Gabriela B. Raina

Gastrointestinal manifestations in Parkinson’s disease: prevalence and occurrence before motor symptoms

To assess the prevalence of gastrointestinal symptoms (GIS) in Parkinson’s disease (PD) compared to control subjects and their timing of appearance in relationship to the onset of motor symptoms. There is a rostrocaudal gradient of alpha-synuclein (α-SYN) neuropathology in the enteric nervous system at early stages of PD with higher burden in the upper than the lower gut. However, only constipation has been recognized as a premotor gastrointestinal manifestation of PD. 129 PD patients and 120 controls underwent a structured questionnaire to assess the presence of GIS and, in PD patients, the time of their appearance respect to the onset of motor manifestations. GIS significantly more prevalent in PD patients were dry mouth, drooling, dysphagia, constipation and defecatory dysfunction. Constipation and defecatory dysfunction preceded motor manifestations. Whereas gastroparesis symptoms preceded motor manifestations, their prevalence was not significantly different from controls. Despite evidence of a higher α-SYN burden in the upper gut, only constipation and defecatory dysfunction were prominent premotor GIS of PD.

Pallidal surgery for the treatment of primary generalized dystonia: Long-term follow-up

OBJECTIVE To describe the results and long-term follow-up after functional surgery of the internal segment of the globus pallidus (GPi) in 10 patients with primary generalized dystonia. PATIENTS AND METHODS Nine of the 10 patients were positive for the DYT1 gene mutation. Bilateral deep brain stimulation (DBS) of the GPi was performed in three cases, bilateral pallidotomy in two, and combined surgery (unilateral GPi lesion with contralateral stimulation) in the remaining five. All patients were evaluated with the Burke-Fahn-Marsden dystonia scale (BFMDS) before, immediately after surgery, at 3 weeks, 3 and 6 months and then yearly. Follow up time ranged from 15 to 105 months (mean: 66.1 months) with six patients having more than 6 years follow up. RESULTS All patients improved after surgery. All patients with unilateral or bilateral DBS experienced an immediate improvement before starting stimulation. The magnitude of this initial micro lesion effect did not predict the magnitude of the long-term benefit of DBS. The mean decrease in the in the BFMDS was 34%, 55%, and 65% in the movement scale; and 32%, 48%, and 49% in the disability scale for patients with bilateral pallidal DBS, combined unilateral DBS and contralateral pallidotomy, and bilateral pallidotomy, respectively. Worsening of dystonia after a plateau of sustained benefit was observed in three patients. Two patients required multiple pallidal surgeries. Adverse events included: permanent anarthria (1), misplacement of the electrode requiring further surgery (2), scalp infection (1), and hardware related problems (3). CONCLUSIONS This long-term follow up study confirms the beneficial effect of pallidal DBS or pallidotomy in primary generalized dystonia. In addition, our results extent previous observations by showing that, in these patients, (1) the microlesion effect of DBS is not predictive of long-term benefit; (2) combined DBS with contralateral pallidotomy appears to be more effective than bilateral pallidal DBS; and (3) dystonia can reappear after an initial good response during long term follow up.

Beneficial effects of botulinum toxin type a for patients with painful tic convulsif.

Botulinum toxin is a well-known therapy for patients with diverse movement disorders. Its application has been extended to other disorders. Here, we document the case of a 70-year-old man with hemifacial spasm associated to trigeminal neuralgia secondary to an ectatic basilar artery. He was treated with botulinum toxin type A, 2.5 mouse units over five sites at the orbicularis oculi and one over the buccinator muscle. After botulinum toxin injections, relief was gained not only from twitching but also from pain. When the effects of the toxin vanished, spasms and pain recurred. Further infiltrations were given every 12 weeks following the same response pattern. This observation further validates the increasing role of botulinum toxin in pain management.

Cluster-like headache associated to a foreign body in the maxillary sinus

Cluster headache is known as one of the most painful cephalalgias to be experience by humans. They are most commonly primary, for which there are well-established diagnostic criteria; but from 3 to 5% of cluster headache cases are secondary to diverse lesions. Although its pathogenesis is uncertain, the following hypotheses have been advanced: 1) a central origin, located in the anterior hypothalamus (suprachiasmatic nucleus), as well as in the posterior hypothalamus1,2⇓; 2) a peripheral origin, with activation of the pericarotid neural plexus in the cavernous sinus3; and 3) the coexistence of a “central trigger,” located in the hypothalamus with peripheral onset of pain, and autonomic symptoms, explained by activation of the trigemino-autonomic reflex.1,2,4⇓⇓ We present a patient with symptoms consistent with cluster headache, according to the 1988 criteria of the International Headache Society,5 associated to a foreign body in the ipsilateral maxillary sinus. A 34-year-old woman sought advice for an 8-year history of right, piercing, intense orbital pain lasting from 30 to 60 …

Micro Lesion Effect of the Globus Pallidus Internus and Outcome with Deep Brain Stimulation in Patients with Parkinson Disease and Dystonia

To determine whether the immediate response to electrode implantation (micro lesion effect, MLE) in the internal segment of the globus pallidus (GPi) predicts symptom improvement with deep brain stimulation (DBS) at 6 months in patients with Parkinsons disease (PD) or generalized dystonia. Electrode implantation in the subthalamic nucleus (STN) prior to electrical stimulation has been reported to predict a beneficial effect of DBS in patients with PD, but whether this is also the case for the GPi in either PD or dystonia patients has not been established. We studied 20 patients (11 with PD and 9 with dystonia) who underwent electrode implantation in the GPi. Effects were assessed using standardized scales after 24 hours, weekly for 3 weeks prior to starting DBS, and after 6 months of DBS. 10 of 11 PD and 8 of 9 dystonia cases who benefited from electrode implantation also showed improvement in all motor and disability scores after 6 months of DBS of the GPi. One dystonia patient who did not show MLE benefited from DBS. The presence of MLE after electrode implantation in the GPi may help predict motor benefit from DBS in PD and generalized dystonia patients.

Use of complementary and alternative therapies in outpatients with Parkinson's disease in Argentina†

We interviewed 300 patients (54.7% male; mean age was 65.8 ± 9.5) attending the Movement Disorders Clinic at the Buenos Aires University Hospital to determine the prevalence of CATs use and their association with demographic, social, or disease‐specific characteristics among patients with Parkinsons disease (PD) in Buenos Aires, Argentina. We found that 25.7% of the PD patients interviewed (77/300) stated they had used CATs to improve their PD symptoms whereas 38.0% (114/300) had used some CATs without any relation to PD, at least once in life. At the moment of the interview, CATs prevalence use was 50.6% in the former group and 25.0% in the latter. The use of CATs was much more frequent among women and more common in the 50‐ to 69‐year age group. Friends and neighbors of the patients had most frequently recommended these therapies. No major association was observed between CATs use and the duration of the disease, side of initial involvement, PD phenotype, or the Hoehn and Yahr staging. Acupuncture, homeopathy, yoga, and therapeutic massage were the most widely used therapies. After the initiation of conventional treatment the use of massage, yoga, and acupuncture in patients using CATs to improve PD significantly increased. Neurologists should be aware and inquire about the use of CATs to rule out potentially harmful effects.

Hyposialorrhea as an early manifestation of Parkinson disease

We sought to determine whether hyposialorrhea is an early manifestation of Parkinson disease (PD). We measured basal and citric acid stimulated secretion of whole saliva in 20 patients with early stage (Hoehn-Yahr I-II) PD who had motor symptoms for less than 1 year and were on no medication and 11 age matched controls. Compared to controls, PD patients had significant reduction of both basal (0.0964+/-0.08 vs 0.293+/-0.112 ml/min, p<0.001) and reflex (0.263+/-0.213 vs 0.537+/-0.313 ml/min, p<0.001) salivary secretion. Our findings confirm that hyposialorrhea is an early autonomic manifestation of PD.

Parkinsonism as a manifestation of multiple sclerosis

We describe a 48‐year‐old patient, with a diagnosis of relapsing–remitting multiple sclerosis, who presented to our service with a parkinsonian syndrome that markedly improved after corticosteroid treatment. To the best of our knowledge, only 12 cases of parkinsonism have been reported from 1970 to the present, of which only 8 seemed secondary to MS, i.e., those presenting conclusive imaging evidence or unequivocal response to corticosteroids.

Low doses of topiramate are effective in essential tremor: a report of three cases.

We here report on 3 patients with essential tremor, otherwise unresponsive to pharmacological treatment, who greatly benefited from low doses of topiramate (50 mg/d). No side effects were observed and improvement was sustained during a mean of 7 months (range 3-12 months) follow up. Our results suggest that topiramate titration should be performed gradually, so as not to neglect cases responsive to low doses.

Holmes tremor: Clinical description, lesion localization, and treatment in a series of 29 cases.

Objective: To describe the clinical features, etiology, findings from neuroimaging, and treatment results in a series of 29 patients with Holmes tremor (HT). Methods: A retrospective study was performed based on review of medical records and videos of patients with HT diagnosis. Results: A total of 16 women and 13 men were included. The mean age at the moment of CNS insult was 33.9 ± 20.1 years (range 8–76 years). The most common causes were vascular (48.3%), ischemic, or hemorrhagic. Traumatic brain injury only represented 17.24%; other causes represented 34.5%. The median latency from lesion to tremor onset was 2 months (range 7 days–228 months). The most common symptoms/signs associated with HT were hemiparesis (62%), ataxia (51.7%), hypoesthesia (27.58%), dystonia (24.1%), cranial nerve involvement (24.1%), and dysarthria (24.1%). Other symptoms/signs were vertical gaze disorders (6.8%), bradykinesia/rigidity (6.8%), myoclonus (3.4%), and seizures (3.4%). Most of the patients had lesions involving more than one area. MRI showed lesions in thalamus or midbrain or cerebellum in 82.7% of the patients. Levodopa treatment was effective in 13 out of 24 treated patients (54.16%) and in 3 patients unilateral thalamotomy provided excellent results. Conclusions: The most common causes of HT in our series were vascular lesions. The most common lesion topography was mesencephalic, thalamic, or both. Treatment with levodopa and thalamic stereotactic lesional surgery seems to be effective.