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ABSTRACT: 'Dropped head syndrome' (DHS) may be associated with a variety of neurological diseases. The absence of neurological clues to the underlying cause of DHS can make management particularly challenging. We review six patients who presented with only DHS, responded to intravenous edrophonium and turned out to have myasthenia gravis (MG) including similar patients who were previously documented. Six patients presented with neck weakness and three had bulbar symptoms. Acetylcholine receptor (AchR) was positive in four patients. One patient had thymoma. The interval from the onset of DH to the presentation of typical MG features was shorter in patients who tested positive for anti-Ach antibody (1-2 months) than in patients who tested negative for anti-AchR antibody (13 months, 4 years). Our results suggest that patients with DHS responding to intravenous edrophonium might turn out to have MG and such patients might respond to a combination of anticholinesterase agents and steroids.
Case Reports 01/2013; 2013.
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ABSTRACT: OBJECTIVES: Hyperthyroidism is often associated with various neuromuscular disorders, most commonly proximal myopathy. Peripheral nerve involvement in hyperthyroidism is very uncommon and has rarely been reported. We describe a 29-year-old woman with untreated hyperthyroidism who presented with chronic severe axonal sensory-motor polyneuropathy. Peripheral nerve involvement developed together with other symptoms of hyperthyroidism 2 years before presentation. She also had anorexia nervosa for the past 6 months, resulting in multivitamin deficiency. Results: Electrophysiological and pathological findings as well as clinical manifestations confirmed the diagnosis of severe axonal polyneuropathy. Anorexia nervosa has been considered a manifestation of untreated hyperthyroidism. We considered hyperthyroidism to be an important causal factor in the polyneuropathy in our patient, although peripheral nerve involvement in hyperthyroidism is rare. To our knowledge, this is the first documented case of chronic severe axonal polyneuropathy ascribed to both hyperthyroidism and multivitamin deficiency. Conclusion: Our findings strongly suggest that not only multivitamin deficiency, but also hyperthyroidism can cause axonal polyneuropathy, thus expanding the clinical spectrum of hyperthyroidism.
Neuro endocrinology letters 12/2012; 33(8). · 1.30 Impact Factor
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Parkinsonism & Related Disorders 12/2012; · 3.80 Impact Factor
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The Journal of infection 10/2012; · 4.13 Impact Factor
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ABSTRACT: Recently, we studied fallers and non-fallers with Hoehn-Yahr stage III Parkinson's disease (PD) using a path that suddenly narrowed, which we originally designed and produced. A risk of future falls was suggested to be related to slow gait with freezing (SGF) elicited by a fear of falling before arrival at a narrowed entrance or while walking on a narrow path, as well as to the Unified Parkinson's Disease Rating Scale part II score, associated with SGF. In the same study, we had faller patients walk on a path that narrowed in a straight-line fashion to determine whether SGF could be improved. In one patient, who showed a unique paradoxical gait, SGF resolved. We describe this patient in the hope that our experience will provide potential clues to effective ways to prevent future falls in patients with Hoehn-Yahr stage III PD. To prevent gait instability elicited by fear of falling in patients with Hoehn-Yahr stage III PD, it might be useful to remove narrowed entrances.
European Neurology 10/2012; 68(5):276-278. · 1.81 Impact Factor
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ABSTRACT: Abnormalities of both motor and sensory nerve action potentials, similar to those found in demyelinating polyneuropathy, may occur in patients with amyotrophic lateral sclerosis (ALS). We analyzed the clinical features of unusual ALS patients with demyelinating polyneuropathy (DPN) to delineate the characteristics and outcomes of this rare condition. We reviewed three ALS patients with DPN who were confirmed to meet the electrophysiological nerve conduction criteria for DPN among 157 patients with ALS. At the initial neurological examination, one patient had both subjective sensory symptoms and abnormal results of sensory examinations, and one patient had sensory symptoms. Motor weakness of the limbs was present in all patients, and fasciculation was present in two patients. Anti-GalNAc-GD1a IgG antibodies were evident in one. Sural nerve biopsy showed a moderate, marginal reduction in myelin thickness, and teased fiber analysis revealed segmental demyelination and remyelination, but axonal degeneration was found in one patient. The mean interval from disease onset to respiratory failure or death in our three patients and seven previously documented ALS patients with DPN was 43.1 ± 18.7 months. Our findings suggest that survival in ALS with DPN is similar to that in classic ALS.
Amyotrophic Lateral Sclerosis 01/2012; 13(1):125-31. · 3.40 Impact Factor
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ABSTRACT: Recently, zonisamide (ZNS) has been approved as a new adjunctive therapy for motor complications of Parkinson's disease (PD). More recently, ZNS was reported to be effective for the management of impulse control behavior in PD, suggesting potential effects on non-motor PD symptoms. Dream enactment associated with aggressive, violent behavior can carry a serious risk of injury to patients, as well as to spouses or caretakers. This report describes a patient with PD who had vivid nightmares and dream-enacting behavior that resolved after treatment with ZNS. The present case raises the question whether ZNS might potentially be effective for the management of vivid nightmares or dream-enacting behavior.
Case Reports in Neurology 01/2012; 4(1):31-3.
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Journal of Neurology 11/2011; 259(6):1229-30. · 3.47 Impact Factor
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Hiroshi Kataoka,
Noriyuki Tanaka,
M Eng,
Keigo Saeki,
Takao Kiriyama,
Nobuyuki Eura,
Masanori Ikeda,
Tesseki Izumi,
Takanori Kitauti,
Yoshiko Furiya,
Kazuma Sugie,
Yoshito Ikada,
Satoshi Ueno
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ABSTRACT: It is difficult to predict the risk of falling, especially in patients with good motor ability, and the mechanisms underlying the relation between gait patterns and falling in Parkinson's disease (PD) remain unclear. We investigated factors related to falling, including walking speed and time, in patients with Hoehn-Yahr stage III PD.
We performed clinical assessments and evaluated balance in 30 patients with PD. Information on falling was obtained from questionnaires and personal interviews. Gait patterns were analyzed with the use of an originally designed, suddenly narrowed path.
Gait velocity was slower in fallers than in non-fallers (p = 0.047). Unified Parkinson's Disease Rating Scale part II (UPDRS part II) score, fear of falling, and gait velocity were significantly related to falling on analysis with a single logistic model. When a multiple logistic model was used, the UPDRS part II score was significantly related to falling (OR: 1.48, p = 0.037, 95% CI: 1.02-2.16).
Patients with Hoehn-Yahr stage III PD showed slow gait velocity attributed to fear of falling before arrival at a narrowed entrance or while walking on a narrowed path. The UPDRS part II score is significantly related to the risk of future falls.
European Neurology 11/2011; 66(5):298-304. · 1.81 Impact Factor
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Journal of neurology, neurosurgery, and psychiatry 09/2011; 83(4):469-70. · 4.87 Impact Factor
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ABSTRACT: Many long-term, follow-up studies have shown that steroids are effective in patients with myasthenia gravis (MG) who have undergone thymectomy. However, few long-term studies have documented the response of MG to steroids alone. We describe two patients who successfully resolved the symptoms of MG without myasthenic crisis or serious side effects by steroid treatment without thymectomy or other alternative therapies for more than 19 years. Our experiences raise an open question of the role of thymectomy or steroid treatment alone, especially in MG patients without thymoma.
Case Reports in Neurology 09/2011; 3(3):239-41.
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ABSTRACT: We describe an adult with Rasmussen's encephalitis associated with widespread decreased accumulation of tracer on 123-I iomazenil (IMZ) SPECT. The patient had a form of epilepsia partialis continua and neurological deterioration associated with hemiatrophy. Cranial MRI showed cerebral atrophy in the left frontal, parietal, and temporal lobes, without abnormal intensity. Delayed IMZ SPECT showed widespread decreased accumulation of tracer in the left temporal and frontal regions. An early scan showed mildly decreased accumulation in the left temporal and frontal regions. Three-dimensional stereotactic surface projection (3D-SSP) analysis showed that the decreased regions on delayed IMZ images were slightly larger as compared with the previous IMZ study performed. Some regions without decreased accumulation of tracer on Tc-ECD or early IMZ images showed decreased accumulation on delayed IMZ images. This case study suggests that benzodiazepine receptors may be reduced in the affected hemisphere of patients with Rasmussen's encephalitis.
Epileptic disorders: international epilepsy journal with videotape 06/2011; 13(2):145-9. · 1.50 Impact Factor
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Clinical neurology and neurosurgery 05/2011; 113(8):693-5. · 1.30 Impact Factor
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ABSTRACT: The middle cerebellar peduncle (MCP) is supplied mainly by the anterior inferior cerebellar artery and partly by the superior cerebellar artery. The bilateral MCP infarctions in previous patients were attributed to alternations of two vessels, such as the unilateral vertebral artery and basilar artery or both vertebral arteries.
We describe a case of bilateral acute infarction of the MCP and the stroke was apparently caused by occlusion of only one vertebral artery.
A 63-year-old man presented with vertigo and auditory distortion, accompanied by difficulty in speaking and walking. Neurological examinations revealed bilateral horizontal nystagmus, scanning speech, and ataxia of the trunk and all four limbs. Brain MRI showed high signal intensity in the both MCPs on diffusion-weighted and T2-weighted images. Enhanced computed tomographic angiography showed fusiform dilatation at the V4 level of the right vertebral artery; a false lumen was evident in the dilatation. Right vertebral angiography showed occlusion from the C1 level to the foramen magnum. Left vertebral angiography revealed a hypoplastic vertebral artery with a maximum diameter of 2.5 mm.
Alternations of the vertebral artery should be considered as a possible cause of MCP infarction, particularly when such anomalies are suggested.
Journal of neuroimaging: official journal of the American Society of Neuroimaging 04/2011; 21(2):e171-2. · 1.72 Impact Factor
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Takao Kiriyama, Hiroshi Kataoka,
Toshiaki Taoka,
Yasuyo Tonomura,
Mari Terashima,
Masami Morikawa,
Emi Tanizawa,
Makoto Kawahara,
Yoshiko Furiya,
Kazuma Sugie,
Kimihiko Kichikawa,
Satoshi Ueno
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ABSTRACT: Features of tumefactive demyelinating lesion (TDL) on magnetic resonance imaging (MRI) can facilitate the differential diagnosis of TDL and neoplastic lesions, but vary considerably among patients. The larger TDL grows, the more difficult it becomes to differentiate TDL from neoplastic lesions. The purpose of this study was to elucidate typical MRI features in 12 patients with large TDL (>30 mm in diameter).
We identified 12 patients with large TDL (six men, six women; age range 17-64 years, median age 27 years) and studied the clinical histories and the results of laboratory and various radiological studies in these patients. All cases of clinically definite multiple sclerosis were diagnosed in accordance with McDonald's revised criteria.
Common MRI features of large TDLs included variable degrees of mass effect (71%) and edema (100%), a T2 hypointense rim (79%), venular enhancement (57%), and peripheral restriction on diffusion-weighted images (50%). Ring enhancement (38%), open-ring enhancement (31%), or decreased N-acetylaspartate ratios on magnetic resonance spectroscopy (22%) were less frequently observed. Brain angiography demonstrated venous dilatations on and around the TDL.
The diagnosis of large TDL is challenging. Our findings suggest that multiple venous dilatations on and around TDLs on angiography can facilitate diagnosis.
Journal of neuroimaging: official journal of the American Society of Neuroimaging 04/2011; 21(2):e69-77. · 1.72 Impact Factor
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ABSTRACT: Previous studies have suggested embolic mechanisms for bihemispheric subcortical infarcts involving the anterior and posterior circulation. However, the mechanism of bihemispheric subcortical infarcts in middle cerebral artery (MCA) territories remains uncertain. We describe a patient with acute bihemispheric subcortical infarcts in restricted MCA territories suggesting an embolic mechanism.
A 62-year-old woman with a history of hypertension and hyperlipidemia suddenly presented with left hemiplegia. Diffusion-weighted and T(2)-weighted magnetic resolution imaging of the brain showed multiple subcortical high intensity in the MCA territories. There were no acute infarctions in the cerebrum, brain stem, or cerebellum, including cortical lesions. The patient had no carotid, internal carotid artery, or MCA disease.
Bihemispheric subcortical infarcts in the MCA territory are likely to have a proximal embolic source and such infarcts could be associated with multiple subcortical infarcts due to small vessel disease.
Clinical medicine insights. Case reports. 01/2011; 4:25-8.
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ABSTRACT: Central nervous system (CNS) demyelination has been suggested to be associated with infections caused by the Epstein-Barr virus (EBV) or herpes simplex virus (HSV)-1. CNS inflammatory demyelinating disease (IDD) rarely presents as a large lesion. We evaluated samples of serum and cerebrospinal fluid (CSF) by enzyme-linked immunosorbent assay to detect recent infection with these viruses and analyzed CSF and brain specimens by polymerase chain reaction (PCR) or immunohistochemical studies for evidence of these viruses in three patients with biopsy-proven CNS IDD. The results of PCR tests for EBV and HSV in CSF or brain specimens were negative. Elevated anti-EBV or -HSV antibody levels were not found in serum or CSF in any patient. Immunohistochemical studies showed that IDD lesions were negative for latent membrane protein (LMP)-1, Epstein-Barr nuclear antigen (EBNA)-2, and EBNA noncoding RNA (EBER)-1. These results suggest a negative association between CNS IDD and EBV or HSV.
Journal of NeuroVirology 11/2010; 16(6):466-71. · 2.31 Impact Factor
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ABSTRACT: To describe a patient with Parkinson disease in whom compulsive singing developed without other types of pathologic behavior after starting treatment with pergolide.
An 82-year-old woman with Parkinson disease was given pergolide (250 microg/d), without modifying the doses of other medications.
She started to hum the same melody and often sang songs repeatedly. Pergolide was discontinued, and the episodes of repetitive humming and singing were markedly decreased.
Our observations suggest that a dopamine agonist may contribute to compulsive singing in Parkinson disease.
Cognitive and behavioral neurology: official journal of the Society for Behavioral and Cognitive Neurology 06/2010; 23(2):140-1. · 1.09 Impact Factor
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Movement Disorders 04/2010; 25(6):786-7. · 4.51 Impact Factor
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ABSTRACT: Brainstem encephalitis is not a classic paraneoplastic syndrome and usually involves monophasic neurological deterioration and has negative magnetic resonance imaging (MRI) findings. We describe a patient with brainstem encephalitis who had elevated anti-Ri antibody levels and double-step neurological deterioration associated with different abnormal lesions on MRI. Immunosuppression with steroids and intravenous immune globulin combined with aggressive treatment of the tumor successfully led to the resolution of brainstem symptoms and MRI lesions. In patients with unusual signs and symptoms of paraneoplastic encephalitis mimicking multiple sclerosis, onconeural antibody studies are recommended.
Journal of Neuro-Oncology 02/2010; 100(1):141-3. · 3.21 Impact Factor
