Deep Brain Stimulation for the Treatment of Dejerine-Roussy Syndrome.

BACKGROUND/AIMS: Patients who suffer from Dejerine-Roussy syndrome commonly experience severe poststroke hemibody pain which has historically been attributed to thalamic lesions. Despite pharmacological treatment, a significant proportion of the population is resistant to traditional therapy. Deep brain stimulation is often appropriate for the treatment of resistant populations. In this review we aim to summarize the targets that are used to treat Dejerine-Roussy syndrome and provide insight into their clinical efficacy. METHODS: In reviewing the literature, we defined stimulation success as achievement of a minimum of 50% pain relief. RESULTS: Contemporary targets for deep brain stimulation are the ventral posterior medial/ventral posterior lateral thalamic nuclei, periaqueductal/periventricular gray matter, the ventral striatum/anterior limb of the internal capsule, left centromedian thalamic nuclei, the nucleus ventrocaudalis parvocellularis internis, and the posterior limb of the internal capsule. CONCLUSIONS: Due to technological advancements in deep brain stimulation, its therapeutic effects must be reevaluated. Despite a lack of controlled evidence, deep brain stimulation has been effectively used as a therapeutic in clinical pain management. Further clinical investigation is needed to definitively evaluate the therapeutic efficacy of deep brain stimulation in treating the drug-resistant patient population.

Alcohol is a risk factor not for thalamic but for putaminal hemorrhage: the Akita Stroke Registry.

BACKGROUND: Although the risk factors of cerebral hemorrhage were established long ago, there is little agreement as to the risk factors for the site of cerebral hemorrhage. METHODS: We obtained mass health screening data collected between 1990 and 2000 regarding 151,796 subjects from the Akita Prefectural Federation of Agricultural Cooperative for Health and Welfare. A first-ever cerebral hemorrhage occurring <3 years after the screening examination was defined as an event. Stroke events were determined from the Akita stroke registry between 1990 and 2003. Clinical risk factors for stroke, such as age, blood pressure, severe obesity (body mass index >30 kg/m(2)), low serum total cholesterol, hepatic disorder, renal disorder, and drinking habits were then assessed. RESULTS: Cerebral hemorrhage developed in 344 cases in the study population. The distribution of subtypes (putaminal hemorrhage [PH], thalamic hemorrhage [TH], and subcortical hemorrhage [SH]) were 122 cases (35.5%), 110 cases (32.0%), and 44 cases (12.8%), respectively. We evaluated the risk factors by multiple logistic regression analysis among these 3 groups. Age was a significant risk factor among these 3 groups, but blood pressure was not a risk factor in SH. Low serum cholesterol and drinking habits were significant risk factors only in PH. Hepatic disorder was a strong risk factor in PH and a weak risk factor in TH. Interestingly, a drinking habit was a significant risk factor only in PH. CONCLUSIONS: Drinking habits had been a risk factor for cerebral hemorrhage, but it was a risk factor not for PH and not for TH or SH.

Diffuse basal ganglia or thalamus hyperechogenicity in preterm infants.

OBJECTIVE: To determine the incidence and factors associated with diffuse basal ganglia or thalamus hyperechogenicity (BGTH) in preterm infants. STUDY DESIGN: (1) Review of serial neurosonograms among neonates with gestational age (GA) <34 weeks born at Weiler Hospital during a 21-month period; (2) Color Doppler flow imaging; (3) Case-control study using GA group-matched controls; and (4) Blind reading of CT scans or MRIs in patients with BGTH. RESULTS: Among 289 infants, 24 (8.3%) had diffuse BGTH. Color Doppler flow imaging was normal in nine patients. The incidence of diffuse BGTH was inversely related to GA (P<0.01). Logistic regression (n=96) showed that diffuse BGTH was significantly associated with requirement of high-frequency oscillation (HFO) (P=0.031), severe intraventricular hemorrhage (IVH) (P=0.004), hypotension requiring vasopressors (P=0.040), hypoglycemia (P=0.031) and male gender (P=0.014). Most patients with diffuse BGTH had normal basal ganglia and thalamus on CT/MRI, one had a hemorrhage, and one had an ischemic infarction. CONCLUSIONS: In our series, diffuse BGTH occurred in 8.3%, and was associated with factors similar to those previously reported. In contrast, several series have reported almost exclusively linear or punctuate hyperechoic foci, corresponding to hyperechogenicity of the lenticulostriate vessels. Our data provide further evidence to suggest that diffuse BGTH and hyperechogenicity of the lenticulostriate vessels are two different entities. Additional studies are required to determine the long-term significance of diffuse BGTH.

Bilateral thalamic glioma: review of eight cases with personality change and mental deterioration.

PURPOSE: To describe the clinical, radiographic, and neuropathologic features of bilateral thalamic glioma. METHODS: We searched our hospital records (1963 to present) to identify patients diagnosed as having the disease. RESULTS: Our search revealed eight patients, ranging in age from 8-63 years, with bithalamic tumor diagnosed by angiography, CT, and/or MR. All patients displayed personality changes and/or mental deterioration, including memory loss, inattention, confusion, hallucination, hyperphagia, or slow mentation. Unilateral motor weakness was also noted in six cases. The tumor always involved the medial aspect of the left and right thalami, but was often more extensive. The pathology was determined to be grades I-IV astrocytoma, confirmed by stereotactic biopsy or autopsy in six. Mild to moderate hydrocephaly occurred in some cases and was considered to be a contributing factor to mental deterioration. No correlation was found between age and type of tumor. CONCLUSIONS: Bilateral glioma of the dorsomedial and intralaminar nuclei of the thalamus can be a primary cause of dementia that has not been well-recognized in the past. CT and particularly MR should be considered for patients presenting with personality change or dementia, because of the possible presence of this unusual but devastating disease.

Current differential diagnoses and treatment options of vascular occlusions presenting as bilateral thalamic infarcts: a review of the literature.

bilateral thalamic infarctions are rare and usually caused by vascular occlusions. When symptomatic, it is important to make a distinction between different vascular etiologies in order to provide an effective and timely therapeutic response. Clinical presentations may not adequately differentiate between the vascular etiologies alone. It is therefore important to use imaging technologies to distinguish appropriately the origin of the infarct so that proper treatment can be administered. Advanced imaging techniques, such as CT angiography and MR angiography, have proved useful for distinguishing between arterial and venous causes of bithalamic infarctions. Bilateral thalamic venous infarctions can be treated with anticoagulation medication and with thrombolysis in more severe cases. Bilateral thalamic arterial infarctions may be treated with thrombolysis.

Lenticulostriate vasculopathy in very preterm infants.

OBJECTIVE: To assess for lenticulostriate vasculopathy (LSV) on cranial ultrasound (cUS) scans of very preterm infants: incidence and aetiology, evolution during neonatal period, association with clinical parameters, and MRI equivalent. DESIGN: Prospective study. SETTING: Tertiary neonatal referral centre. PATIENTS: Very preterm infants (<32 weeks) underwent sequential cUS throughout the neonatal period and MRI around term age. cUS were evaluated for LSV and other changes, and MRI for changes in signal and myelination in deep grey matter. LSV was divided into early-onset (7 postnatal days). Perinatal clinical parameters were collected for all infants and compared between groups. RESULTS: In 22/111 (20%) infants LSV was detected: early-onset in 5 and late-onset in 17. LSV mostly presented some weeks after birth and persisted for several months. There were no associations between LSV and other changes on cUS or deep grey matter changes on MRI. Infants with late-onset LSV were younger and smaller at birth than infants with early-onset LSV. Postmenstrual age at first detection was comparable for both LSV groups. There were no associations between LSV and perinatal clinical parameters, but infants with LSV had less episodes of hypotension than infants without LSV. CONCLUSIONS: LSV is a frequent finding on cUS in very preterm infants, but does not show on MRI. The postmenstrual age, rather than gestational and postnatal age, seems important in LSV development. LSV is not associated with clinical parameters. When encountered in otherwise healthy preterm infants, LSV is probably a benign temporary phenomenon.

The germinomas arising from the basal ganglia and thalamus.

OBJECTIVE: To introduce the features of germinomas arising from the basal ganglia (BG) and thalamus. METHOD: Retrospective analysis was done with the clinical cases of germinomas in BG and thalamus from 1996 to 2000. The data included the symptoms, signs, neuroimaging findings, treatment, and outcomes. RESULT: Fourteen cases were included, only one female was included. The main symptoms are disorder of numbness and weakness in limbs. Neuroimaging showed no or mild peritumor high signal in T2 weighted imaging of magnetic resonance, accompanied with cyst, calcification or bleeding. Total gross resection was obtained in nine cases, subtotal resection in four. Follow-up data were available in 11 cases with average of 56 months. Eight cases underwent only postoperative radiotherapy, one underwent only chemotherapy, and two underwent both. One case died of complication 6 months after chemotherapy, the rest lived good life. CONCLUSION: Germinoma in BG and thalamus predominate in a boy. The neuroimaging features are very informative for diagnosis. Surgical resection should not be the first choice although it is has lesser complications. The long-term outcome is favorable.

CNS germ cell tumor (CNSGCT) of childhood: presentation and delayed diagnosis.

OBJECTIVE: To describe the relationship between symptomatology and time to diagnosis of an institutional series of patients with CNS germ cell tumor (CNSGCT) over a 16-year period. METHODS: Thirty consecutive patients newly diagnosed with CNSGCT (mean age 10.9 years; range 6 to 17 years; 70% boys) were evaluated at our institution between 1990 and 2006. RESULTS: Duration of symptoms prior to diagnosis ranged from 5 days to 3 years (mean 8.4 months). Tumor location included pineal (14), suprasellar (8), pineal/suprasellar (3), pineal/thalamic (4), and basal ganglionic/thalamic (3). Five patients had disseminated disease at the time of diagnosis. Features including headache, nausea, vomiting, and visual changes led to earlier diagnosis. Symptoms including movement disorders, enuresis, anorexia, and psychiatric complaints delayed diagnosis in 9 of 30 patients, diagnosed 7 months to 3 years (mean 22.3 months) from symptom onset. In 7 of 9 patients with delayed diagnosis, enuresis was present. Seventeen of 30 patients had signs of endocrine dysfunction at presentation that included diabetes insipidus (4), hypothyroidism (8), and growth hormone deficiency (4). Ophthalmologic findings of decreased visual acuity, visual field deficits, or ocular abnormalities were present in 13 patients. Duration of symptoms did not correlate with tumor subtype or event-free survival. In three patients with basal ganglionic/temporal lobe, thalamic, or pineal/suprasellar signal abnormalities on MRI, neuroradiographic diagnosis was difficult. CONCLUSIONS: Diagnosis of CNS germ cell tumor is often delayed, and presentation may include movement disorders or mimic psychiatric disease. MRI interpretation can be challenging and may require serum/CSF markers and biopsy for diagnosis.

Evaluation and treatment of central pain syndromes.

Central pain syndromes represent a form of neuropathic pain that is associated with lesions of the brain or the spinal cord after a stroke or other traumatic injury. Although spinal cord injury (SCI) pain and central post-stroke pain (CPSP) are both classified as central pain syndromes, they may have differing etiologies. The pathophysiology of SCI pain and CPSP has yet to be completely elucidated, but both spinal and supraspinal pathways may be involved. Pain resulting from an injury to the CNS may be vague or difficult to classify or characterize, and patients may describe painful sensations that are poorly localized or that change over time. Pharmacologic interventions that have demonstrated efficacy in central pain syndromes include iv lidocaine and opioids as well as the tricyclic antidepressant amitriptyline and the AEDs gabapentin and lamotrigine. Nonpharmacologic interventions have also demonstrated benefit in some patients who are refractory to pharmacologic treatments. Additional studies are needed to further evaluate the efficacy and safety of both pharmacologic and nonpharmacologic treatments for central pain syndromes.

Athetoid cerebral palsy with cysts in the putamen after hypoxic-ischaemic encephalopathy.

Three cases of athetoid cerebral palsy after hypoxic-ischaemic encephalopathy (HIE) are reported. All three neonates had haemorrhagic lesions in the basal ganglia and thalami on magnetic resonance imaging (MRI). Prior cranial ultrasound had detected the lesions in only two cases. In all three children athetoid movements began within the first year of life. Follow up MRI scans showed bilateral symmetrical cystic lesions in the posterior putamen. Although haemorrhagic lesions within the basal ganglia are a common MRI finding in neonates with HIE, few of these babies develop athetoid cerebral palsy. We believe this to be the first report of discrete cystic lesions found in the basal ganglia of children with athetoid cerebral palsy.

Los síndromes de lesión talámica

En la unidad de Neurología del Centro Hospitalario San Juan de Dios de Bogotá, durante cuatro años (1986 a 1989), se estudiaron en forma consecutiva 25 pacientes con lesiones talámaticas no fatales. Se registraron los hallazgos neurológicos, neurosicológicos y neurooftalmológicos y los diagnósticos se confirmaron por tomografía computarizada (TC). Fueron 14 mujeres y 11 varones con una edad promedio de 52.5 y un rango de 25 a 84 años. La lesión talámica fue de origen vascular en 24 casos, ocho por infarto isquémico, cuatro por infarto hemorrágico y 12 con hematomas parenquimatosos. Diecisiete pacientes tenían hipertensión arterial sistémica y el único factor de riesgo en otros dos era el consumo de cocaína base (basuco). Ocho infartos se presentaron en el tálamo derecho, 12 en el izquierdo y cinco pacientes tuvieron lesiones bilaterales, uno de ellos con un glioma complobado por biopsia. En 5 pacientes con lesiónes bilateral se observó el síndrome del "Tope" de la arteria basilar, por compromiso del pedículo retromamilar; en todos ellos encontramos alteraciones sensitivomotoras, cerebelosas, oculomotoras bilaterales y demencia. Solamente un paciente presentó el clasicó síndrome de hiperpatía (Dejerine-Roussy). En los restantes se observaron asociaciones de síndromes sensitivomotores, cerebelosos, neurooftalmológicos, neuropsicológicos, y del comportamiento motor que remedan con frecuencia los hallazgos clínicos de la alteración cortical frontal, temporal o parietal.