Effect of acupuncture treatment on nonketotic hyperglycemic hemichorea-hemiballismus: a case report.

Nonketotic hyperglycemic hemichorea-hemiballismus is a rare syndrome in the clinic, and treatment is often delayed. Hypoglycemic therapy is the most widely used and effective treatment, but some patients experience a slower improvement. Other symptomatic treatment medicines have some degree of side effects. Acupuncture treatment is beneficial for hemichorea-hemiballismus. A male patient, aged 59 years, first visited our hospital outpatient department due to motor agitation with involuntary movements of the right limb. He had a history of type 2 diabetes mellitus and poor blood glucose control. His serum glucose was 26.5 mmol/L (normal: 4.4-6.1 mmol/L), and magnetic resonance imaging demonstrated an irregular area of high signal intensity in T1-weighted imaging, low signal intensity on T2-weighted imaging, and high signal intensity in the left corpus striatum in T2-FLAIR imaging. Hospitalization was recommended for the patient. After ruling out other possibilities, he was eventually diagnosed with nonketotic hyperglycemic hemichorea-hemiballismus. Intensive glycemic control was immediately started with subcutaneous injection and acupuncture treatment at "governor vessel 13 acupoints", and the involuntary movements completely disappeared on the ninth day of hospitalization. The pathophysiology of nonketotic hyperglycemic hemichorea-hemiballismus is unclear. Different patient histories lead to different brain tissue conditions, and relapses and uncontrolled blood glucose add difficulties to treatment. According to Traditional Chinese Medicine theory, insufficient kidney essence leads to brain dystrophy and causes the symptoms of hemichorea-hemiballismus. Research evidence has shown that acupuncture at "governor vessel 13 acupoints" has a beneficial treatment effect on nonketotic hyperglycemic hemichorea-hemiballismus.

Dystonia, chorea, hemiballismus and other dyskinesias.

Hyperkinesias are heterogeneous involuntary movements that significantly differ in terms of clinical and semeiological manifestations, including rhythm, regularity, speed, duration, and other factors that determine their appearance or suppression. Hyperkinesias are due to complex, variable, and largely undefined pathophysiological mechanisms that may involve different brain areas. In this chapter, we specifically focus on dystonia, chorea and hemiballismus, and other dyskinesias, specifically, levodopa-induced, tardive, and cranial dyskinesia. We address the role of neurophysiological studies aimed at explaining the pathophysiology of these conditions. We mainly refer to human studies using surface and invasive in-depth recordings, as well as spinal, brainstem, and transcortical reflexology and non-invasive brain stimulation techniques. We discuss the extent to which the neurophysiological abnormalities observed in hyperkinesias may be explained by pathophysiological models. We highlight the most relevant issues that deserve future research efforts. The potential role of neurophysiological assessment in the clinical context of hyperkinesia is also discussed.

Crossed Choreoathetosis Caused by Unilateral Thalamic Hemorrhage.

In general, involuntary movements after stroke are due to a disturbance in the unilateral cortico-basal ganglia loop and appear contralateral to stroke lesions. Crossed involuntary movements after unilateral stroke are very rare. We observed a case of crossed involuntary movements in the left upper limb and right lower limb after a right thalamic hemorrhage expanded to the right subthalamic nucleus. We considered a possible three-step theory as the basis of crossed choreoathetosis. This case informs our better understanding of the cortico-basal ganglia loop and involuntary movements after stroke.

Hypoxaemia and interstitial lung disease in an infant with hypothyroidism and hypotonia.

A 7-month-old-term male infant presented with cough, tachypnoea, hypoxaemia and post-tussive emesis. Clinical history was significant for respiratory failure and pulmonary hypertension in the neonatal period requiring assisted ventilation, congenital hypothyroidism, mild hypotonia, recurrent respiratory infections, hypoxaemia requiring supplemental oxygen and nasogastric tube feeds. Physical examination showed tachypnoea, coarse bilateral breath sounds and mild hypotonia. Chest radiograph revealed multifocal pulmonary opacities with coarse interstitial markings and right upper lobe atelectasis. Following antibiotic therapy for suspected aspiration pneumonia, chest CT scan was performed and showed multiple areas of pulmonary consolidation and scattered areas of bilateral ground-glass opacities. Genetic studies showed a large deletion of chromosome 14q13.1-14q21.1, encompassing the NK2 homeobox 1 (NKX2-1) gene consistent with a diagnosis of brain-thyroid-lung (BTL) syndrome. Our case highlights the importance of genetic studies to diagnose BTL syndrome in infants with hypothyroidism, hypotonia and lung disease.

Combined mutations of NKX2-1 and surfactant protein C genes for refractory low oxyhemoglobin saturation and interstitial pneumonia: A case report.

RATIONALE: Mutations of the NKX2-1 gene are associated with brain-lung-thyroid syndrome, which is characterized by benign hereditary chorea, hypothyroidism, and pulmonary disease with variable presentation. Surfactant protein C (SFTPC) gene mutations result in chronic interstitial lung disease in adults or severe neonatal respiratory distress syndrome. PATIENT CONCERNS: Recurrent hypoxemia was observed shortly after birth in a baby at a gestational age of 40 weeks and birth weight of 3150 g. The need for respiratory support gradually increased. He had hypothyroidism and experienced feeding difficulties and irritability. DIAGNOSIS: Genetic examination of the peripheral blood revealed combined mutations of the NKX2-1 and SFTPC genes. INTERVENTIONS: The patient was administered respiratory support, antibiotics, low-dose dexamethasone, supplementary thyroxine, venous nutrition, and other supportive measures. OUTCOMES: The patient's guardian stopped treatment 3 months after commencement of treatment, due to the seriousness of his condition and the patient died. LESSONS: Combined mutations of NKX2-1 and SFTPC genes are very rare. Thus, idiopathic interstitial pneumonia with hypothyroidism and neurological disorders require special attention.

Corea / No disponible

Corea significa danza y se utiliza para designar movimientos rápidos, involuntarios, irregulares, no predecibles, de duración breve, que cambian de una zona corporal a otra sin una secuencia definida, suelen tener una localización distal y baja amplitud y asociarse a hipotonía de los miembros y a reflejos rotulianos pendulares.Suele relacionarse con lesiones o alteraciones del caudado y el putamen.En nuestro caso, los movimientos rápidos e irregulares sedebían a lesiones vasculares, una de las causas de corea. El paciente empezó con la sintomatología hacía quince días. Las exploraciones complementarias realizadas fueron análisis completos con hemograma, bioquímica, coagulación, hormonas tiroideas, serología a lúes, borrelia, brucella, proteína 14-3-3, vitamina E, anticuerpos antineuronales, electrocardiograma,ecocardiograma, ecografía de abdomen, eco-dopplerde troncos supraaórticos, electromiograma/electroneurograma/blink-reflex, resonancia magnética cerebral, metoxisobutil isonitrilo-tomografía computarizada por emisión de fotón único.Tratamiento realizado: médico (clonazepam y toxina botulínica)

Chorea means dance and it is used to designate short duration- fast, involuntary, irregular and unpredictable movements that change from one area of the body to another without a defined sequence. They generally have a distal localization and low amplitude and are usually associated to hypotonia of the limbs and pendular patellar reflexes.This is generally realted to injuries or alterations of the caudate and putamen.In our case, the fast and irregular movements were causedby vascular injuries, one of the causes of Chorea. The patient began with the symptoms 15 days ago. The complementary analyses performed were complete blood count, biochemistry, coagulation, thyroid hormones, serologies for syphilis, borrelia, brucella serology, protein 14-3-3, vitamin and antineuronal antibodies, electrocardiogram, echocardiogram, abdomen ultrasonography, TSA echo-Doppler, EMGENG/blink-reflex, cerebral MRI, MIBI-SPECT.The treatment performed was medical (clonazepam andbotulinic toxin)

Benign hereditary chorea: clinical, neuroimaging, and genetic findings.

Benign hereditary chorea is an autosomal dominant disease with an early onset of symptoms. In some families, symptoms tend to decrease in adulthood, suggesting that the disorder results from a developmental disturbance in the brain. Individuals with benign hereditary chorea, a nonprogressive disease, have normal or slightly below normal intelligence. The locus for benign hereditary chorea is on chromosome 14. Benign hereditary chorea is a result of mutations in the thyroid transcription factor 1 gene. Previous neuroimaging and pathological investigations of the brain showed no notable abnormalities in patients with this condition. In this study, 5 patients from 1 family with typical clinical features of benign hereditary chorea are presented. Clinical severity varied considerably in the family. Brain magnetic resonance imaging results were normal. Brain single photon emission computed tomography in 3 children, performed 1 hour after intravenous injection of 0.35 mCi/kg of body weight of technetium 99m ethyl cysteinate dimer, showed markedly decreased uptake in the right striatum and the right thalamus in 1 child. The oldest child had mildly reduced uptake in the right putamen and the left thalamus. Brain single photon emission computed tomographic findings in the youngest child were normal. Contrary to other reports of radionuclide brain imaging, notable brain single photon emission computed tomography changes were detected in 2 of 5 patients. Brain single photon emission computed tomography findings did not seem to correlate with the clinical status of the children.

Molecular diagnosis of pseudohypoparathyroidism type Ib in a family with presumed paroxysmal dyskinesia.

We describe 2 sisters diagnosed initially with paroxysmal kinesigenic choreoathetosis, a condition characterized by brief episodes of spasms precipitated by sudden movement. However, subsequent testing showed hypocalcemia, hyperphosphatemia, and elevated parathyroid hormone levels consistent with pseudohypoparathyroidism type Ib. This diagnosis was confirmed by genetic testing, which identified a 3-kilobase deletion on chromosome 20q13.3. Our report describes the neurologic presentation, metabolic derangement, and underlying genetic mutation in a family. It also reinforces the importance of metabolic testing in the evaluation of pediatric patients with movement disorders.

Diagnostic role of 99mTc hexamethyl-propyleneamine oxime brain single photon emission computed tomography in Sydenham's chorea.

BACKGROUND: The objective of this study was to determine whether technetium-99m hexamethyl-propyleneamine oxime (HMPAO) brain single photon emission computed tomography (SPECT) imaging is capable of detecting perfusional abnormalities in Sydenham's chorea (SC) patients and contributing to diagnosis of SC. METHODS: In this study, 17 SC patients were evaluated. HMPAO SPECT was performed on all patients at the acute phase and six of them at the recovery phase. Magnetic resonance imaging (MRI) of the brain was performed to 13 patients. SPECT images of basal ganglia and thalamus were evaluated both visually and by measuring the radioactivity uptakes. Ten subjects constituted the control group. RESULTS: HMPAO SPECT was visually evaluated as indicating hyperperfusion in the basal ganglia and thalamus in 16 patients, and evaluated as normal in one patient at the acute phase. The radioactivity uptakes of basal ganglia and thalamus of the patients at the acute phase were found statistically higher than those of the patients at the recovery phase, and also higher than those of the control group (P < 0.05). A significant difference between the radioactivity uptake of the patients at the recovery phase and those of the control group was not found (P > 0.05). Only one patient did not show any abnormality in both acute and recovery phases. MRI study did not show any abnormality in the basal ganglia and thalamus. CONCLUSION: It is suggested that brain SPECT can contribute to the diagnosis of SC as an objective tool. Resolving of the hyperperfusion at the recovery phase provides further support for the diagnosis of SC.

[A case of symptomatic paroxysmal kinesigenic dsykinesia with primary central nervous system lymphoma]. / Paroxysmale Dyskinesien. Kasuistik eines Patienten mit symptomatischer paroxysmaler kinesigener Dyskinesie bei primärem ZNS-Lymphom.

Paroxysmal dyskinesias are rare movement disorders. In one fourth of cases, a symptomatic cause can be found, e.g., brain ischemia or inflammatory diseases. We report a unique case of secondary paroxysmal kinesigenic dyskinesia (PKD). The 37-year-old patient suffered from a primary CNS lymphoma invading the right thalamus. Consecutively, he showed dystonic-athetoid movements in the left half of the body. The involuntary movements had a duration of approximately 20 s and occurred more than 100 times per day. Carbamazepine treatment showed excellent results. This is the first reported case of a symptomatic paroxysmal dyskinesia induced by a primary CNS lymphoma.

[Generalized choreic involuntary movements in the course of stroke: case report]. / Ruchy mimowolne pod postacia plasawicy uogólnionej w przebiegu udaru niedokrwiennego mózgu--opis przypadku.

Involuntary movements are rare ischemic stroke complication. Hemichorea, hemibalism and dystonia cases were described, especially during haemorrhage into basal ganglia and thalamus. The case of 73 years old woman is presented who was admitted to our Clinic cause of sudden choreatic involuntary movements occurrence within axial muscles and limbs. Computed Tomography showed ischemic focus in basal ganglia (head of caudate nucleus and lentiformis nucleus) and anterior branch of internal capsule. Cerebral angio--CT confirmed partial occlusion both medial arteries and atherosclerotical changes within the rest of cerebral arteries.

Acute mania and hemichorea.

A 61-year-old man suddenly became euphoric and talkative. Later the same day, he developed hemichoreic movements of the left limbs. The patient fulfilled the DSM-IV criteria for a manic episode by abnormally and persistently elevated mood, decreased need for sleep, high distractibility, pressured speech, increased goal-directed activity, and hypersexuality. The mood changes persisted for several weeks. Magnetic resonance imaging of the brain showed a right thalamic infarction. The co-occurrence of hemichorea and mania caused by focal thalamic lesion is very rare. It may be explained by dysfunction in basal ganglia thalamocortical circuitry.

Increased ictal perfusion of the thalamus in paroxysmal kinesigenic dyskinesia.

The ictal and interictal cerebral blood flow (CBF) were evaluated in a patient with right unilateral short lasting paroxysmal kinesigenic dyskinesia, by means of single photon emission computed tomography (SPECT). The patient was a 6 year old boy with no family history. During an attack, increased CBF was seen in the left thalamus. Subtraction of interictal CBF from ictal CBF disclosed a prominent increase in CBF in the left posterolateral part of the thalamus. This finding suggests that abnormal hyperactivity of thalamic neurons could be responsible for the pathophysiology of paroxysmal kinesigenic dyskinesia in this patient.

Deep brain stimulation of the internal pallidum did not improve chorea in a patient with neuro-acanthocytosis.

We report the failure of bilateral globus pallidus internus deep brain stimulation to improve chorea in a patient with chorea-acanthocytosis. Prior to this surgery the patient had experienced a striking but short lived amelioration of symptoms with clozapine therapy.

On defining Sydenham's chorea: where do we draw the line?

Sydenham's chorea (SC) is a major manifestation of rheumatic fever characterized by an array of neuropsychiatric symptoms that vary in severity, timing, and character. Some of the same symptoms are seen in Tourette's syndrome and childhood-onset obsessive-compulsive disorder. Genetic vulnerability appears to play a role in all three conditions. The term PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcus) has been introduced to describe a putative subset of obsessive-compulsive disorder and Tourette's syndrome that bears some resemblance to Sydenham's chorea. This article discusses whether PANDAS should be subsumed under Sydenham's chorea, thus expanding the diagnostic boundaries of Sydenham's chorea to include primarily neuropsychiatric presentations now classified as cases of obsessive-compulsive disorder or Tourette's syndrome. We conclude that PANDAS is a useful construct, but that it would be premature to view it as a subset of Sydenham's chorea-whether defined narrowly or broadly.

MRI assessment of children with obsessive-compulsive disorder or tics associated with streptococcal infection.

OBJECTIVE: The authors assessed selective basal ganglia involvement in a subgroup of children with obsessive-compulsive disorder (OCD) and/or tics believed to be associated with streptococcal infection. METHOD: Using computer-assisted morphometric techniques, they analyzed the cerebral magnetic resonance images of 34 children with presumed streptococcus-associated OCD and/or tics and 82 healthy comparison children who were matched for age and sex. RESULTS: The average sizes of the caudate, putamen, and globus pallidus, but not of the thalamus or total cerebrum, were significantly greater in the group of children with streptococcus-associated OCD and/or tics than in the healthy children. The differences were similar to those found previously for subjects with Sydenham's chorea compared with normal subjects. CONCLUSIONS: These results support the hypothesis that there is a distinct subgroup of subjects with OCD and/or tics who have enlarged basal ganglia. These findings are consistent with the hypothesis of an autoimmune response to streptococcal infection.

[Morvan's fibrillary chorea and acrodynic syndrome following mercury treatment]. / Chorée fibrillaire de Morvan et syndrome acrodynique après un traitement mercuriel.

A 31 year-old inhabitant of French Guiana was prescribed mercuric iodide per os for two and a half months. Shortly before the end of the treatment he developed fasciculations in the trunk and particularly the lower limb muscles, distal painful paresthesias with vasomotor disorders, episodes of excessive perspiration and palmoplantar erythema, moderate fluctuating hypertension, progressive loss of weight and irritability with insomnia. Clinical and electrical signs of neuropathy were lacking. The clinical picture was that of Morvan's fibrillary chorea with acrodynia, the conditions of onset strongly suggesting a mercurial intoxication. Blood and particularly urine mercury levels were elevated. Administration of dimercaprol (BAL) considerably increased urinary excretion of mercury and there was progressive improvement and finally recovery after two months of BAL treatment. This case exemplifies the possible co-existence of fibrillary chorea and acrodynia. Whereas in many cases of fibrillary chorea a precise etiology cannot be determined, the affection can be induced by mercury as by gold administration. The fact that cases of fibrillary chorea due to mercury poisoning are rarely reported may be the result of individual patient hypersensitivity or particular metabolic absorption and excretion features of mercury. This case cannot be included within the continuous activity syndrome of muscle fibers described by Isaacs, since muscle contractures were absent and there was associated acrodynia. Moreover, there was no latent polyneuropathy, in spite of the intense fasciculations. It must be concluded, therefore, that in spite of its rarity fibrillary chorea should keep its semiologic autonomy.