Search

Immune-mediated cerebellar ataxias (IMCAs) have diverse etiologies. Patients with IMCAs develop cerebellar symptoms, characterized mainly by gait ataxia, showing an acute or subacute clinical course. We present a novel concept of latent autoimmune cerebellar ataxia (LACA), analogous to latent autoimmune diabetes in adults (LADA). LADA is a slowly progressive form of autoimmune diabetes where patients are often initially diagnosed with type 2 diabetes. The sole biomarker (serum anti-GAD antibody) is not always present or can fluctuate. However, the disease progresses to pancreatic beta-cell failure and insulin dependency within about 5 years. Due to the unclear autoimmune profile, clinicians often struggle to reach an early diagnosis during the period when insulin production is not severely compromised. LACA is also characterized by a slowly progressive course, lack of obvious autoimmune background, and difficulties in reaching a diagnosis in the absence of clear markers for IMCAs. The authors discuss two aspects of LACA: (1) the not manifestly evident autoimmunity and (2) the prodromal stage of IMCA's characterized by a period of partial neuronal dysfunction where non-specific symptoms may occur. In order to achieve an early intervention and prevent cell death in the cerebellum, identification of the time-window before irreversible neuronal loss is critical. LACA occurs during this time-window when possible preservation of neural plasticity exists. Efforts should be devoted to the early identification of biological, neurophysiological, neuropsychological, morphological (brain morphometry), and multimodal biomarkers allowing early diagnosis and therapeutic intervention and to avoid irreversible neuronal loss., (© 2023. The Author(s).)

Background: Oculogyric tics are identified in a substantial proportion of patients with Tourette syndrome (TS) and related tic-disorders. Nevertheless, studies assessing its frequency and clinical correlations are lacking., Materials and Methods: We reviewed video-recordings and clinical history of 201 patients with TS and chronic motor/phonic tic disorder, diagnosed according to DSM-5., Results: Oculogyric tics presented in 22.4% of our patients. Transient upward gaze was the most common phenomenology in patients with simple oculogyric tics; whereas eye-closure followed by upward and lateral deviations was the most commonly observed phenomenon in patients with combined oculogyric tics. Oculogyric tics have a median duration of 799 ms (range 299-34,500 ms). Patients with oculogyric tics were younger (P = 0.023) and had a higher frequency of cranial tics (P = 0.037) compared to those without oculogyric tics. No differences in tic severity, frequency of attention-deficit/hyperactivity disorder, obsessive-compulsive disorder or use of dopamine receptor antagonists were observed in patients with and without oculogyric tics., Conclusions: Oculogyric tics are a common phenomenology in chronic tic disorders. They are more common in younger patients with TS and are markers of other cranial tics, but not of TS severity or comorbid neuropsychiatric syndromes., (© 2022. The Author(s) under exclusive licence to Belgian Neurological Society.)

Tremor and parkinsonism are recognized side effects of valproate; however, the relationship between rest tremor and other signs of parkinsonism has not been addressed in patients taking valproate. We studied a cohort of 125 consecutive patients treated with valproate due to epilepsy or migraine, evaluated with the Fahn-Tolosa-Marin Tremor Rating Scale (FTM-TRS). A total of 14 (11.2%) patients had rest tremor (bilateral n = 10, unilateral n = 4). Patients with rest tremor had significant higher scores in the FTM-TRS (P < 0.001), but only one was diagnosed with parkinsonism. Patients may have valproate-induced parkinsonism or exacerbated motor features of Parkinson's disease by valproate. The frequency of parkinsonism was 1.6% in this cohort and of 3% in the pooled data of 717 patients from previous reports. Rest tremor is observed in 11.2% of patients treated with valproate and is related to the burden of valproate-induced tremor, rather than the presence of parkinsonism.

OnabotulinumtoxinA has recently proved beneficial for the treatment of chronic migraine; even though, headaches are not infrequently reported by patients receiving botulinum toxins for different conditions. Here the author discusses the potential mechanisms on how the immunological response following the application of botulinum toxins may trigger headaches and/or migraine.

We aimed to define the frequency and risk factors associated with flu-like symptoms (FLS) and other systemic symptoms following treatment with botulinum toxins (BoNT) and correlate them with the immunological response as determined by blood cytokines. The study involved prospective clinical and serological evaluation for cytokine analysis in patients receiving BoNT for movement disorders. We interviewed 218 patients about FLS following BoNT injections and prospectively studied 117 patients (females 67.5 %; mean age 59.74 ± 12.39 years) treated with BoNT in a total of 143 treatment cycles. While no patient reported any FLS at baseline, the symptom complex was subsequently reported in 19 patients (16.3 %) and in 20 (14 %) treatment cycles, with at least one systemic symptom reported in 49 (42 %) patients in 59 (41.3 %) treatment cycles. FLS and at least one symptom were reported more frequently by women (P = 0.006 and P = 0.049, respectively) and by younger patients: 55.6 versus 61.7 years (P = 0.022). Although the symptoms were usually considered mild, they were rated as moderate to serious after 18 (12 %) cycles. The following interleukins showed increased levels at 7-10 days after the BoNT injections: IL-1β, IL-8, GROα, eotaxin, MCP-1 and 2, RANTES, TARC, and inducible protein 10 (IP10), but only the latter showed significantly increased levels in patients with FLS: 69 versus 3 pg/ml (P = 0.007). FLS and other systemic symptoms occur after about 14 % of treatment visits in patients receiving BoNT. IP10 levels correlate with the presence and severity of FLS.