Syringomyelia extending to the basal ganglia

1989 ◽  
Vol 71 (4) ◽  
pp. 616-617 ◽  
Author(s):  
Satoshi Okada ◽  
Yoshio Nakagawa ◽  
Kimiyoshi Hirakawa
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pyramidal Tract ◽  
Type I ◽  
Sensory Disturbance ◽  
Resonance Imaging ◽  
Content Type ◽  
Chiari Type I Malformation ◽  
The Right ◽  
Chiari Type I ◽  
Fine Print

✓ A 10-year-old girl was admitted to the hospital with complaints of progressive right hemiparesis and sensory disturbance. Magnetic resonance imaging revealed a Chiari Type I malformation and syringomyelia from T-10 to C-1. The syrinx extended from the medulla to the right putamen along the pyramidal tract.

Coerced training of the nondominant hand resulting in cortical reorganization: a high-field functional magnetic resonance imaging study

2004 ◽  
Vol 101 (2) ◽  
pp. 310-313 ◽  
Author(s):  
Tsutomu Nakada ◽  
Yukihiko Fujii ◽  
Ingrid L. Kwee
Keyword(s):  
Magnetic Resonance Imaging ◽  
High Field ◽  
Cortical Reorganization ◽  
Functional Magnetic Resonance ◽  
Resonance Imaging ◽  
Content Type ◽  
Hand Motion ◽  
Left Handed ◽  
The Right ◽  
Fine Print

Object. The authors investigated brain strategies associated with hand use in an attempt to clarify genetic and nongenetic factors influencing handedness by using high-field functional magnetic resonance imaging. Methods. Three groups of patients were studied. The first two groups comprised individuals in whom handedness developed spontaneously (right-handed and left-handed groups). The third group comprised individuals who were coercively trained to use the right hand and developed mixed handedness, referred to here as trained ambidexterity. All trained ambidextrous volunteers were certain that they were innately left-handed, but due to social pressure had modified their preferred hand use for certain tasks common to the right hand. Although right-handed and left-handed volunteers displayed virtually identical cortical activation, involving homologous cortex primarily located contralateral to the hand motion, trained ambidextrous volunteers exhibited a clearly unique activation pattern. During right-handed motion, motor areas in both hemispheres were activated in these volunteers. During left-handed motion, the right supplemental motor area and the right intermediate zone of the anterior cerebellar lobe were activated significantly more frequently than observed in naturally right-handed or left-handed volunteers. Conclusions. The results provide strong evidence that cortical organization of spontaneously developed right- and left-handedness involves homologous cortex primarily located contralateral to the hand motion, and this organization is likely to be prenatally determined. By contrast, coerced training of the nondominant hand during the early stages of an individual's development results in mixed handedness (trained ambidexterity), indicating cortical reorganization.

Spontaneous drainage of syringomyelia

1993 ◽  
Vol 79 (1) ◽  
pp. 132-134 ◽  
Author(s):  
Antonio Santoro ◽  
Roberto Delfini ◽  
Gualtiero Innocenzi ◽  
Claudio Di Biasi ◽  
Guido Trasimeni ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Mr Imaging ◽  
Clinical Course ◽  
Spontaneous Resolution ◽  
Type I ◽  
Content Type ◽  
Chiari Type I Malformation ◽  
Spinal Subarachnoid Space ◽  
Chiari Type I ◽  
Fine Print

✓ Two cases are reported of Arnold-Chiari type I malformation associated with syringomyelia, in which magnetic resonance (MR) imaging revealed spontaneous decompression of the syrinx. In one case axial MR imaging sections showed a communication between the syrinx and the spinal subarachnoid space, which supports the hypothesis that fissuring of the cord parenchyma is instrumental in the spontaneous resolution of syringomyelia. The MR imaging changes were not accompanied by variations in the patients' clinical course.

Chiari Type I malformation and syringomyelia in unrelated patients with blepharophimosis

1998 ◽  
Vol 89 (5) ◽  
pp. 835-838 ◽  
Author(s):  
Philippe Paquis ◽  
Michel Lonjon ◽  
Madeleine Brunet ◽  
Jean Claude Lambert ◽  
Patrick Grellier
Keyword(s):  
Autosomal Dominant ◽  
Radicular Pain ◽  
Hereditary Diseases ◽  
Type I ◽  
Content Type ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Familial Type ◽  
Chiari Type I ◽  
Fine Print

✓ Syringomyelia is a rare, mainly sporadic disease of the spinal cord, which is associated with 80% of cases in which a Chiari Type I malformation is also present. A mendelian transmission of syringomyelia (autosomal dominant or recessive) has been proposed in approximately 2% of reported cases. The association of syringomyelia with hereditary diseases (Noonan's syndrome, phacomatoses) has been mentioned frequently in the literature. The authors report the presence of a Chiari Type I malformation accompanied by syringomyelia in two unrelated patients affected by a familial Type II blepharophimosis—ptosis—epicanthus inversus syndrome (BPES). The first patient was a 35-year-old woman who presented with a right C-8 root paresia. The second case involved a 20-year-old man who complained of cervical radicular pain. Both belong to families in which BPES was segregated in an autosomal dominant modality, but other family members had no known neurological symptoms. To the authors' knowledge, such a combination has never been described. Perhaps the possible involvement of a genetic component in some cases of Chiari Type I—associated syringomyelia will someday be debated.

Hindbrain herniation: an unusual occurrence after shunting an intracranial arachnoid cyst

1994 ◽  
Vol 81 (1) ◽  
pp. 126-129 ◽  
Author(s):  
Maher I. Hassounah ◽  
Bengt E. Rahm
Keyword(s):  
Arachnoid Cyst ◽  
Type I ◽  
Content Type ◽  
Stereotactic Aspiration ◽  
Chiari Type I Malformation ◽  
Hindbrain Herniation ◽  
Intracranial Arachnoid Cyst ◽  
Unusual Occurrence ◽  
Chiari Type I ◽  
Fine Print

✓ The case is presented of a prepontine arachnoid cyst in a 28-year-old woman. She was treated by craniotomy, stereotactic aspiration, internal shunting, and finally by insertion of a cystoperitoneal shunt. Eight months later she developed an asymptomatic Arnold-Chiari type I malformation. The pathogenesis of this unusual hindbrain herniation is explained on the basis of the differential pressure between two compartments and cephalocranial disproportion.

MRI demonstration of clot in a small unruptured aneurysm causing stroke

1986 ◽  
Vol 65 (3) ◽  
pp. 411-412 ◽  
Author(s):  
Theodore W. Eller
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Middle Cerebral Artery ◽  
Computerized Tomography ◽  
Cerebral Artery ◽  
Unruptured Aneurysm ◽  
Resonance Imaging ◽  
Content Type ◽  
The Right ◽  
Fine Print

✓ The case is reported of a 69-year-old woman with an 18-mm unruptured aneurysm of the right middle cerebral artery which caused a moderate stroke. Magnetic resonance imaging revealed a clot inside the aneurysm that was not visible on computerized tomography scans. The danger of embolism from the clot prompted clipping of the aneurysm.

A novel fibroblast growth factor receptor 2 mutation in Crouzon syndrome associated with Chiari Type I malformation and syringomyelia

2002 ◽  
Vol 97 (2) ◽  
pp. 396-400 ◽  
Author(s):  
Hironori Fujisawa ◽  
Mitsuhiro Hasegawa ◽  
Shinya Kida ◽  
Junkoh Yamashita
Keyword(s):  
Fibroblast Growth Factor Receptor ◽  
Growth Factor Receptor ◽  
Type I ◽  
Crouzon Syndrome ◽  
Content Type ◽  
Chiari Type I Malformation ◽  
Lambdoid Synostosis ◽  
Chiari I ◽  
Chiari Type I ◽  
Fine Print

Object. It has been reported that due to premature synostosis of the lambdoid suture in the first 24 months of life, more than 70% of patients with Crouzon syndrome concurrently suffer from chronic tonsillar herniation (Chiari Type I malformation) and some (20%) associated syringomyelia. The goal of the present study was to examine mutations in the fibroblast growth factor receptor (FGFR) genes in Crouzon syndrome and its related conditions. Methods. Five patients were studied: three with Crouzon syndrome (one sporadic and two familial), one with sporadic Chiari I with syringomyelia, and one with unilateral lambdoid synostosis. Deoxyribonucleic acid was screened for FGFR1–3 mutations by using single-strand conformational polymorphism and subsequent direct sequencing. Two types of missense mutations were detected in the FGFR2 gene, Cys342Trp (1205, TGC → TGG) in a patient with sporadic Crouzon syndrome and Tyr281Cys (1021, TAC → TGC) in two siblings (brother and sister) with familial Crouzon syndrome, respectively. The former has been reported only in sporadic cases but the latter has not previously been identified. A polymorphism in the FGFR3 gene, Asn294Asn (882, AAT → AAC), was also detected in three patients. No mutation was found in the patient with sporadic Chiari I with syringomyelia. Conclusions. The FGFR2 missense mutation was detected in Crouzon syndrome but not in sporadic Chiari I with syringomyelia or lambdoid synostosis. A novel FGFR2 mutation, Tyr281Cys, was found in familial Crouzon syndrome with Chiari I and syringomyelia. It may be informative to look for this in patients with Crouzon syndrome and associated syringomyelia.

Childhood meningioma associated with meningioangiomatosis

1993 ◽  
Vol 78 (2) ◽  
pp. 287-289 ◽  
Author(s):  
Deborah Blumenthal ◽  
Mariana Berho ◽  
Stephen Bloomfield ◽  
Sydney S. Schochet ◽  
Howard H. Kaufman
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Computerized Tomography ◽  
Resonance Imaging ◽  
Content Type ◽  
Histological Features ◽  
Interhemispheric Fissure ◽  
Mitotic Figures ◽  
The Right ◽  
Fine Print

✓ This paper reports a childhood meningioma in association with meningioangiomatosis. The patient was an 11-month-old baby boy who presented with a left focal seizure. He had no stigmata of neurofibromatosis. Computerized tomography and magnetic resonance imaging revealed an extra-axial, contrast-enhancing mass in the interhemispheric fissure which indented the right frontal lobe. The tumor was totally removed. Microscopically, the lesion was a fibrous and transitional meningioma with foci of necrosis and scattered mitotic figures. The adherent neural parenchyma showed the histological features of meningioangiomatosis. It is concluded that meningioangiomatosis may accompany childhood meningiomas more often than is generally appreciated.

Rosai—Dorfman disease with dural sinus invasion

2005 ◽  
Vol 102 (3) ◽  
pp. 550-554 ◽  
Author(s):  
Cheng-Hong Toh ◽  
Yao-Liang Chen ◽  
Ho-Fai Wong ◽  
Kuo-Chen Wei ◽  
Shu-Hang Ng ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Frontal Lobe ◽  
Cerebellar Hemisphere ◽  
Dural Sinus ◽  
Resonance Imaging ◽  
Content Type ◽  
Rosai Dorfman Disease ◽  
The Right ◽  
Fine Print

✓ Rosai—Dorfman disease (RDD) is an idiopathic proliferation of histiocytes that affects the lymph nodes. Central nervous system involvement in the absence of nodal disease is extremely rare. On neuroimaging studies, intracranial RDD appears as solitary or multiple well-circumscribed, dura-based lesions. The authors report on two cases of RDD with locally aggressive features including dural sinus invasion, which to their knowledge has never before been described. A 60-year-old woman presented with progressive dizziness and vertigo that had lasted for 1 week. Cranial computerized tomography and magnetic resonance imaging revealed an extraaxial homogeneous lobulated enhancing mass involving the right occipital lobe and the right cerebellar hemisphere. Invasion of the right transverse sinus was identified on a cerebral digital subtraction angiogram. A 59-year-old man with no prior medical illness experienced progressive weakness of both upper extremities and a partial complex seizure. Magnetic resonance imaging of his brain revealed a well-circumscribed enhancing mass in the left frontal lobe with extension to the right frontal lobe and invasion of the superior sagittal sinus. Both patients underwent resection of their brain masses. Pathological studies identified the disease as RDD in both patients.

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