Head tremor in dentatorubral-pallidoluysian atrophy.

Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal-dominant neurodegenerative disorder characterized by variable combination of clinical manifestations including ataxia, myoclonus, seizures, dementia, and choreic movements. Head tremor has been rarely reported. We report a 66-year-old-woman with genetically determined DRPLA who presented with head tremor. A "no-no" type head tremor was the initial and the most prominent symptom, and mild cerebellar signs and choreic movements were also observed later. Neither hand tremor nor dystonia was noted. The patient did not show dementia, myoclonus, or seizures. Surface electromyogram (EMG) revealed 3.5-4 Hz rhythmic EMG bursts in both sternocleidomastoid muscles. DNA analysis disclosed expanded trinucleotide repeats (n = 54) in the DRPLA gene. We suggest that isolated head tremor can be a clinical manifestation of DRPLA.

Sweet's syndrome associated with encephalitis.

The involvement of the central nervous system (CNS) in Sweet's syndrome (acute febrile neutrophilic dermatosis) is rare. We report a 47-year-old woman who presented with acute encephalitis and was subsequently diagnosed as having Sweet's syndrome. She developed altered consciousness following fever and erythematous skin plaques in the extremities. Cerebrospinal fluid (CSF) examination disclosed neutrophilic pleocytosis without decreased glucose level. Brain magnetic resonance imaging (MRI) showed abnormal signal intensity lesions in the basal ganglia and the hippocampus. Skin biopsy revealed a dense dermal infiltration of neutrophils, which is compatible with Sweet's syndrome. Treatment with acyclovir and antibiotics failed, but the subsequent corticosteroid therapy was effective. Awareness of neurological complication in Sweet's syndrome may avoid unnecessary empiric therapy for meningoencephalitis and will lead to a successful treatment with corticosteroids.

[A quadriplegic patient with chronic inflammatory demyelinating polyneuropathy (CIDP) who responded well to corticosteroids and intravenous immunoglobulin therapy].

We report an elderly patient with chronic inflammatory demyelinating polyneuropathy (CIDP) presenting with complete quadriplegia who responded well to the treatment. A 74-year-old woman was transferred to our hospital from a hospital for the elderly patients. The patient had a history of progressive limb weakness over three years, and has been quadriplegic for the last six months. The patient was unable to move her extremities, but neither respiratory nor bulbar dysfunction was observed. Deep tendon reflexes were absent. Glove and stocking type sensory disturbance was noted. Nerve conduction studies showed slowed motor and sensory conduction velocities with diminished compound muscle action potentials (CMAP). Abnormal temporal dispersion and conduction blocks were also demonstrated. Cerebrospinal fluid examination revealed an elevated protein level of 78 mg/dl with normal cell counts. The patient was diagnosed as having CIDP. She was treated with methylprednisolone pulse therapy and oral prednisolone, followed by high dose intravenous immunoglobulin treatment. Significant recovery occurred during the first week, and she became able to walk four months later. Motor nerve conduction velocity (MCV) and CMAP were also improved. It is suggested that CIDP must be considered in patients with quadriplegia of unknown etiology: such patients may be seen in hospitals for elderly patients.

[Transient improvement in motor function and hemineglect by vestibular stimulation in a patient with right middle cerebral artery embolism].

A 62-year-old Japanese man presented left hemiparesis and left visuospatial hemineglect following a right hemispheric stroke. His CTs and MRIs of the brain revealed a large embolic infarction of the middle cerebral artery territory. A month after the cerebrovascular event, his weakness of the left lower limb almost recovered fully. However, his upper limb motor function was still disabled; in particular, his ability of finger flexion in the left hand was almost lost. Then, vestibular stimulation using either a cold caloric stimulation to the left ear or a warm caloric stimulation to the right ear was performed, and the effect on the hemineglect symptoms were assessed by a line bisection task. After vestibular stimulation, not only his hemineglect symptoms but also his motor functions of left upper limb transiently improved; he became able to make a fist. The improvement of his hemineglect symptoms was obtained by vestibular stimulation using either a cold or a warm caloric stimulation. However, the effect on the motor function was obtained only by the cold caloric stimulation applied to the left ear. Based on the effect of the vestibular stimulation, we postulates that the impairment of the motor function in the present patient is not only a paresis caused by the pyramidal tract lesion but also symptoms related to the hemineglect syndrome.

[The dissecting aortic aneurysm associated with polymyalgia rheumatica: a case report].

We report a patient with a dissecting aortic aneurysm associated with polymyalgia rheumatica (PMR). The patient is a 55-year-old Japanese man without a history of hypertension, diabetes mellitus and syphilis. He was admitted to an emergency hospital because of severe back pain, and was diagnosed as having a dissecting aneurysm of the descending aorta. After the admission, he began to notice severe muscle pain in his bilateral shoulder. Although his back pain gradually improved, his muscle pain progressively worsened, and his lower extremities were also involved. Then, he was introduced to our hospital. On neurological examination, he was alert and oriented. His cranial nerves were all intact. There was no muscle weakness nor sensory disturbance. Laboratory studies revealed that his erhythrocyte sedimentation rate was extremely high without elevation of the serum level of creatine phoshpokinase, rheumatoid factors and c-reactive protein. He was diagnosed as having PMR, and oral administration of prednisolone++ was started. Within several days, his muscle pain dramatically disappeared. As is known, there is a close relationship between PMR and temporal arteritis of giant cell arteritis. In general, PMR is a benign disease and responds well to steroid therapy, and prevalence of the giant cell arteritis is low in Japanese people. However, it should be kept in mind that the dissecting aneurysm is a relevant, severe complication of PMR because arteritis can be latently present in PMR.

Effective high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation in a patient with the aggressive form of cytophagic histiocytic panniculitis.

A 20-year-old Japanese man developed generalized, subcutaneous, painless nodules, fever, abnormal liver function, serosal effusions, hepatosplenomegaly, lymphadenopathy and anemia. Skin biopsies revealed lobular panniculitis with a morphologically benign histiocytic infiltration and prominent phagocytosis. Atypical T lymphocytes were also present in the skin and liver. The diagnosis given was aggressive cytophagic histiocytic panniculitis (CHP) or aggressive subcutaneous panniculitic T cell lymphoma (SPTCL). He received cyclophosphamide, doxorubicin, and vincristine on day 1, prednisolone on days 1-5, and etoposide on days 1, 3 and 5 (CHOP-E), with the support of granulocyte colony-stimulating factor. This regimen was repeated every 2 weeks and complete clinical remission (CCR) was attained after three cycles of CHOP-E. As the clinical course of aggressive CHP is recurrent and often fatal, he was given high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (APBSCT), after five cycles of CHOP-E. He has remained in CCR for 12 months after APBSCT. High-dose chemotherapy followed by APBSCT is considered to be one of the most beneficial therapies for patients with aggressive CHP and aggressive phase SPTCL.