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Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a specific subtype of the stiff-person syndrome, which is rare and difficult to diagnose clinically. A case of PERM in a 66-year-old female with a fluctuating progressive course was reported in this article. She had increased facial muscle tone, pruritus and sensory hypersensitivity mainly in the head and neck, medullary involvement syndrome and bilateral lower limb rigidity as the main clinical manifestations, and a previous history of pulmonary malignancy, thymoma, typeⅠ diabetes and Hashimoto′s thyroiditis. The patient′s serum and cerebrospinal fluid were positive for anti-glutamic acid decarboxylase antibody. The electromyogram showed a large number of motor unit potentials in the trunk and proximal extremities in the quiet state, which were significantly enhanced during spastic episodes, consistent with the electromyographic manifestations of stiff-person syndrome. The final diagnosis was PERM, and immunotherapy including gamma globulin and hormone responded well. PERM is a rare neurological autoimmune disease with atypical early symptoms, which can be easily misdiagnosed, and it requires attention to avoid delaying the diagnosis.
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Presynaptic dopaminergic PET imaging is a useful method for the diagnosis of parkinsonism. Based on the expert consensus on operation and clinical application of dopamine transporter brain PET imaging technology published in 2020, this paper further recommends the relevant elements of result interpretation of presynaptic dopaminergic PET imaging.
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Objective To observe the effects of early rehabilitation on the motor function of post-stroke hemiplegic patients treated with intravenous thrombolysis.Methods Thirty-six post-stroke hemiplegic patients after intravenous thrombolysis with rtPA were recruited in this study.They were randomly divided into the early rehabilitation group (with the course less than three days) and the control group (with the course between three and seven days),18 cases in each group.Both group received a two-week rehabilitation programme.They were assessed using the Fugl-Meyer assessment (FMA) of up extremity,FMA of lower extremity and ADL score (modified Barthel index,MBI) before and after treatment,as well as at the 3rd month after treatment (during the following-up).Results After treatment and at the following-up,FMA scores of up extremity,FMA scores of lower extremity and MBI scores had improved significantly compared with before treatment in both groups.Furthermore,after treatment,in the early rehabilitation group the average FMA score of lower extremity and MBI score reached (23.33 ± 4.37) and (56.11 ± 22.27) respectively,significantly higher than those of the control group [(17.06 ± 4.70) and (40.00 ± 15.81) respectively].Conclusion Early rehabilitation contributes to short-term recovery of lower extremity motor function and ability in the activities of daily living in post-stroke hemiplegic patients after intravenous thrombolysis.
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Objective To evaluate the value of cranial MRI on diagnosing nonalcoholic Wernickes encephalopathy (WE). Methods The clinical characters, cranial MRI features, and outcomes materials in six cases of nonalcoholic Wernickes encephalopathy were analyzed.Results Cranial MR and Flair imaging of the patients exhibited areas of increased T 2W and flair signals symmetrically surrounding the aqueduct and third ventricle and within the medial thalamus. One patient who became persistent vegetative state coexistenced increased T 2W and flair signal of the cortex. According to the follow-up results, the alterations of four patients in T 2W and Flair signals showed to resolve being consistent with the clinical recovery. One patient with persistent vegetative state had no change within two years of the follow-up.Conclusions Cranial MRI is of great value in diagnosing nonalcoholic Wernickes encephalopathy and reflects appropriately the pathological evolution of this disease.