Síndrome de la mano inútil con astereognosia secundaria a un astrocitoma cervical / Useless hand syndrome with astereognosis secondary to a cervical astrocytoma

Resumen El presente caso es un ejemplo del síndrome de la mano inútil de Oppenheim secundario a un astrocitoma cervical alto. La pérdida sensorial propioceptiva y discriminativa suspendida con conservación de la termoalgesia y el tacto crudo en ambas extremidades superiores es secundaria al daño a la entrada de la raíz dorsal y al núcleo cuneiforme. La torpeza y las dificultades para una prensión precisa con la mano se deben al daño del núcleo proprioespinal en las astas dorsales a nivel C3-C4. Este núcleo integra influencias excitadoras descendentes del tracto corticoespinal e interneuronas inhibitorias controladas por los sistemas descendentes y las aferencias de las extremidades anteriores. Probablemente la pérdida de las aferentes cervicales propioceptivas inhibitorias sea la culpable de las dificultades para agarrar con las manos.

The present case is an example of the useless hand syndrome of Oppenheim secondary to a high cervical astrocytoma. The suspended proprioceptive and discriminative sensory loss with conservation of thermoalgesia and crude touch in both upper extremities is secondary to damage to dorsal root entry and cuneate nucleus. The clumsiness and difficulties in precise grasping with the hand are due to damage of the propriospinal nucleus in the dorsal horns at C3-C4 level. This nucleus integrates descending excitatory influences from corticospinal tract and inhibitory interneurons controlled by descending systems and the forelimb afferents. Probably the loss of the inhibitory proprioceptive cervical afferents is the culprit of the difficulties in grasping by the hands.

Síndrome diencefálico en un niño con desnutrición crónica e hiperactividad / Diencephalic syndrome in a child with chronic malnutrition and hyperactivity

El síndrome diencefálico es una causa infrecuente de desnutrición. Se produce por la disfunción del hipotálamo y está asociado a tumores del encéfalo. Los pacientes presentan una grave y progresiva pérdida de peso, aunque el apetito y la ingesta calórica son, por lo general, adecuados. Característicamente, los síntomas neurológicos son tardíos, lo que retrasa la sospecha diagnóstica. Se presenta a un paciente de 2 años y 6 meses de edad con desnutrición crónica grado II, derivado con diagnóstico presuntivo de enfermedad celíaca con mala adherencia y fracaso del tratamiento. Durante la internación, se arribó al diagnóstico de síndrome diencefálico secundario a un astrocitoma pilocítico grado I.

Diencephalic syndrome is an infrequent cause of malnutrition. It is produced by a malfunctioning hypothalamus, and it is related to encephalic tumors. Patients present a serious and progressive weight loss although the appetite and calorie intake are, usually, adequate. Neurological symptoms typically have a late appearance, delaying diagnostic suspicion. We present a patient aged 2 years and a half with grade II chronic malnutrition, referred with presumptive diagnosis of celiac disease, with poor adherence and treatment failure. During hospitalization, diagnosis of diencephalic syndrome secondary to grade I pilocytic astrocytoma was reached.

First Report of Brain Abscess Associated with Pseudozyma species in a Patient with Astrocytoma / 대한진단검사의학회지

A yeast-like strain was isolated from the brain abscess of a patient diagnosed with astrocytoma. Morphological and molecular analysis on D1/D2 domain in the 26S rRNA gene and internal transcript spacer region of the strain revealed that the strain belonged to the genus Pseudozyma. To the best of our knowledge, this is the first report on the isolation of a Pseudozyma strain from brain abscess.

Acute Comitant Esotropia in a Child With a Cerebellar Tumor

We report a case of acute comitant esotropia in a child with a cerebellar tumor. A 3-year-old boy was referred for management of a 9 month history of acute acquired comitant esotropia. On first presentation, the patient's angle of esodeviation was 50 prism-diopters (PD) at distance and near fixation without any lateral incomitance. The cycloplegic refraction revealed +0.75 diopters in both eyes. Very mild bilateral papilledema was found on the fundus examination, but the neurological examination did not reveal any other pathological findings. Brain MRI showed a 5 cm mass located in the midline of the cerebellum as well as hydrocephalus. The mass was completely excised and histological examination confirmed the diagnosis of pilocytic astrocytoma. Despite neurosurgery, the patient's strabismus remained unresolved. One year after neurosurgery, both medial rectus muscles were surgically recessed by 6 mm, resulting in esotropia of 8PD at distant and near fixation without restoration of bifoveal fusion at follow-up 2 years after the eye muscle surgery. Therefore, acute onset comitant esotropia in a child can be the first sign of a cerebellar tumor, even without any other neurological signs and symptoms.

Congenital irreducible atlantoaxial dislocation associated with cervical intramedullary astrocytoma causing progressive spastic quadriparesis.

Simultaneous presence of congenital irreducible atlantoaxial dislocation (AAD) and cervical intramedullary astrocytoma has not been previously described and may cause disabling myelopathy. This 55-year-old lady presented with suboccipital pain, spastic quadriparesis, Lhermitte's phenomenon and sphincteric disturbances. Lateral radiographs and magnetic resonance imaging showed irreducible AAD, occipitalized atlas, C2-3 fusion, and,an intramedullary tumor from C2-5 level iso-to-hypointense, non-enhancing, except in a small segment in the dorsal C2 level. A suboccipital craniectomy with C2-5 laminectomy revealed a greyish-white tenacious tumor. The tumor was decompressed using a C2-5 midline myelotomy and duroplasty. An occipitocervical lateral mass fixation was performed. Histopathology revealed a low-grade astrocytoma. At three-month follow-up, her spasticity had decreased and quadriparesis and sphincteric disturbances were persisting. Postoperative lateral radiographs and intrathecal contrast CT scan showed a stable occipitocervical construct. Thus, the suboccipital craniectomy and laminectomy with midline myelotomy and duroplasty facilitated space for progressively expanding intramedullary astrocytoma with irreducible AAD; the lateral mass fixation provided stability at the craniovertebral junction.

Bilateral ptosis and supranuclear downgaze paralysis

The purpose of this article is to highlight an uncommon combination of supranuclear downward gaze paralysis with bilateral eyelid ptosis in a 53-years-old man with a radiation induced midbrain tumor and to discuss the aspects regarding the centers and pathways that mediate supranuclear vertical gaze movements.

O objetivo deste artigo é ressaltar uma rara condição caracterizada por paralisia supranuclear do olhar conjugado para baixo associada a ptose palpebral bilateral em um homem de 53 anos, causada por tumor mesencefálico radio-induzido, e discutir os aspectos relacionados ao controle supranuclear dos movimentos oculares verticais.

Fascicular multiple ocular motor nerve paresis as first presentation of anaplastic astrocytoma.

A case of spontaneous, painless partial III (pupil-sparing) and IV fascicular nerve paresis as the first presentation of anaplastic astrocytoma is reported. The other ocular, neurological and systemic examination was within normal limits. The literature and possible anatomical location of this atypical presentation is reviewed.

Subependymal giant cell astrocytoma with high choline/creatine ratio on proton MR spectroscopy / Astrocitoma subependimário de células gigantes com alta relação colina/creatina à espectroscopia de prótons por ressonância magnética

OBJECTIVE: To report a case of subependymal giant cell astrocytoma (SEGA) in a patient with tuberous sclerosis, emphasizing the proton MR spectroscopy (MRS) findings. CASE: A three year-old boy with tuberous sclerosis presented with a 3-month history of mild headache. The physical examination showed discrete mental retardation and multiple hypomelanic macules in the legs and back. MRI showed many cortical tubers and subependymal nodules. At the left foramen of Monro, there was a 15 mm nodule, with heterogeneous signal on T1 and T2-weighted images, and strong enhancement after contrast administration. MRS study (multi-voxel PRESS, TE=144ms) showed the following ratios: a) left foramen of Monro nodule N-acetylaspartate (NAA)/creatine (Cr)=0.93 and Choline (Cho)/Cr=1.6 and b) at the right Monro foramen NAA/Cr=1.56 and Cho/Cr=1.29. CONCLUSION: The MRS performed at the SEGA may show high Cho/Cr and low NAA/Cr ratios, similar to the other brain neoplasms. As a consequence, MRS may be a valuable tool for the early detection of neoplastic transformation of subependymal nodules near the foramina of Monro in patients with tuberous sclerosis.

OBJETIVO: Relatar caso de astrocitoma subependimário de células gigantes (ASCG) em paciente com esclerose tuberosa, enfatizando os achados de espectroscopia de prótons por ressonância magnética (EPRM). CASO: Um menino de três anos de idade apresentou-se com cefaléia por três meses. O exame físico demonstrou retardo mental discreto e múltiplas máculas hipomelanóticas no dorso e pernas. A ressonância magnética (RM) evidenciou múltiplos túberes corticais e nódulos subependimários. No forame de Monro esquerdo foi observado um nódulo de 15 mm, com sinal heterogêneo nas imagens ponderadas em T1 e T2, e realce intenso após a administração de contraste. A EPRM (multi-voxel PRESS, TE=144ms) demonstrou as seguintes relações: a) no nódulo no forame de Monro esquerdo N-acetilaspartato (NAA)/creatina (Cr)=0,93 e colina (Cho)/Cr=1,6 e b) no forame de Monro direito NAA/Cr=1,56 and Cho/Cr=1,29. CONCLUSÃO: A EPRM pode demonstrar relações altas de Cho/Cr e baixas de NAA/Cr em pacientes com ASCG, semelhante a outros tumores cerebrais. Sendo assim, a EPRM pode representar importante ferramenta diagnóstica na detecção precoce de transformação neoplásica de nódulos subependimários próximos ao forame de Monro em pacientes portadores de esclerose tuberosa.

Accelerated cerebral vasculopathy after radiation therapy to the brain.

Brain irradiation is commonly used for many primary brain malignancies. We will present two cases of post-radiation vasculopathy of large arteries of patients who received radiation therapy to the brain as part of their tumor management. We will underscore the significance of this condition and suggest the overall management of patients receiving brain radiation at an early age.

An intramedullary tumor presenting with hyperhidrosis.

A case of a cervical intramedullary tumor is reported whose presentation was with disabling hyperhidrosis. The symptom resolved after surgical debulking of the tumor. Hyperhidrosis as a presenting manifestation of an intramedullary tumor has not been reported earlier.

Diencephalic syndrome: a rare and easily overlooked cause of failure to thrive.

BACKGROUND: Diencephalic syndrome (DS) is an uncommon cause of failure to thrive in infants and young children. The major manifestations are emaciation, hyperkinesia, and euphoria. Most patients have a tumor in the hypothalamic-optic chiasma region. CASE REPORT: Two children, aged 14 months and 5 years 9 months, who presented with classic features of DS at an onset of 2 and 3 months respectively, were reported. Neurologic examination was normal, except for papilledema in the second child. Imaging of the brain showed a suprasellar mass, identified as pilocytic astrocytoma in both cases. The first case was lost to follow up. The latter underwent partial resection of the tumor and received radiotherapy postoperatively. He gradually gained in weight and height. CONCLUSION: DS should be a differential diagnosis in any children with emaciation despite adequate caloric intake and an inappropriately euphoric mood. Awareness of this syndrome, careful history taking, general detail as well as neurological examination including fundoscopic examination and appropriated investigations are crucial.

An oligo astrocytoma with widespread calcification along axonal fibres.

A rare case of a young man with a diffusely calcified oligo astrocytoma in right parieto occipital region, which spread along the axonal fibres into right temporal lobe and to the left parietal lobe is presented. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated the extent of the lesion. The mass was partially resected.

Cerebral abscess with astrocytoma.

A child with a right parieto-occipital astrocytoma, caped by a large acute pyogenic abscess with flimsy capsule, detected at emergency craniotomy, is presented. Patient succumbed to the disease three hours following surgery.

Extramedullary astrocytoma of conus region : a short report.

A 55 year old man presented with features of cauda equina syndrome. Magnetic resonance imaging (MRI) showed a well demarcated intradural extramedullary tumour at L2 vertebra. At surgery it was found to be well encapsulated and had no attachment to spinal cord or root. Histopathology including immunohistochemistry confirmed it to be a low grade astrocytoma.

Co-existence of dual intracranial pathology clinical relevance of proton MRS.

The co-existence of neuro-cysticercosis (NCC) and intracranial neoplasm in an individual is a rare entity. Atypical presentation of cerebral cysticercosis may mimic glioma, metastasis, cerebral abscess or vice versa. The dual existence of these two lesions have led to several postulates which may have clinical impact in due course of time i.e. NCC as an oncogenetic factor for glioma or similarity of antigen found in glioma and NCC etc. An adequate management of such cases poses a challenge to both imageologists as well as clinicians. Thus, a proper diagnostic evaluation is essential for successful management of such cases. MR spectroscopy (MRS), although still a clinical research tool, may be extremely useful for exclusion or confirmation of neoplastic lesions in such a clinical scenario. The findings of MRS in collaboration with imaging parameters may increase the diagnostic yield of a MR investigation. The authors encountered five cases of dual intracranial pathologies i.e. neurocysticercosis and glioma. MR spectroscopy was useful to arrive at a definitive diagnosis in such a situation.

Movement disorders caused by brain tumours.

Movement disorders are uncommon presenting features of brain tumours. Early recognition of such lesions is important to arrest further deficit. We treated seven patients with movement disorders secondary to brain tumours over a period of seven years. Only two of these were intrinsic thalamic tumours (astrocytomas) while the rest were extrinsic tumours. The intrinsic tumours were accompanied by hemichorea. Among the extrinsic tumours, there was one pituitary macroadenoma with hemiballismus and four meningiomas with parkinsonism. Symptoms were unilateral in all patients except one with anterior third falcine meningioma who had bilateral rest tremors. There was relief in movement disorders observed after surgery. Imaging by computed tomography or magnetic resonance imaging is mandatory in the evaluation of movement disorders, especially if the presentation is atypical, unilateral and/or accompanied by long tract signs.