[A 39 years old woman responding to modafinil with bilateral hypothalamic lesion associated with hyperthermia and hypersomnia: a case report].

A 39 years old woman was admitted to our hospital with a status epilepticus, with high fever of 41°C. Magnetic resonance Imaging (MRI) revealed high signal intensities of both sides of thalami and hypothalami in T2 weighted and fluid attenuated inversion recovery (FLAIR) images. A needle biopsy of the thalamic lesion was consistent with neuromyelitis optica spectrum disorder although her serum antibody to aquaporin-4 was negative. The level of orexin in celebrospinal fluid (CSF) was reduced. She presented hypersomnia, which didn't improve even after intravenous methylprednisolone 1 g daily for 3 days. Administration of oral modafinil extended her waking time. There is a number of reports about neuromyelitis optica (NMO) with hypothalamic lesions. We report this case as important suggestion of treatment of these cases.

Isolated thalamic tuberculoma presenting as ataxic hemiparesis.

Lacunar syndrome is a neurodeficit secondary to a deep cerebral lesion, usually because of microatheroma of small arteries. Ataxic hemiparesis (AH) is a lacunar syndrome with unilateral pyramidal weakness and ipsilateral ataxia. Thalamic tuberculoma, as a cause of AH, has not been previously described in the literature. We describe an elderly man who presented with left hemiparesis and ipsilateral ataxia. Clinical examination revealed upper motor neuron left facial paresis and left-sided hemiparesis. The patient had incoordination in left upper and lower limbs. Mantoux test was positive and erythrocyte sedimentation rate was elevated. MRI of brain showed a conglomerated hypointense lesion in the right thalamus with a peripheral hyperintensity on T1-weighted imaging and a hyperintense lesion in T2-weighted imaging with significant perilesional oedema, suggesting a tuberculoma. The patient was treated with antitubercular therapy and was symptomatically better at the 9 months follow-up.

Schizophrenia-like psychosis associated with right lacunar thalamic infarct.

Thalamic dysfunction has been associated with schizophrenia and other psychotic disorders. We describe an adult patient with a lacunar infarct in the posterior region of the right thalamus exhibiting a paranoid schizophrenia-like psychosis as the only clinical manifestation. Neuropsychological assessment showed alterations in visuospatial memory and executive functions at follow up. This case highlights the role of information processing by the thalamus in the development of delusions. We suggest that dysfunction of the right mediodorsal and pulvinar thalamic nuclei disrupts both thalamic sensory processing and thalamo-prefrontal circuits mediating belief evaluation, leading to delusional beliefs.

[Successful treatment of brain stem and thalamic abscesses with high-dose meropenem].

The incidence of brain abscess remains high, despite the development of novel antibiotics. Vancomycin or carbapenems, which are third-generation cephems, are recommended as standard therapy for bacterial meningitis or brain abscess. The effectiveness of the high-dose meropenem therapy on brain abscess has occasionally been reported. We experienced 2 consecutive cases of brain abscess in adults. The first patient was a 67-year-old man with diplopia, dizziness, and dysesthesia on the left upper and lower extremities. Images of T1-weighted magnetic resonance imaging (MRI) with contrast medium and diffusion-weighted MRI showed a ring enhancing cystic lesion and a high intensity lesion, respectively, in the right pons. The second patient was a 37-year-old man who complained of right hemiparesis. MRI revealed a ring-enhancing cystic mass in the left thalamus. On the basis of MRI findings, patients were diagnosed with brain abscess and were given high-dose meropenem (6g/day) continuously for 2 months. The abscess resolved completely after treatment with meropenem administered intravenously. Further, neurological deficits caused by abscess successfully improved. High-dose meropenem therapy should be considered as an effective treatment for brain abscess, even in the brain stem and basal ganglia, where it is quite difficult to achieve surgical access.

Compound heterozygosity of the protein S-gene as a cause of severe cerebral sinovenous thrombosis in a 7-year-old child.

The genotype-phenotype relationship of compound heterozygous protein S-deficiency in a 7-year-old girl with reduced protein S-levels and a severe cerebral sinovenous thrombosis is illustrated. In this patient we identified a novel deletion in the protein S-gene causing a compound heterozygous state and subsequently a symptomatic protein S-deficiency. In case of thrombosis analysis of protein S is recommended. Low levels of protein S should be further investigated by molecular diagnostics.

Isolated astasia in acute infarction of the supplementary-motor area.

Astasia, which is the inability to stand in the absence of motor weakness or marked sensory loss, is an uncommon clinical feature of stroke in the thalamic ventrolateral region. The authors describe a patient with a unilateral supplementary motor area (SMA) infarction presenting with contralateral astasia. On neurological examination, he would lean to the left side and would fall unless supported. He showed no muscle weakness, sensory deficits or cerebellar ataxia. Magnetic resolution imaging of the brain showed acute infarction only involving the right SMA. On the basis of the anatomy that the SMA is connected to the vestibulocerebellar system through the ventrolateral nucleus of the thalamus, the authors concluded that contralateral astasia probably resulted from disruption of this connection following infarction of the SMA.

Pediatric thalamic glioblastoma associated with Ollier disease (multiple enchondromatosis): a rare case of concurrence.

Ollier disease and Maffucci syndrome are rare syndromes in which there is deforming dysplasia of cartilage, primarily but not exclusively involving the metaphyses and diaphyses of long bones. In a minority of patients, dysplasia can lead to sarcomatous degeneration, producing chondrosarcomas. There also appears to be an association with other neoplasms. Little has been written about the association between Ollier disease and intracranial tumors, and these papers have largely consisted of case reports in adults. The authors present the case of a 6-year-old girl with left arm osseous changes consistent with Ollier disease and a biopsy-proven thalamic glioblastoma multiforme. They then examine the co-occurrence of brain tumors in conjunction with a dyschondroplasia syndrome in children and adolescents to assess the presentation, treatment offered, and disease course of similar cases. Eight other such cases were identified, 6 in patients with Ollier disease (ranging in age from 7 to 18 years), and 2 with Maffucci syndrome (both in late adolescence). Including our own patient, 7 of the 9 cases of comorbid dyschondroplasia and intracranial malignancy occurred in girls. Some patients presented soon after the acute onset of symptoms, and others had a more subtle, protracted course over as many as 2 years. Some tumors were deemed resectable and others not. In only 1 instance was follow-up beyond 1 year reported.

Anticoagulation therapy and imaging in neonates with a unilateral thalamic hemorrhage due to cerebral sinovenous thrombosis.

BACKGROUND AND PURPOSE: Cerebral sinovenous thrombosis is a rare disorder with a high risk of an adverse neurodevelopmental outcome. Until now, anticoagulation therapy has been restricted to neonates without an associated parenchymal hemorrhage. In this study, we describe sequential neuroimaging findings and use of anticoagulation therapy in newborn infants with a unilateral thalamic hemorrhage due to cerebral sinovenous thrombosis. METHODS: Ten neonates with a unilateral thalamic hemorrhage and cerebral sinovenous thrombosis were studied. Diagnosis was suspected using cranial ultrasound and confirmed with MRI/MR venography. Eight infants had a repeat MRI at 3 to 7 months. Neurodevelopmental outcome was assessed from 3 months until 5 years. RESULTS: One infant died. Seven infants were treated with low-molecular-weight heparin. No side affects were noted. MRI showed involvement of multiple sinuses, additional intraventricular hemorrhage, and white matter lesions in all infants. Recanalization was present on the repeat MRI at 3 months in all infants. Treatment was delayed in one infant and anticoagulation was started only after extension of the thalamic hemorrhage. He required a ventriculoperitoneal drain for posthemorrhagic ventricular dilatation and developed cerebral visual impairment and global delay. Two other infants showed global delay and one of them also developed postneonatal epilepsy. Mild asymmetry in tone was present in 4 children. CONCLUSIONS: Cerebral sinovenous thrombosis was found in 10 neonates with unilateral thalamic hemorrhage. Diagnosis was suspected on cranial ultrasound and confirmed with MRI/MR venography. Treatment with low-molecular-weight heparin in newborn infants with a thalamic hemorrhage due to cerebral sinovenous thrombosis appears to be safe and should be considered. Long-term follow-up will be needed to assess cognitive outcome.

Germinoma with synchronous involvement of midline and off-midline structures associated with progressive hemiparesis and hemiatrophy in a young adult.

INTRODUCTION: Cerebral germinomas, the most common and least malignant intracranial germ cell tumors, usually arise in the pineal or suprasellar region and have characteristic clinical and radiological features. Germinomas more rarely occur in the thalamus, basal ganglia, and internal capsule, causing sometimes cerebral hemiatrophy and hemiparesis. More rarely, other clinical features can be fever of unknown origin, visual disturbance, and neuropsychiatric symptoms. Cerebral hemiatrophy can precede the imaging depiction of the off-midline mass. CASE: The authors present the first case of cerebral germinoma with synchronous involvement of the midline and off-midline structures, with unusual clinical and radiological presentation. DISCUSSION: The literature is reviewed, and the pathogenesis, the clinical findings, the imaging, and the therapy are discussed.

Selective anterograde amnesia with thalamus and hippocampal lesions in neuro-Behcet's disease.

Anterograde amnesia and minimal retrograde amnesia with thalamic and hippocampal lesions in neuro-Behcet's disease is rare. A 50-year-old man presented with forgetfulness and severe memory disturbance after suffering multiple oral and genital aphthous ulcers with erythema nodosum. A neurological examination and a neuropsychological assessment revealed prominent anterograde memory impairment without focal neurological deficits. On brain MRI there were high signal intensity lesions involving right anterior thalamus, left posterior basal ganglia, and left hippocampus. This is a quite selective anterogrde memory deficit in a case of neuro-Behcet's disease caused by parenchymal lesions in the thalamus and hippocampus.

Clinical assessment of usefulness, effectiveness and safety of jackyakamcho-tang (shaoyaogancao-tang) on muscle spasm and pain: a case series.

This study is a retrospective single case series. Two hundred and thirty-seven patients were treated with jackyakamcho-tang (JKT) for relief of muscle spasm and pain; 81 of them were included in analysis. (The others were excluded because of insufficient medical records to confirm the diagnosis or assess the response.) There were 29 patients with nocturnal leg cramps, 28 with cervical spondylosis, 13 with thalamic pain and 11 with carpal tunnel syndrome, for which the effectiveness was assessed as 86.2%, 60.7%, 45.5% and 72.8%, respectively. Adverse effects (indigestion, diarrhea or edema) were seen in 11.1% of the total patients, but severe cases were only 3.7%. Taking the effectiveness and the safety into consideration, the usefulness was assessed as 86.2%, 57.1%, 53.9% and 72.8% for treating nocturnal leg cramps, cervical spondylosis, thalamic pain and carpal tunnel syndrome, respectively. In conclusion, we suggest that JKT is a useful herbal medicine with analgesic and anti-spasmodic effects.

Thalamic stroke secondary to straight sinus thrombosis in a nephrotic child.

A 7-year-old Chinese boy with steroid-resistant nephrotic syndrome developed thalamic stroke secondary to straight sinus thrombosis. He was hospitalized due to status epilepticus and coma. The child recovered after treatment by low-molecular-weight heparin (LMWH) and warfarin. This case highlights the importance of magnetic resonance imaging with venography in the early diagnosis of cerebral sinus thrombosis (CST) in nephrotic children and the effectiveness of anticoagulation therapy in improving the neurological outcome.

The natural history and treatment of acquired hemidystonia: report of 33 cases and review of the literature.

OBJECTIVE: To evaluate the natural history and response to treatment in hemidystonia. METHODS: 190 Cases of hemidystonia were identified; 33 patients in this series and 157 from the world literature. Data was collected on aetiology, age of onset, latency, lesion location, and response to treatment. RESULTS: The most common aetiologies of hemidystonia were stroke, trauma, and perinatal injury. Mean age of onset was 20 years in this series and 25.7 years in the literature. The average latency from insult to dystonia was 4.1 years in this series and 2.8 years in the literature, with the longest latencies occurring after perinatal injury. Basal ganglia lesions were identified in 48% of cases in this series and 60% of the cases in the literature, most commonly involving the putamen. Patients experienced benefit from medical therapy in only 26% of medication trials in this series and in only 35% of trials in the literature. In the patients reported here, the benzodiazepines clonazepam and diazepam were the most effective medications with 50% of trials resulting in at least some benefit. In the literature, anticholinergic drugs were most effective with 41% of trials resulting in benefit. Surgery was successful in five of six cases in this series and in 22 of 23 cases in the literature. However, in 12 cases, results were transient. CONCLUSIONS: The most common cause of hemidystonia is stroke, with the lesion most commonly involving the basal ganglia. Hemidystonia responds poorly to most medical therapies, but some patients may benefit from treatment with benzodiazepines or anticholinergic drugs. Surgical therapy may be successful but benefit is often transient.

Surgical treatment of thalamic hematomas via the contralateral transcallosal approach.

Acute management of deep-seated hematomas remains controversial. Since patients with these hematoma later tend to develop severe edema and necrosis around the lesion, when surgery is indicated it should be done as early as possible. The purpose of this study was to compare whether early surgical removal and conservative treatment of primary thalamic hematoma correlated with improved neurological outcome. Last year, 61 patients with primary thalamic hematomas were admitted to our institution. Of these, 21 underwent surgery via contralateral transcallosal approach during the ultraearly stage (within 6 hours) after the apoplectic attack, and 24 patients were treated conservatively. Another 16 patients were excluded from the study due to systemic disease, mild hematoma (<40 cc), and deep coma associated with absence of brain stem reflexes. Initial Glasgow coma scores (GCS) at admission were similar for operated and nonoperated patients (8.64 +/- 1.93 versus 9.50 +/- 2.10, P>0.05). In the operated group, two patients had good recoveries and returned to normal life (Glasgow Outcome Score, or GOS, I), four had moderate disability and needed partial care (GOS II), six had severe disability and needed nursing care (GOS III), and six had a vegetative state (GOS IV). However, in the nonoperated group, one patient had good recovery and returned to normal life (GOS I), two had moderate disability and needed partial home care (GOS II), three had severe disability and needed nursing care (GOS III), and six had a vegetative state (GOS IV). In this group, the 30-day mortality rate was 50%. Mortality was markedly lower in the operated group (14.3%) than the nonoperated group. and this difference was statistically significant (chi2=3.33, P<0.05). From this study, we believe that evacuation of primary thalamic hematoma via the contralateral transcallosal microsurgical approach may be useful for deciding on the indication and predicting the functional prognosis.

Isolated inferior rectus palsy as a result of paramedian thalamopeduncular infarction.

The authors present the cases of two patients with isolated inferior rectus muscle paresis presumed to be caused by paramedian thalamopeduncular infarction that involved supranuclear descending pathways, just before the inferior rectus subnucleus in one patient, and just before subnucleus or fascicular fibers in the other patient. Both patients had no other associated neurologic dysfunction. The lesions that cause isolated inferior rectus palsy in these patients are documented by magnetic resonance findings. Although vascular ischemic lesions as the cause of isolated inferior rectus palsy were reported previously, to the authors' knowledge, it has not been demonstrated radiologically.

Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy.

BACKGROUND: The outcome of a child with a primitive neuroectodermal tumors arising supratentorially (SPNET) is not well characterized and may differ from the outcome of a patient with a histologically similar cerebellar tumor (medulloblastoma [MB]). Recently, 5-year progression free survival rates as high as 80% have been reported for children with MB treated with craniospinal radiation (CRT) and chemotherapy including cisplatin, lomustine (CCNU), and vincristine (VCR). METHODS: The authors reviewed the outcome of 22 consecutive patients age 3 years and older (mean age, 10 years; range, 3-18 years) with SPNET who were treated at the study institutions between 1981 and 1996. Tumor location included was 13 pineal, 6 cortical, and 3 thalamic or suprasellar. Five patients had disease dissemination at diagnosis. All patients underwent surgery and staging, followed by CRT and chemotherapy with cisplatin, CCNU, and VCR. RESULTS: Of the 22 patients, 13 had developed disease progression and 10 had died at the time of last follow-up. Overall progression free survival (PFS) was 47% +/- 11% at 3 years and 37% +/- 11% at 5 years. There was a significant difference in PFS between patients with localized disease versus those with disseminated disease (P = 0.04). There was no statistical association between tumor location and survival. Although not significant (P = 0.21), there was a trend toward better survival of those patients with complete or near-complete resection compared with those with partial resection or biopsy. CONCLUSIONS: The results of the current study demonstrate that the outcome for children with SPNET treated with radiation and chemotherapy appears worse than for children with MB treated with identical therapy. This suggests that there may be biologic differences between supratentorial and infratentorial primitive neuroectodermal tumors, thus requiring refinements in treatment.