Objective: Ataxic gait can be remarkably improved by a simple method called the “handkerchief guide” involving the patient and caregiver holding opposite ends of a handkerchief and walking together. Our objective was to assess the effect of the handkerchief guide on gait in patients with cerebellar ataxia. Methods: Gait analysis was carried out on seven patients with degenerative cerebellar disease (DCD), seven patients with unilateral cerebellar vascular disease (CVD), and seven healthy control (HC) subjects. All subjects performed two walking tasks: free walking (FW) and handkerchief-guided walking (HGW) on a 10 m pathway. In the HGW condition, each subject walked with the caregiver while maintaining slight tension on the handkerchief. The HCs and patients with DCD held the handkerchief with their right hand, while the patients with unilateral limb ataxia due to CVD grasped it with their affected and unaffected hands in different trials. We measured 10 gait parameters. Results: The HGW attenuated body-sway, lengthened step, and increased gait velocity in patients with cerebellar ataxia. In DCD, the HGW significantly improved seven parameters. In CVD, HGW with the affected hand improved five parameters, and HGW with the unaffected hand improved seven parameters. Conclusions: The HGW stabilized upright posture in patients with cerebellar ataxia during level-ground walking, probably by enabling subconscious postural adjustments to minimize changes in the arm and hand position relative to trunk, and in arm configuration. This led to improvement of gait performance. The handkerchief guide may be useful for walk training in patients with cerebellar ataxia. Abbreviations: COM, center of mass; COG, center of gravity (projection of the COM onto the ground plane); COP, center of pressure; CVD, cerebellar vascular disease; DCD, degenerative cerebellar disease; FW, free walking; HAT, head, arms, and trunk segment; HC, healthy control; HGW, handkerchief-guided walking.
Although amyotrophic lateral sclerosis with dementia (ALS-D) has been characterized by symptoms of fronto-temporal dysfunction, we report two patients with ALS-D who showed constructive disturbance and low-level perfusion in the parietal areas. The first was a 69-year-old woman (Case 1) who had been diagnosed with the bulbar type of ALS. She showed fronto-temporal dementia as well as low scores and disturbance on block construction and copying; however, she showed a better score on the imitation of finger postures. The second was a 73-year-old woman (Case 2) who had been diagnosed with the leg onset type of ALS. She showed mild impairment of the frontal function as well as mild disturbance on block construction and copying, but no problem on the imitation of finger postures. Case 1 showed more severe symptoms of dementia and constructive disturbance than Case 2, whereas Case 2 showed lower levels of cerebral perfusion over more extensive areas than Case 1. Cases 1 and 2 were compatible with definite ALS according to the El Escorial Criteria, and they showed constructive disturbance with characteristics reported previously, such as both left and right hemisphere damage and constructive disturbance similar to those seen in Alzheimer’s disease. In addition, they showed poorer scores on performing tasks requiring the use of objects (block construction and copying) rather than using their body (imitation of finger postures).
Lemierre syndrome is a clinical syndrome that presents with internal jugular thrombophlebitis, septicemia and systemic abscess formations. In general, the condition is preceded by oropharyngeal infections. We report a case of a 73-year-old man with Lemierre syndrome, clivus osteomyelitis and a steroid-responsive mass in the cavernous sinus-suprasellar region. He complained of fever, occipital pain, diplopia and right ptosis. Administration of oral steroids ameliorated the ophthalmic symptoms for a period before he was admitted to our hospital. After admission, the severity of his headache advanced, and his ophthalmic symptoms progressed bilaterally. Brain magnetic resonance imaging showed contrast enhancement of the clivus and revealed a mass lesion contrast-enhancement effect in the cavernous sinus-suprasellar region. Fusobacterium nucleatum was detected by blood culture, and computed tomography revealed multiple bacterial emboli in both lung fields and thrombosis of the left internal jugular vein; thus, he was diagnosed with Lemierre syndrome. After venous administration of antibiotics, his fever and headache markedly improved, but the ophthalmic symptoms did not. We prescribed an oral steroid because the cavernous sinus-suprasellar lesion was probably an inflammatory granuloma caused by a para-infectious mechanism rather than by infection. After the series of treatments, his ophthalmic symptoms improved, and the cavernous sinus-suprasellar region mass lesion decreased. He was eventually discharged in a fully ambulatory state and had no ophthalmic difficulties. We thought that the osteomyelitis of clivus was caused by Lemierre syndrome and its inflammatory processes formed the granuloma in the cavernous sinus-suprasellar region. This was a case of Lemierre syndrome with a rare combination of clivus osteomyelitis and a steroid-responsive tumour in the cavernous sinus-suprasellar region that was successfully treated.
A 56-year-old man suffered from diffuse large B-cell lymphoma (DLBCL) originated from the stomach. He received R-CHOP therapy, and had a complete remission. However, at age 57, he experienced left shoulder pain and weakness of left arm, and his muscle weakness and sensory disturbance subacutely progressed to other limbs. Cervical and lumbosacral MRI showed enhanced extended lesions of cervical, thoracic, lumbar, and sacral nerve roots and cauda equina. Cerebrospinal fluid analysis revealed a sustained low glucose level. Nerve conduction study showed abnormalities of measurement parameters of F-waves in all limbs. A diagnosis of recurrent DLBCL presenting neurolymphomatosis could be established by repeated cytology of cerebrospinal fluid. He received high dose methotrexate therapy, but his symptoms were worsened to tetraplegia. It should be noticed that DLBCL can involve spinal nerve roots extensively.
We report a rare case of autonomic neuropathy associated with cytomegalovirus (CMV) infection. The patient, a 53-year-old male, was admitted to our hospital because of prolonged fever, headache and neck stiffness followed by urinary retention. Cerebrospinal fluid examination revealed pleocytosis (219/mm3, predominantly lymphocytes) with a markedly increased protein level (217 mg/dl) and serum IgM anti-CMV antibody was detected. While his meningitic symptoms gradually improved after intravenous administration of ganciclovir, he complained of numbness in the extremities and difficulty in walking. Neurologically, marked orthostatic hypotension, glove and stocking type of paresthesia, severe muscle weakness in extremities, and neurogenic atonic bladder were noted. Nerve conduction studies showed normal except for F-waves, which were absent in the left tibial nerve. A sural nerve specimen appeared normal in both myelinated and unmyelinated fibers. He was given immunological therapies such as corticosteroid and intravenous high dose immunoglobulin therapies. After corticosteroid therapies, not only sensory and motor symptoms but also autonomic symptoms remarkably improved. Of the anti-ganglioside antibodies, IgM anti-GM1 antibody and IgM anti-GM2 antibody were detected. Although some cases with Guillain-Barré syndrome preceded by CMV infection have been reported, few cases with autonomic neuropathy have been described in association with successful corticosteroid therapies.
A 77-year-old woman presented with a 6-month history of slowly progressive cerebellar ataxia. T2-weighted MRI showed high signal intensity in the left upper dorsal pons and bilateral middle cerebellar peduncles. JC virus (JCV) DNA was detected in cerebrospinal fluid (CSF). The patient had no HIV infection, collagen disease, or a history of immunosuppressive treatment, but she was found to have CD4+ lymphocytopenia. We made a diagnosis of cerebellar brainstem form of progressive multifocal leukoencephalopathy (PML) presenting as cerebellar ataxia, which was presumably associated with idiopathic CD4+ lymphocytopenia. Following the treatment with mefloquine, the patient slightly improved clinically and JCV-DNA became negative in CSF.
We report the case of an 84-year-old man with sensory ataxic polyneuropathy and IgGλ monoclonal gammopathy of undetermined significance (MGUS), which was successfully treated with intravenous immunoglobulin (IVIG) therapy. He had developed progressive ataxic gait over the span of 2 years before he was admitted to our hospital. On admission, he was unable to walk without assistance because of severe sensory ataxia. He performed poorly on the finger-nose-finger and heel-knee tests, and his vibration and position sense in the feet was remarkably diminished. However, motor involvement was not remarkable. Serum immunoelectrophoresis revealed IgGλ monoclonal gammopathy, and MGUS was diagnosed. Nerve conduction studies revealed sensory-dominant axonal polyneuropathy. The patient was successfully treated with IVIG (400 mg/kg/day, for 5 days). He regained his capacity to walk independently after treatment, but his nerve conduction results remained unchanged. This sensory ataxia might be partially due to underlying cervical spondylotic myelopathy. To our knowledge, this is the first report in our country of the successful use of IVIG therapy to treat a patient with IgGλ monoclonal gammopathy and related sensory ataxic axonal polyneuropathy.
A patient started anticoagulation for secondary prevention of stroke, achieving a prothrombin time-international normalized ratio within the optimal therapeutic range. The patient subsequently complained of pain in the left thigh on hospital day 25, and ultrasonography showed a large intramuscular hematoma in the left hamstring. The intramuscular hematoma gradually reduced without surgical intervention. Ultrasound examination plays an important role in precisely diagnosing intramuscular hematoma and monitoring changes in hematoma size.
A 73-year-old man presented with continuous hemichoreic movement of right arm and leg and with dyskinesia in his tongue. Magnetic resonance image (MRI) showed no ischemic lesion within the basal ganglia, but magnetic resonance angiography (MRA) and carotid duplex ultrasonography showed the left internal carotid occlusion and 80% stenosis in the right common carotid artery. Tc-99m-ECD-SPECT showed hypoperfusion of the frontal lobe, temporal lobe, parietal lobe, basal ganglia and thalamus. A trial of haloperidol had no effect; therefore, the right carotid artery stenting was performed. Hypoperfusion in the left internal carotid artery area was improved by cross flow from the right side, and his hemichorea gradually improved. This result supports the notion that hypoperfusion-related hemichorea may occur, even in the absence of cerebral ischemia.