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PubMed2004

Multifocal acquired demyelinating sensory and motor neuropathy: report of a case and review of the literature.

Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is characterized by an asymmetric multifocal pattern of motor and sensory loss, and conduction block and other features of demyelination in nerve conduction studies. MADSAM neuropathy needs to be differentiated from chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In classic CIDP, there are symmetric proximal and distal weakness, sensory deficit in both upper and lower extremities and reduced deep tendon reflex. In MMN, limb weakness without sensory loss is asymmetric in the distribution of individual peripheral nerves and the weakness typically begins in the distal upper extremities. We report one patient with chronic progression of asymmetric numbness and weakness in four extremities. MADSAM neuropathy was diagnosed after extensive clinical and laboratory evaluations. It is very important to distinguish between CIDP, MADSAM neuropathy, and MMN by clinical, laboratory, and histological features because of different effective therapeutic strategies.

Adult↗

PubMed2004

Infantile-onset glycogen storage disease type II (Pompe disease): report of a case with genetic diagnosis and pathological findings.

Glycogen storage disease type II (GSD-II), also known as Pompe disease, is a rare autosomial recessive disease due to deficiency of lysosomal acid alpha-glucosidase (GAA). The infantile-onset form is the most severe, and most patients present with hypotonia and cardiomyopathy in early infancy. We report on a typical case of Pompe disease in a patient who died at 8 months of age due to aspiration pneumonia and hypertrophic cardiomyopathy. Genetic studies showed deficient GAA activity and mutation of the GAA gene with Gly615Arg (exon 13, G1845A). On autopsy, glycogen had markedly accumulated in the liver, myocardium and skeletal muscle. The neurons of the anterior horn of the spinal cord and medulla were also involved, but the cortex was spared. These neurological-histologic findings may explain the clinical features of poor motor function, decreased deep tendon reflexes and lack of mental retardation.

Base Sequence↗

PubMed2004

[Diabetic Neuropathy. Diagnosis and Treatment in the Physician's Office].

The most common form of diabetic neuropathy is the distal symmetrical sensorimotor variety. It is all the more likely to develop, the longer diabetes mellitus has been clinically manifest. Clinical signs of this neuropathy include the loss of the Achilles tendon reflex, sensory disorders beginning distally, distal muscle paresis and such signs of autonomic nerve involvement as erectile disorders, disordered bladder emptying, diaphoretic disorders or orthostatic hypotension. The diagnostic work-up should also include a number of laboratory investigations to exclude other causes of neuropathy. The basis of treatment is the earliest detection of diabetes mellitus and the establishment of normoglycemia. The individual manifestations are treated symptomatically. For the treatment of pain, it may become necessary to apply opioids if antidepressants and anticonvulsants fail to prove effective.

Analgesics↗

PubMedDate not supplied

Lambert-Eaton myasthenic syndrome. Clinical and electrophysiological findings in seven cases.

We report the clinical and electrophysiological findings in seven patients with Lambert-Eaton myasthenic syndrome (LEMS). All patients were males aged 40-73 years old. Six presented proximal muscle weakness and one both proximal and distal. The tendon reflexes were absent in four patients, depressed in two and normal in one patient. Three patients presented ophthalmic and four autonomic symptoms. The syndrome was diagnosed 3-12 months after the onset of symptoms in six patients and 4 years later in one. Acetylcholine receptor antibodies were negative in all patients. Voltage-gated calcium channel antibodies (VGCC) were measured in five patients and were positive in four. All patients had low compound muscle action potential (CMAP) at rest, a decrement in CMAP amplitude of 20-47% at 3 Hz repetitive nerve stimulation, and an increment of 200-700% at 40 Hz. In three patients the syndrome was associated with histologically verified small-cell lung cancer (SCLC). In the younger patient (40 years old), a lymph node biopsy performed nine years before the diagnosis of LEMS, had shown an atypical microcellular cancer of undetermined origin, which was treated with chemotherapy. LEMS 9 years after the diagnosis of cancer has not been described previously. The fifth patient had a two years history of bladder cancer (grade II). Three years after the diagnosis of LEMS he presented chronic lymphogenic leukemia. No malignancy was found in the remaining 2 patients.

Action Potentials↗

PubMed1992

Nonoperative management of herniated nucleus pulposus: patient selection by the extension sign. Long-term follow-up.

We have previously reported that the ability of patients with herniated nucleus pulposus (HNP) and radiculopathy to achieve full passive lumbar extension (negative extension sign) is a useful predictor of favorable response to conservative management. Of 154 patients with back pain and radiculopathy seen between 1979 and 1985, 73 were selected for conservative management based on this criterion. We also reviewed results of electromyography (EMG), computed tomography (CT), myelography, straight leg raise, crossed straight leg raise, deep tendon reflex, and dermatomal-sensory and motor deficits. Ninety-four percent of the subjects achieved full extension within 5 days of hospital admission. Although some patients who had full extension on admission subsequently underwent chemonucleolysis or surgery, none of those who could not extend initially and who later gained full lumbar extension required surgery. Of the 33 patients who were available for long-term follow-up, 94% were satisfied with the results, 82% were able to resume working, 73% required no analgesics, and 9% later required chemonucleolysis or surgery. Positive findings on admission EMG (5 of 7 patients), myelography (5 of 8 patients), or CT (9 of 11) did not preclude good results with conservative management. Female patients without complaint at follow-up (53%) outnumbered their male counterparts (25%). Patients (19 of 33) whose extension sign was initially positive on admission and became negative within 5 days of hospitalization (average, 1.6 days) had better outcomes with 95% satisfied, 90% without job changes, 74% not requiring analgesia, and none requiring surgery. At long-term follow-up, the extension sign effectively predicted a favorable response to nonoperative therapy of HNP in 91% of cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed2004

[Clinical features of neuropathies in a group of patients associated with anti-GalNAc-GD1a antibody].

To clarify the clinical features of patients with anti-GalNAc-GD1a antibody, we retrospectively investigated which conditions were associated with anti-GalNAc-GD1a antibody in a large number of patients. Sixty-four out of 1,713 patients had anti-GalNAc-GD1a IgG antibody. Fifty-seven (89%) were diagnosed with Guillain-Barré syndrome (GBS) or atypical GBS with preserved deep tendon reflexes. These patients were characterized by young man, antecedent diarrhea, distal-dominant limb weakness, and absence of cranial nerve impairment and sensory disturbance. Some of sera from patients had anti-GM1, anti-GM1b, and anti-GD1a IgG antibodies associated with axonal GBS. Our study suggests that the anti-GalNAc-GD1a antibody testing is useful for supporting the diagnosis of GBS. In addition, fine specificity of the antibody also was clarified.

Autoantibodies↗

PubMed2004

[A variant of Guillain-Barré syndrome with prominent bilateral peripheral facial nerve palsy--facial diplegia and paresthesias].

A patient with Facial diplegia and paresthesias, a rare regional variant of Guillain-Barré syndrome (GBS), is described. A 37-year-old woman developed paresthesias in the distal limbs and subsequently bifacial weakness. She had had a preceding episode of laryngitis. Neurological examination showed severe facial diplegia with loss of taste sensation on the tip of the tongue. Limb muscle power was preserved. Deep tendon reflexes were generally absent. She complained of paresthesias in the distal limbs, but sensory examination was normal. Cerebrospinal fluid showed albuminocytological dissociation. Electrophysiological studies revealed severe facial nerve involvements and demyelinative findings in her limbs. Intravenous immunoglobulin rapidly improved the abnormal sensation in her limbs. Although facial diplegia gradually lessened after the therapy, mild residual weakness was noted 6 months after the neurological onset. To diagnose facial diplegia and paresthesias, it is important to clarify the findings common to typical GBS such as the antecedent illness, acute and monophasic course, distal paresthesias, areflexia, cerebrospinal fluid albuminocytological dissociation, and demyelinative conduction abnormalities in the limbs.

Adult↗

PubMed2001

[Botulinum toxin treatment of hip adductor spasticity in multiple sclerosis].

Spasticity results in a resistance to passive movement and decrease of passive mobility of the involved joints and is defined as a state of hypertonicity with exaggeration of tendon reflexes mediated by a loss of inhibitory control of upper motor neurons. In patients with severe stages of multiple sclerosis (MS) spasticity of the lower limbs often leeds to a spastic pattern with hip adduction resulting in decreased range-of-motion (ROM), increased pain, spasms, and functional disability (disturbed gait and sitting position) as well as difficulties with perineal hygiene. Local botulinum toxin type A (Btx-A) injections in spastic muscles offer a new treatment approach for managing spasticity and associated problems. Up to now Btx-A is approved for the treatment of blepharospasm and cervical dystonia and the treatment of equinous gait in children with cerebral palsy in Austria and Germany. Up to now only in Switzerland Botox is licensed for the treatment of focal spasticity. Btx-A is a neurotoxin derived from Clostridium botulinum. In most european countries Btx-A is available as Dysport (vial = 500 units) and Botox (vial = 100 units). In prospective studies a ratio of 1 unit Botox to 3-4 units Dysport was found. Following intramuscular injection Btx-A blocks the release of acetylcholine at the neuromuscular junctions, thereby inhibiting muscle contraction, and decreases spastic muscle tone and muscle spindles afferent information to the spinal cord. The spectrum of side effects includes local weakening of the injected and adjacent muscles as well as pain and haematoma at the injection site. At therapeutic doses side effects are local and transient. According to a double blind, placebo controlled, dose ranging study published by Hyman et al. (2000, Dysport in a dose of 500, 1000 and 1500 units reduced the degree of hip adductor spasticity associated with MS, and this benefit was evident despite concomitant use of oral antispasticity medication. According to the results of the study there was a clear trend towards greater efficacy and duration of effects with higher doses of Dysport. Taking efficacy and adverse events into account (incidence of muscle weakness was higher for the 1500 units group than for placebo) the optimal dose for hip adductor spasticity seems to be 1000 units Dysport divided between the adductor magnus, longus and brevis muscles and between both legs. To increase Btx-A effects following injection of hip adductors additional physiotherapy and casting or orthosis to increase passive hip-abduction is recommended. According to the literature anatomical localisation of the adductor muscles for injection and aspiration following insertion of the needle, to avoid injection of the toxin into a vessel, should be performed. A maximum dose of 1500 units Dysport (400 units Botox) per treatment session and 250 units Dysport (50 units Botox) per injection site is recommended. See table for dose-range of Dysport, and Botox in the treatment of adult patients with hip-adductor spasticity. For evaluation of treatment effects in hip adductor spasticity clinical examination with specific scales and measurements (see Appendix) at baseline, 4 and 12 weeks following BtxA injection is recommended:--Global rating of severity (0-4; patient's self assessment and physician's rating) --Global rating of response (-4 - +4; patient's self assessment and physician's rating)--Visual Analogue Scale (patient's self assessment of pain)--Active and passive ROM (manual goniometer)--Distance between the medial femur condyles in thigh extension (distance in cm)--Modified Ashworth scale (0-4)--Ten meter walking time (seconds)--Functional Ambulation Categories (0-5)--Score of perineal hygiene (0-5).

Adult↗

PubMed2004

[A case of adolescent adrenoleukodystrophy with clinical improvement after allogeneic bone marrow transplantation (allo-BMT)].

We reported a case of adolescent type adrenoleukodystrophy. A 20-year old man noticed slight hemiparesis on his right side. After admission, high level of serum VCLFA and ACTH and a point mutation in ALDP codon 606 were recognized along with white matter lesions in the left medulla, basal ganglia, splenium of the corpus callosum on brain MRI. Then we diagnosed the patient as having adolescent type adrenoleukodystrophy. We started Lorenzo's oil and seaching for BMT donor. Six months later, he received allo-BMT at our hospital. His clinical symptoms gradually deteriorated during waiting allo-BMT, and just before allo-BMT, right hemiparesis, gait disturbance, exaggerated deep tendon reflexes on his right side, right lower quadrantanopia and mild cognitive deterioration. Two months after allo-BMT, gait disturbance and right hemiparesis were alleviated. We also observed improvement of abnormal findings on brain MRI and magnetic stimulation. We believed that allo-BMT is effective for the treatment of adolescent type adrenoleukodystrophy.

Adrenoleukodystrophy↗

PubMed2004

[A case of dementia with motor neuron disease associated with agraphia--the omission of kana letters].

We report a 55-year-old right-handed Japanese man with motor neuron disease and dysgraphia of kana letters. He was admitted to our hospital because of dysarthria and dysphasia. On admission, the results of general physical examination were within normal limits. Neurological examination revealed severe dysarthria, dysphasia, impaired movement of the tongue without fasciculation and slight distal muscle weakness in the bilateral upper limbs. There were no fasciculation of the muscle. Deep tendon reflexes were hyperactive without Babinski's signs. Sensation, coordination, and gait were normal. Neurophysiological studies demonstrated normal motor nerve conduction velocities and sensory action potential. The results of needle electromyography of the upper limbs were compatible with motor neuron disease (MND). Magnetic resonance imaging (MRI) showed atrophy of the bilateral temporal region of the brain. 99mTc-HMPAO SPECT (Single Photon Emission Computed Tomography) showed reduced uptake of tracer in the bilateral temporal region. On neuropsychological examination, his behavior was normal, and orientation and intelligence were also preserved, but his speech was severely impaired. Reading comprehension was slightly impaired. In regard to writing comprehension, he had no difficulty in copying of words though dictation was found to be impaired. He omitted one kana letter in a word. Agraphia is accompanied by various factors such as aphasia, dementia, agnosia, alexia. But in this case at least for early stage, agraphia existed without other higher cortical dysfunction. He did not show severe dementia in his early stage of his disease, but developed it later in the disease's progression. In this case, agraphia might be due to the atrophic changes in the temporal lobe.

Agraphia↗

PubMed1992

[Extrapyramidal manifestations in hereditary olivopontocerebellar atrophy--clinical study of 10 cases in three affected pedigrees].

We studied 3 pedigrees affected with autosomal dominant olivopontocerebellar atrophy (OPCA), and clinical features of 10 patients were reported. Clinically, 6 cases were dominated with cerebellar ataxia, one with Parkinsonism, and three with choreiform movements. Furthermore, they were characterized by slow eye movement and progressive loss of tendon reflexes. Generally, extrapyramidal manifestations, such as Parkinsonism or choreiform movements, are occasionally observed in dominant OPCA. However, the pathogeneses are different from each other. The choreiform movements are unique in dominant OPCA, and generally not observed in sporadic OPCA. In the distribution of degenerated foci, dominant OPCA is often associated with degeneration of not only substantia nigra, but also with other structures such as dentate nucleus, red nucleus, external segment of globus pallidus (GPe), and subthalamic nucleus. This dentatorubral and pallido-Luysian system degeneration are parts of neuropathological findings in hereditary DRPLA (Naito-Oyanagi form). In both disorders, choreiform movements is observed. Current theory indicates that hyperactivity of neostriatal dopamine neurons and degeneration of GPe play roles in the pathogenesis of chorea. Clinically, choreiform movements are common in hereditary DRPLA, but not in dominant OPCA. This difference could be explained by the nigral degeneration in dominant OPCA, which suppress the generation of choreiform movements.

Adult↗

PubMed1992

[The effect of high-dose peroral zidovudine treatment in a 4-year-old child with HIV encephalopathy].

We report a four-year-old boy with HIV encephalopathy after vertical HIV infection. On the first admission to our hospital he showed ataxia, loose of expressive language and interstitial pneumonia. After treatment of the pneumonia the patient was started on oral zidovudine with 6 x 6 mg/kg bw/day, because of persisting neurologic symptoms and deep white matter lesions in the MR tomogram of the brain. Two months later he showed an improvement of the gait and the reappearance of the expressive language. Seven months after the start with zidovudine the MR tomogram of the brain revealed the disappearance of white matter lesions with exception of little areas of demyelinisation. No side effects of treatment were observed. The only persisting pathological clinical signs were developmental delay of about a half year and moderately hyperactive tendon reflexes of the lower extremities. Our case suggests that even oral treatment with zidovudine can have a beneficial effect on the HIV encephalopathy in infants.

AIDS Dementia Complex↗

PubMed2004

Neuromuscular weakness in critically ill.

Neuromuscular weakness in critically ill has been increasingly reported in the recent years. Occasionally it may lead to difficulty in weaning the patients from mechanical ventilation, which is difficult to diagnose clinically. Though in well-planned studies the incidence has been reported to be high, the diagnosis is often missed due to the presence of various confounding factors in the form of drug effects, underlying disorder and coexisting abnormalities of the central nervous system. A high index of suspicion with detailed neurological and electrophysiological examination is required for an early and accurate diagnosis. A wide spectrum of disorders and drugs can be responsible for the critical illness neuromuscular abnormality. The most frequent and defined disorders include; critical illness polyneuropathy (CIP) which is characterized by a sensorimotor reversible polyneuropathy presenting as distal symmetrical weakness with loss of deep tendon reflexes. Acute myopathy is another important disorder in this group which usually presents with quadriplegia often related to steroid use. Persistent blockade of neuromuscular junction is also defined in critically ill patients. It is, therefore, important to understand these disorders and their implications in the management of these patients. Some of the conditions require prolonged neuro-rehabilitation. The various acquired disorders leading to neuromuscular abnormalities in critical care, and their diagnosis and management are discussed.

Critical Illness↗

PubMed2004

[Anterior spinal artery syndrome due to cervical spondylosis presenting as cervical angina].

A 54-year-old woman developed acute progressive paraparesis after repeated precordial pain. Neurological examination revealed bilateral four-limb weakness predominant in the distal part of the upper limbs, upper limbs brisk tendon reflexes, superficial sensory impairment below the C8 level, and atonic bladder. T2-weighted cervical MRI disclosed hyperintense lesion with disc herniation in gray matter of spinal cord between C5 and C7. No vertebral artery abnormalities were detected. We hypothesized that she developed anterior spinal artery syndrome after cervical angina caused by cervical spondylosis. We conclude that physicians need to be aware of patients who experience chest pain without evidence of cardiac disease and that they take into consideration spinal cord infarction.

Angina Pectoris↗

PubMed1992

[A case report of cervical disc hernia presenting fecal incontinence evoked by walking; spinal intermittent rectal dysfunction].

Intermittent clinical manifestations, the representative one of which is claudication, can be classified into two types; neurogenic and vasculogenic. Although cauda equina lesions are well known as a neurogenic cause, spinal disorders, especially cervical or thoracic cord lesions, have been paid more attention to by several authors recently. We encountered a 42-year-old man with cervical soft disc hernia and ossification of longitudinal ligament, who showed intermittent rectal dysfunction evoked by walking. This peculiar clinical manifestation successfully disappeared after surgical decompression of the spinal cord. Such an interesting case has not been reported in the world literature. The patient was admitted because of numbness in both hands. Neurological examinations on admission showed neither motor weakness nor abnormally increased tendon reflex. Hypesthesia and hypalgesia were noticed below the Th4 dermatome on both sides. Adding to those symptoms, he complained of fecal incontinence evoked by walking 100 meters. This rectal dysfunction became gradually worse. At last he showed fecal incontinence after walking only 10 meters. This was ten days after his admission. Myelogram and computed tomographic scan revealed a cervical soft disc hernia at the C5/6 level and findings of OPLL at the C5 and C6 level. Anterior cervical approach for OPLL and soft disc was used for bone graft insertion from the C4 to the C7 vertebral body. The rectal dysfunction completely disappeared after the operation. The possible mechanisms of intermittent rectal dysfunction evoked by walking were discussed.

Adult↗

PubMed2005

[An autopsy case of origin-unidentified meningeal carcinomatosis presenting with monoradiculopathy multiplex in the lower extremities].

A 54-year-old man with a history of partially dissected epidermoid cyst in the left cerebellopontine angle suffered from a slowly progressive dysesthesia and weakness in the lower extremities and trunk. Neurological examination revealed segmental muscular weakness and sensory disturbance in those regions, giving rise to the possibility of monoradiculopathy multiplex, as well as loss of tendon reflexes, dysuria and right facial nerve palsy. Electrophysiological studies indicated irregular motor axonopathy or neuronopathy and interruption of more central sensory pathways than the lumbosacral spinal nerve roots with spared peripheral sensory nerves. Although MRI demonstrated enhanced lesions in the cauda equina and lumbosacral leptomeninges, CSF cytology or a cauda equina biopsy showed no malignancy. His symptoms gradually progressed and he died 15 months after the onset. The autopsy failed to reveal any tumors in the general systemic internal organs. Histopathology demonstrated meningeal carcinomatosis with squamous-type carcinoma cells scattered in the cerebrospinal leptomeninges, and perineurium in almost all the spinal and cranial nerve roots, causing severe axonal degeneration. The dorsal root ganglions escaped tumor cell invasion. Absence of the malignant tumors in the systemic organs and the history of the operated epidermoid cyst indicate that the tumor may be the cause of the meningeal carcinomatosis in this case. Meningeal carcinomatosis almost always shows rapid progression and extremely poor prognosis with several month survival in general, and little attention has been paid that it can exhibit symptoms and signs of segmental involvement in the lumbosacral regions. Our present case prompts us to bear in mind that patients with this condition can survive fairly long, and raises the possibility that a careful neurological examination with segmental involvement will reveal such a feature more frequently than considered so far in this condition.

Carcinoma, Squamous Cell↗

PubMed2001

[Lambert-Eaton myasthenic syndrome: diagnosis and treatment].

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of neuromuscular and autonomic transmission in which IgG autoantibodies lead to presynaptic voltage-gated calcium channel (VGCC) loss, or as a paraneoplastic disorder in association with small cell lung carcinoma (SCLC). Recent results strongly suggest that the antibodies to P/Q-type VGCC are the principal pathogenic factors in LEMS. Here, we present diagnosis and treatment of LEMS patients. Proximal weakness, depressed tendon reflexes, autonomic symptoms, and electrical posttetanic potentiation together are essential to accurately diagnose LEMS. The diagnosis is established immunologically by the presence of anti-P/Q-type VGCC antibodies, detected using the (125)I-omega-conotoxin MVIIC radioimmunoassay, which will be present in 85% of LEMS patients. The drug 3,4-diaminopyridine with anti-cholinesterase inhibitor is most effective in LEMS patients with or without SCLC. In LEMS with SCLC, specific tumor therapy will often improve the neurological disorder. In some cases plasmapheresis or intravenous immunoglobulin may be indicated.

English Abstract↗

PubMed2005

[A 65-year-old man with rigid-bradykinetic parkinsonism, vertical gaze palsy, difficulty of eye-lid opening, and marked pseudo-bulbar palsy].

We report a 65-year-old man with rigid-bradykinetic parkinsonism, vertical gaze palsy, difficulty in eye-lid opening, and marked pseudo-bulbar palsy. He felt difficulty of it, hand movement at 59 years old. When he was 60 years old, monotonous speech and slowness of movement appeared. He visited a neurologist who noted vertical gaze palsy, neck rigidity, and bradykinesia. He was diagnosed as progressive supranuclear palsy (PSP) and given 300 mg L-Dopa/Benserazide by the neurologist. This medication improved his rigidity and bradykinesia. At 62 years of the age, his eye-lids closed involuntary and it was difficult to open. In addition, he began to complain of wearing-off, autonomic symptoms, and dysphagia. Anti-parkinsonian drugs were increased, but his bradykinesia progressed. At 64 years of the age, he was admitted to the Neurology Service of Juntendo Hospital. On admission, he was alert and not demented. No aphasia, apraxia, or agnosia was noted. In the cranial nerves, upward and downward gaze were markedly restricted. His face was hypomimic and seborrhoic. It was difficult to swallow liquid or solid for him. No weakness was noted, but he walked in small steps with freezing and falling tendency to backward. Rigidity was noted on his extremities and stronger on his left side than right. Tremor was absent. Bradykinesia of his body and extremities was marked. No cerebellar ataxia was noted. Deep tendon reflexes were within normal range. Planter response was flexor bilaterally. Myerson's sign was noted. Sensory and autonomic function were normal. He was treated with L-Dopa, Pergolide, and Bromocriptine. However, these medications improved his bradykinesia and gait disturbance only slightly, dysphagia became progressively worse. He developed aspiration pneumonia when he was 65 years old and admitted to Juntendo Hospital. A large amount of sputum was aspirated from his trachea. Two days after from admission, he was found dead on his bed. He was discussed in a neurological CPC and the chief discussant arrived at a conclusion that the patient had progressive supranuclear palsy (PSP). Other differential diagnoses included Parkinson's disease, pallido-nigroluysian atrophy (PNLA), multiple system atrophy (MSA), and corticobasal degeneration(CBD). Many participants considered that PSP or PNLA was most likely. Post-mortem exmination revealed marked nigral neuronal loss and gliosis. The globus pallidus and the luysian body changed mildly. However, the frontal cortex was relatively spared, there were many ballooned neurons in the cortical layer. Other parts were spared. With sliver (Bodian and Gallyas-Braak) and anti-phsphorylated tau stain, abundant astrocytic plaques, neurofibrillary tangles, and argyrophilic threads on the frontal cortex, striatum, and substantia nigra were seen. There was no tufted astrocyte which was hallmark of diagnosis of PSP. In addition, several Lewy bodies were seen in the brainstem. Because astrocyte plaque was considered specific for pathology of CBD, the pathologist revealed that the pathological diagnosis of this patient was CBD. Nevertheless, discussion was focused on the relatively mild degeneration of the frontal cortex for CBD.

Aged↗