Clinical Features, Neuroimaging, and Levodopa-Responsiveness in Holmes' Tremor: A Video-Based Case-Series with a Review of the Literature.

Background: Holmes' tremor (HT) is a low-frequency tremor characterized by a combination of rest, posture, and action components. We are reporting the clinical features, neuroimaging findings, and levodopa responsiveness in 12 patients with HT. Cases: The majority of the patients were male (11/12). Dystonia was observed in 10 patients and the remaining two patients had head tremor, a "forme-fruste" of cervical dystonia. The underlying etiologies were vascular (n = 8), head trauma (n = 2), and tumor resection (n = 2). Neuroimaging showed isolated involvement of the midbrain in four, thalamus in two, and basal ganglia and cerebellum in one patient each. A combination of the lesion (thalamus and cerebellum = 2; cerebellopontine angle = 1, and cortical/subcortical = 1) was present in four patients. Levodopa responsiveness was seen in 75% of patients including one with levodopa-induced dyskinesia. Literature Review: Of 139 patients from 49 studies, levodopa was tried in 123 patients. Improvement with levodopa was seen in 71 patients (57.72%). No improvement with levodopa was observed in 33 patients (26.82%) and details regarding therapeutic response were unavailable in 19 patients (15.44%). Conclusions: Dystonia is an important clinical manifestation of HT. Levodopa responsiveness seen in the majority of the patients is consistent with the hypothesis that nigrostriatal pathway damage is crucial for the pathophysiology of HT.

Functional Neurological Disorders: Clinical Spectrum, Diagnosis, and Treatment.

BACKGROUND: Functional neurological disorders (FNDs) are common but often misdiagnosed. REVIEW SUMMARY: The incidence of FNDs is between 4 and 12 per 100,000, comparable to multiple sclerosis and amyotrophic lateral sclerosis, and it is the second most common diagnosis in neurology clinics. Some clues in the history are sudden onset, intermittent time course, variability of manifestation over time, childhood trauma, and history of other somatic symptoms. Anxiety and depression are common, but not necessarily more than in the general population. Although there are no tests currently capable of demonstrating whether symptoms are willfully produced, there may not be a clear categorical difference between voluntary and involuntary symptoms. The prognosis of an FND is linked to early diagnosis and symptom duration, but unfortunately, the majority of the patients are diagnosed after considerable delays. CONCLUSIONS: A positive diagnosis of FNDs can be made on the basis of history and neurological signs without reliance on psychological stressors. Past sensitizing events and neurobiological abnormalities contribute to the pathogenesis of FNDs. Physical rehabilitation and psychological interventions alone or in combination are helpful in the treatment.

The Spectrum of Repetitive Behaviors Associated With Subacute Sclerosing Panencephalitis.

BACKGROUND: Repetitive behaviors refer to a broad class of responses ranging from stereotypic body movements to impulsive/compulsive behaviors. They may be associated with neurological disorders. METHODS: This is a case series of six subacute sclerosing panencephalitis (SSPE) patients who presented with a wide spectrum of repetitive motor behaviors and vocalizations. RESULTS: Repetitive motor behaviors involved the upper limbs in all patients and lower limbs in 3 patients. The repetitive movements in the upper limbs were clapping, finger-clicking, hand rubbing, flailing, and dystonic posturing. In the lower limbs, the repetitive movements were rubbing with the heel, pelvic thrusting with flexion extension of the leg, and foot tapping. The spectrum of vocalizations included palilalia, whistling, grunting with spitting, and pathological crying. Repetitive behaviors were the presenting features in 2 patients. CONCLUSIONS: This case series expands the spectrum of repetitive behaviors seen in neurological disorders associated with brain infections. © 2020 International Parkinson and Movement Disorder Society.

Cranial Functional Movement Disorders: A Case Series with Literature Review.

Background: Cranial functional movement disorders (CFMDs) affect the face, eyes, jaw, tongue, and palate. Objectives: We aimed to examine our large series of functional movement disorders (FMDs) patients where the cranial muscles were involved to determine their phenomenology and other clinical features. Methods: This is a chart review of 26 patients who presented with CFMDs. Results: There were 16 (61.53%) females and 10 (38.46%) male patients. The mean ± [standard deviation (SD)] age at the presentation was 33.96 ± 16.94 (Range: 11-83) years. The duration of symptoms ranged from one day to 6 years (Mean ±SD: 402.03 ±534.97 days). According to the Fahn-Williams criteria, CFMDs were documented in 24 patients and clinically established in two patients. The facial [38.46% (10/26)] involvement was the most common in our CFMDs patients. Oromandibular [19.23% (5/26)], ocular [15.38% (4/26)], lingual [15.38% (4/26)], speech [15.38% (4/26)] and palatal [(3.85; 1/26)] involvement was also seen. 10 (38.46%) patients also had associated FMD in the extracranial regions. Precipitating factors were present in 84.61% (22/26) of the patients and associated illnesses were present in 42.30% (11/26) of the patients. At 3 months follow-up, 9 (34.61%) patients had improved, 13 (50%) had partial improvement and 4 (15.38%) had no improvement. Conclusions: There was a slight female preponderance in our patients. CFMDs are more likely to involve facial muscles. Associated medical conditions like neuropsychiatric disturbances and headaches are frequently present in CFMDs patients. Early clinical diagnosis will avoid unnecessary investigations and allow the patient to seek the right treatment.