscopic inspection formalin-fixed sections of the central nervous system (CNS) showed prominent atrophic frontal and temporal lobes, brownish discoloration of the putamen and an atrophic pyramidal tract. Light microscopy revealed severe neuron loss with fibrillary gliosis at both the above-mentioned lobes and the putamen. Both the facial and hypoglossal nuclei had almost disappeared. Motor neurons in the spinal cord were moderately to markedly decreased. Neither Bunina nor Lewy bodies, senile plaque, nor Pick's argyrophilic neuronal inclusions were observed, but very occasionally ubiquitin-positive neurons were found in the temporal cortex. In conclusion, the hitherto-unrecognized neuropathological findings in the CNS corresponding to progressive head drooping (Kubisagari) suggest that this is a neurodegenerative disorder of the CNS, possibly an atypical form of amyotrophic lateral sclerosis.

Aged↗
PubMed1991

Cerebro-ocular dysplasia--muscular dystrophy (Walker Warburg) syndrome. Findings in 20-week-old fetus.

A 20-week fetus affected with cerebro-ocular dysplasia and muscular dystrophy (Walker-Warburg Syndrome) is reported. The central nervous system (CNS) findings were typical of those previously described in this disorder, and were characterized by lissencephaly, hydrocephalus, and cerebral and cerebellar cortical dysplasia with glial and neuronal displacement into the leptomeninges. In addition, severe hypoplasia of pyramidal tracts were noted in the brain stem and spinal cord, as well as malformation of the inferior olivary and dentate nuclei. Skeletal muscle and eyes appeared normal on light microscopy. The genetic defect in this disorder is expressed in the CNS early during the first trimester and causes a marked disorder of cellular migration. Overt changes in muscle occur during a later period. The changes in the CNS are similar to, but more severe than, those found in Fukuyama congenital muscular dystrophy, and both may represent a failure of constraint of neuronal migration. Whether the syndromes characterized by cerebro-ocular dysplasia and muscular dystrophy are genetically heterogeneous or allelic variations is unknown. Molecular genetic analysis should elucidate this question.

Brain↗
PubMed1990

An adult-onset case of sporadic motor neuron disease with basophilic inclusions.

A 36-year-old man developed motor neuron signs consisting of weakness and atrophy of the right upper limb, which progressed to involve the other limbs along with development of upper motor neuron signs including pseudobulbar palsy. He died 8.5 years after onset. Bilateral precentral gyri and putamina were grossly atrophic. In addition to severe degeneration of bilateral pyramidal tracts and marked neuronal cell loss of the precentral gyri and putamina, basophilic inclusions were widely distributed in the motor cortex, putamina, general somatic motor neurons such as the hypoglossal nucleus and spinal anterior horns, and other areas like the red nucleus and inferior olive. The inclusions were clearly shown with Nissl stain to be anilinophilic irregular masses with distinct rims. Ultrastructurally the inclusions appeared to consist of thick filamentous structures of 12-25 nm in diameter studded with electron-dense ribosome-like granules. Thick filamentous profiles were relatively short or occasionally fragmentary, haphazardly mingled with various amounts of granules and other organelles. No prominent accumulation of 10-nm neurofilaments or eosinophilic inclusions like Bunina bodies were found. The inclusions were indistinguishable from those reported in so-called "juvenile" amyotrophic lateral sclerosis.

Adult↗
PubMed1990

An autopsy case of human T lymphotropic virus type I-associated myelopathy (HAM) with a duration of 28 years.

An autopsy case of human T lymphotropic virus I-associated myelopathy (HAM) of a duration of 28 years in a 61-year-old man with serological confirmation of HTLV-I infection was reported. The spinal cord was grossly atrophic. There was severe symmetrical degeneration of the lateral funiculi, particularly of the bilateral pyramidal tracts, involving all levels of the spinal cord, but anterior horn cells were relatively well preserved. In the most severely damaged middle and lower thoracic segments, the white matter degeneration also involved the anterior funiculi. In the degenerated white matter, both the myelin and axons were equally lost and had been replaced by glial scars. Marked adventitial fibrosis was commonly seen in small parenchymal vessels in both the white and gray matter. Although there was no evidence of inflammation in the spinal cord, mild lymphocytic infiltration was occasionally observed in the subarachnoid and perivascular spaces in the brain stem, cerebellum and cerebrum. This case was considered as a burnt-out case of HAM.

Autopsy↗
PubMed1985

Mild central pontine myelinolysis: a frequently undetected syndrome.

Over a period of 1 year we diagnosed central pontine myelinolysis (CPM) in five patients all of whom survived, two of them with complete functional recovery despite extensive lesions on cranial computerized tomography and magnetic resonance imaging. Diagnosis was based upon the combination of an acute brainstem dysfunction with typical neuroradiological features; a history of chronic alcoholism or a preceding hyponatremia may serve as a diagnostic hint. The spectrum of symptoms ranged from severe tetraplegia and cranial nerve palsies to latent signs of pyramidal tract lesions and discrete ocular motor abnormalities. In two patients pontine and extrapontine manifestations of demyelination were confirmed neuroradiologically; in one patient a solely extrapontine manifestation was present. Thus it is reasonable that: (1) the incidence of comparatively mild forms of CPM as well as extrapontine manifestations are more frequent than hitherto assumed, (2) the clinical outcome of the syndrome is better than expected from earlier fatal case reports and is quite independent of the extent of the lesion as it appears with brain imaging methods.

Adult↗
PubMed1977

Effect of H+ on spontaneous neuronal activity in the surface layer of the rat medulla oblongata in vitro.

The effect of changing extracellular pH (pHe) on the spontaneous activity of neurons in brain slices taken from the ventral layer of the rat medulla oblongata was compared to the response of neurons in dorsal slices. In the ventral medulla, more than 50% of the neurons were excited by H+. These neurons were found just lateral to the pyramidal tract between the root of the hypoglossal nerve and the trapezoid body. In the dorsal medulla, low pHe caused an inhibition of activity in most neurons, although a few were excited. The fact that H+ elicted excitation predominantly in the ventral medullary substrate to respond to pHe changes. Depression of synaptic transmission within the neuronal network in the slice by reducing the [Ca2+]e and increasing the [Mg2+]e altered the nature of responses of neurons to H+: In the ventral medulla, the majority of neurons were inhibited by H+, whereas in the dorsal medulla more than 50% of neurons were excited. Therefore, "specificity" of the ventral medullary neurons seemed to be dependent upon intact synaptic connections. A possible role of acetylcholine-acetylcholinesterase system in the response of ventral medullary neurons to H+ is discussed.

Action Potentials↗
PubMed1979

Elimination of central chemosensitivity by coagulation of a bilateral area on the ventral medullary surface in awake cats.

Breathing and respiratory response to CO2 were observed in 6 awake cats and 1 control before and after bilateral coagulation of the formerly described area S (Schläfke and Loeschcke, 1967) on the ventral medullary surface under hyperoxic conditions. Ventilation decreased, PCO2 rose and CO2 response was almost or completely abolished in 4 cats, and moderately reduced in 2 cats. Inhalation of CO2 had an inhibitory effect on ventilation in two cases. In some instances the respiratory frequency was increased by CO2. Periodic breathing as well as spontaneous hyperventilation elicited by 'arousal' indicate parallels to the Pickwickian or Ondine's curse syndrome. No respiratory changes were produced by a lesion on the pyramidal tract medial to the area S. It is concluded that central chemosensitivity can be eliminated within the superficial layer of the area S. The loss of CO2 response seems to be correlated with complete destruction of the superficial nerve cells located within the area S (Petrovický, 1968) and degeneration within the ventral part of the nucleus paragigantocellularis.

Animals↗
PubMed1989

Stable human immunodeficiency virus encephalopathy in two infants receiving early intravenous gammaglobulin plus antimicrobial prophylaxis.

Two infants with human immunodeficiency virus (HIV) infection, encephalopathy, intrathecal anti-HIV IgG antibody production and (in one case) the presence of HIV antigen received monthly doses of intravenous gammaglobulin (IVGG) and daily antimicrobial prophylaxis starting at the ages of 6 and 9 months respectively. The follow-up over 15 and 12 months revealed a favourable course with remarkable improvement in visuo-spatial functions, receptive language, play behaviour and fine motor skills, as well as in muscle tone, pyramidal tract signs and vigilance in case 1, and near normalization in case 2. Viability of HIV in peripheral blood mononuclear cells, antigen in serum and cellular immunodeficiency, however, all remained unchanged. We suggest that neurological complications of encephalopathy in paediatric acquired immunodeficiency syndrome may have a slower progression when IVGG treatment plus antimicrobial prophylaxis is started early.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Penetration of praziquantel into cerebrospinal fluid and cysticerci in human cysticercosis.

Two patients with cysticercosis received praziquantel (PZQ) 75 mg/kg/day orally together with 30 mg prednisone daily for 3 weeks. The first patient presented with grand-mal seizures, a pyramidal tract syndrome and subcutaneous cysticerci, and the other had internal hydrocephalus necessitating drainage. Serial plasma samples were taken after the first dose of PZQ. Lumbar CSF was obtained from the first patient and ventricular CSF from the second. Subcutaneous cysticerci were removed from the first patient. PZQ in the specimens was assayed by GLC. For distribution between plasma and CSF a rate constant of 4.9 h-1 for free PZQ, corresponding to a t1/2 of 8 min or less for the non-protein bound fraction was calculated for Patient 1. In the second patient the distribution was so rapid that the rate constant could not be calculated. The difference in distribution rate might have been due to use of different sampling times or to a time lag in the entry of PZQ between the ventricles and the lumbar sac. The rate constant for distribution of the drug between plasma and parasites was 1.4 h-1, corresponding to a t1/2 of 30 min or less. Thus PZQ penetrates rapidly into the CSF. It enters the parasite more slowly, although still more rapidly than the plasma half-life of PZQ (1-1 1/2 h).

Adult↗
PubMed1983

Changes of steady state activity in motor cortex consistent with the length-tension relation of muscle.

Steady state activity of motor cortex (MI) neurons and muscles was examined in relation to joint position. Two monkeys performed either isometric or load-bearing isotonic contractions, at different joint positions and during variation of steady torque. In either condition, MI steady state firing rate were found to be related to the amount of muscular excitation necessary to adjust muscle tension to length at any given position and load. The results obtained from 526 neurons (including pyramidal tract neurons) demonstrate for 206 neurons a correlate of the length-tension relation of muscle in the motor cortex.

Animals↗
PubMed1986

Degeneration of Betz cells in motor neuron disease. A Golgi study.

A morphological study using a modified Golgi impregnation technique was carried out on Betz cells in the motor area of eight patients with motor neuron disease (MND) and age-matched controls without abnormal neuropathological findings during routine examination. Betz cells were categorized as normal (type I), or those with moderate (type II) or marked (type III) degeneration of dendrites. Types II and III Betz cells tended to be dominant in the cases of MND, whereas type I Betz cells constituted the highest percentage in most of the control cases. The frequently observed morphological alterations of Betz cells in MND were accumulation of lipofuscin, degeneration or loss of dendrites, and reactive astrocytic gliosis around the Betz cell soma. It was suggested that Betz cell changes in MND are not only due to aging but also to evident pathological processes, since the above-mentioned findings were more extensive in the MND cases than in the control cases. Also, the so-called lower motor neuron disease belongs to one basic disease termed "motor neuron disease", because the pathological changes of Betz cells were observed even in cases of spinal progressive muscular atrophy alone or associated with progressive bulbar palsy, which are devoid of pyramidal tract degeneration and signs of upper motor neuron lesion.

Aged↗
PubMed1985

Cerebellar plaques in familial Alzheimer's disease (Gerstmann-Sträussler-Scheinker variant?).

A large kindred, with two brothers coming to autopsy, of a syndrome consisting of ataxia, dementia, and some Parkinsonian features is reported; inheritance appears to be autosomal dominant. Neuropathologically, there were plaques and neurofibrillary tangles in the cerebral cortex as well as some in the basal ganglia, particularly reminiscent of the plaques seen in Kuru; there was only minimal spinal cord disease (pyramidal tract field). The problems of classifying this condition--Alzheimer's disease with cerebellar involvement or other entities, such as the Gerstmann-Sträussler-Scheinker condition (1936), especially now that transmission to animals in the latter has been reported--are discussed. Some relevant theoretical considerations derived from animal work, particularly in scrapie, are also reviewed.

Alzheimer Disease↗
PubMed1986

Extensive intraneuronal spread of horseradish peroxidase from a focus of vasogenic edema into remote areas of central nervous system. Observations on mouse central nervous system subjected to cortical cold injury.

A study was made of the uptake of horseradish peroxidase (HRP) into neurons from a cryogenic cortical lesion in the mouse brain associated with vasogenic edema, following intravenous administration of the tracer. Particular emphasis was placed on the axonal spread of HRP from the primary lesion to other areas of the central nervous system. The distribution of HRP was studied by light microscopy, using highly sensitive histochemical methods, 3-144 h after the onset of the injury. Extravasated HRP was taken up into nerve cell bodies in and around the primary lesion, forming different patterns of labelling: (1) granular, (2) diffuse, and (3) a combination of granular and diffuse staining. Granularity is considered to be the result of HRP accumulation in lysosomes occurring in undamaged or slightly damaged nerve cells, whereas the diffuse, non-granular pattern presumably occurs in severely damaged neurons. Nerve cell bodies containing HRP reaction product were also found in the contralateral cortex, ipsilateral thalamus, substantia nigra, amygdala and ventral tegmental area, presumably a consequence of retrograde axonal transport of the tracer from the primary injury. HRP-containing axons were present in the corpus callosum and in the pyramidal tract of the injured hemisphere all the way down to the cervical spinal cord. Labelling of axonal terminals and preterminal axons in the ipsilateral thalamus, entopeduncular nucleus, subthalamic nucleus, substantia nigra and pons indicated anterograde transport of HRP to these regions. Thus very extensive intraneuronal spread of a macromolecular edema component takes place from a primary focal brain lesion to areas located far away from but neuroanatomically connected to this injured region. The brain thus seems to be affected by focal vasogenic edema in many more ways than are recognized at present.

Animals↗
PubMed1985

Progressive diffuse leukoencephalopathy in patients with acquired immune deficiency syndrome (AIDS).

Two adult patients with acquired immune deficiency syndrome (AIDS) presented with psycho-organic symptoms produced by an extensive cerebral and cerebellar leukoencephalopathy. Diffuse loss of myelin and axons with reactive astrocytosis and distinctive multinucleated giant cells were prominent in the deep white matter, but less so in the subcortical white matter and in compact myelinated pathways. Bilateral involvement of the centrum semiovale produced distal Wallerian degeneration of the descending pyramidal tracts, which in one patient correlated with progressive paraparesis and bladder dysfunction. Although there were morphological indications of cytomegalovirus infection and immunohistochemical evidence of papovavirus antigens, the neuropathology did not resemble that usually associated with infection by these opportunistic agents. The possibility is entertained that the progressive diffuse leukoencephalopathy (PDL) in these patients was directly related to infection with human T-cell lymphotropic virus (HTLV-III/LAV), the etiologic agent of AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1977

Intra-axonal corpora amylacea in the CNS.

The distribution of intra-axonal corpora amylacea (IACA) in the CNS was studied in 2 cases of ALS, a hepatic encephalopathy and a Shy-Drager syndrome. A total of 149 IACAs were found. IACAs were observed far most frequently in the gracile nuclei, followed by the anterior horns of the spinal cord and in descending order; lateral geniculate bodies, reticular formation, sensory and motor nuclei in the brain stem, cerebellum and cerebral cortex. The basal ganglia, including the thalamus, contained only a few. IACA were not observed in the pyramidal tracts or in the spinal roots. About 3% of the IACA were sectioned longitudinally, together with the original small nerve fibres and abrupt enlargement of the axons due to the deposits was well demonstrated.

Amyloid↗
PubMed1979

Neurotoxicity of sodium cyanate. New pathological and ultrastructural observations in Maccaca nemestrina.

The effects of sodium cyanate (NaNCO) on the nervous system of Maccaca nemestrina were studied at 2, 4, and 6 months of administration of the drug. The two groups injected with daily doses of 35 and 25 mg/kg/day of Na-cyanate developed a predominantly demyelinating lesion in the pyramidal tracts of the spinal cord. No neuronal changes were observed in the motor cortex, basal ganglia, midbrain, medulla or anterior horn cells of the spinal cord. There was no evidence of peripheral neuropathy. A comparison between the cyanate induced neuropathy in the rat and in the primate was drawn. Ultrastructurally, both species developed a demyelinating process of central or peripheral myelin characterized by vacuolation of the myelin sheath, removal of myelin debris by macrophages and re-myelination. There was little evidence of axoplasmic damage except for an occasional distended fiber containing abundant dense bodies and whorls of neurofilaments. Oligodendrocytes and Schwann cells were electron microscopically intact and participated actively in remyelination. Maccacas maintained at 15 mg/day and sham animals remained normal clinically and anatomically. The predominantly myelinotoxic effect of cyanate is similar to that produced by other myelinotoxic agents and is attributed to a selective modification of myelin proteins by carbamylation.

Animals↗