Transient left temporal lobe lesion in Menkes disease may influence the generation of tonic spasms

We report a 7-month-old boy with Menkes disease who presented West syndrome. Magnetic resonance imaging (MRI) revealed atrophy of the frontal and parietal lobes, subdural hematoma on the right side, and left temporal lobe lesion (low intensity in T1-weighted imaging (T1-WI), high intensity in T2-weighted imaging (T2-WI) and low intensity in diffusion-weighted imaging (DW-I)) at 7 months of age. The apparent diffusion coefficient (ADC) was 1.68 × 10⁻³ mm²/s in the left temporal lobe lesion and 1.15 × 10⁻³ mm²/s on the contralateral side. ¹H-magnetic resonance spectroscopy (¹H-MRS) revealed a decrease in N-acetylaspartate/(creatine + phosphocreatine) (NAA/Cr) (0.71) and a lactate peak in the left temporal lobe lesion. At 8 months of age, the left temporal lobe lesion disappeared, the ADC of this lesion was within the normal range (1.10 × 10⁻³ mm²/s), and ¹H-MRS revealed a slight increase in NAA/Cr (1.12) and disappearance of the lactate peak. We suspected that the transient temporal lobe lesion in Menkes disease was mainly vasogenic edema. Electroencephalography (EEG) revealed left hemisphere dominant hypsarrhythmia and slowing in the left hemisphere. Ictal EEG revealed generalized slow wave burst with P3, T3 spike antecedence and the antecedent spike was consistent with left temporal lobe lesion. After disappearance of the left temporal lobe lesion, tonic spasms disappeared and EEG findings improved. In this case, the clinical course and ictal EEG suggested that epileptic activity from the left temporal lobe lesion may have given rise to tonic spasms.     

Giant cell tumor of the temporal bone with intratumoral hemorrhage

We report a case of hemorrhagic giant cell tumor (GCT) of the temporal bone in a 77-year-old woman. The patient suffered from sudden-onset headache and vomiting associated with left temporal hemorrhage. MRI revealed a left temporal extradural mass lesion expanding to the subtemporal fossa, showing strong hypointensity on T(2)-weighted imaging. Subsequent MRI revealed tumor growth with multiple cystic components at 1-month follow up. The tumor was found to be a GCT associated with recent intratumoral hemorrhage and abundant hemosiderin pigmentation. T(2)-weighted MRI of the GCT strongly supported hemosiderin deposition. Secondary formation of cystic components in the GCT can also reflect prior hemorrhage and indicate the progression of shape modification. A literature review revealed that hemosiderin deposition in this rare entity is not as rare as previously thought and that massive intratumoral hemorrhage may occur.     

术中1.5T核磁共振外科治疗难治性癫癎

目的探讨应用术中1.5T核磁共振(MRI)治疗难治性癫癎的手术效果。方法手术治疗难治性癫癎15例,利用术中1.5T核磁共振,术前常规行T1、T2及T1加强,及弥散张量成像,术中切除(切开)后行T1、T2及T1加强及弥散张量成像检查,以确定切除范围及功能区定位,其中5例术中MR检查后扩大切除。结果左侧枕叶局灶性皮质发育异常2例,左侧颞叶海绵状血管瘤4例,左侧颞叶海马硬化1例,右侧额叶胚胎发育不良性神经上皮肿瘤1例,右侧中央前回局灶性皮质发育不良1例,右侧颞叶海马硬化1例,右侧颞叶海绵状血管瘤1例,右侧颞叶胶质瘤1级1例,右侧中央后回海绵状血管瘤1例,胼胝体切开2例,engle分级:Ⅰ级11例,Ⅱ级4例。结论术中1.5T核磁共振对切除(切开)病灶及功能保护有指导意义。     

A case of astrocytoma with extracranial extension after malignant transformation]

A case of astrocytoma with extracranial extension after malignant transformation is presented. The patient was a 58-year-old female who suffered from headache. The initial magnetic resonance imaging (MRI) demonstrated a slightly hyperintense tumor on T 2-weighted images in the tip of the left temporal lobe, and no contrast enhancement on gadolinium-enhanced T 1-weighted images(Gd-T 1 WI). On digital subtraction angiography, there was no tumor staining. The initial diagnosis was made as low-grade astrocytoma. However two months later, her symptoms aggravated suddenly. MRI revealed a remarkably growing tumor with ring-like enhancement on Gd-T 1 WI. She underwent a temporal lobectomy, which pathologically demonstrated a glioblastoma. After surgery, chemotherapy and radiotherapy were performed. The tumor invades the skull base and extended into the infratemporal fossa 25 months after surgery.     

MRI and SPECT of Klinefelter's Syndrome with Various Neuropsychiatric Symptoms: A Case Report

Abstract: We present here magnetic resonance imaging (MRI) and single photon emission computed tomography with ¹²³I-N-isopropyl-p-iodoamphetamine (¹²³I-IMP-SPECT) of a patient suffering from Klinefelter's syndrome with various neuropsychiatric symptoms. He was a 30-year-old male, who showed impaired consciousness seizures, auditory hallucination, delusion of reference, delusion of grandeur, psychomotor excitement and intellectual impairment. Although no focal lesion was detected by computed tomography or T1-weighted MRI, T2-weighted MRI provided a heterogeneous high-signal-intensity lesion of the inferior part of the left temporal lobe, which was not enhanced with Gd-DTPA. In addition ¹²³-IMP-SPECT exhibited focal hypoperfusion in the left temporal lobe on the early images. We suggest that the neuropsychiatric symptoms of this case are associated with the focal organic brain dysfunction which was revealed by MRI and ^123I-IMP-SPECT.     

Chiasmal radionecrosis after irradiation of the sella turcica using a conventional dosage. Contribution of magnetic resonance imaging

A 47 year-old man developed rapid visual loss, visual field defects and memory disturbances after radiotherapy with conventional doses for a pituitary metastasis from a renal carcinoma. CT and MRI did not show recurrent tumour, pituitary apoplexy or empty sella. Eventually, T2-weighted MRI images showed abnormal high signals in the optic chiasm, the left mesial temporal lobe and the right inferior frontal lobe, supporting the diagnosis of delayed radionecrosis. The role of chemotherapy associated with radiotherapy is discussed.     

Glioblastoma Mimicking Herpes Simplex Encephalitis

We report a case of 70-year-old man with glioblastoma presenting as acute encephalitic illness. The patient exhibited sudden onset of cognitive impairment and headache for 2 days. Initial brain MRI showed left temporal lobe hyperintensity, and cerebrospinal fluid cytology revealed a mild pleocytosis. The patient had initially improved after medical treatment with a presumptive diagnosis of herpes simplex encephalitis (HSE). After 8 months, the patient complained of recurrent seizures. A follow-up brain MRI revealed marked increases in size and surrounding perilesional edema in the left temporal lesion on T2-weighted images and a new contrast-enhancing lesion on gadolinium-enhanced T1-weighted images. Stereotactic brain biopsy revealed a glioblastoma. The atypical encephalitic presentation of glioblastoma should be considered if definitive evidence for the diagnosis of HSE cannot be obtained.     

A rare case of dysembryoplastic neuroepithelial tumour in occipital lobe presenting with only headache.

We report an unusual case of dysembryoplastic neuroepithelial tumour (DNT) located in the occipital lobe presenting with only headache. A 31 year old woman presented with headache. She had no history of epilepsy and neurological examination revealed no abnormal findings. Computed tomography (CT) scanning revealed a multilobulated mass lesion with calcification in the right occipital lobe. Magnetic resonance (MR) imaging demonstrated a heterogeneously enhanced mass with hypointense signals on T1- and hyperintense signals on T2-weighted images. The lesion was totally resected and histopathologically diagnosed as DNT. Physicians must bear in mind that DNT may occur in the occipital lobe and present with only mass effect.     

Serial brain imaging analysis of stroke-like episodes in MELAS

We report 2 patients of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) and consider the pathophysiology of stroke-like lesions, using magnetic resonance imaging (MRI), diffusion-weighted imaging (DWI) on MRI, perfusion imaging on MRI, and 1H magnetic resonance spectroscopy (1H-MRS). In Patient 1, T2-weighted imaging (T2-WI) on MRI at onset and even at 44 days after onset of the stroke-like episode showed high intensity in left parietal, temporal, and occipital lobe lesions. In the temporal lobe lesion, the apparent diffusion coefficient (ADC) at 44 days after onset was higher (average: 1.219x10(-3)mm2/s) than that in a normal region (average: 0.796x10(-3)mm2/s). (1)H-MRS of the left parietal lobe lesion at the same day showed a decrease in N-acetylaspartate/(creatine+phosphocreatine) (NAA/Cr) (0.43) and a peak in lactate. 1H-MRS of the contralateral side at the same day showed NAA/Cr (1.57) and no peak in lactate. Thereafter, ADC gradually decreased and NAA/Cr gradually increased, and the peak in lactate disappeared in the lesion. In Patient 2, T2-WI at onset showed high intensity in bilateral occipital lobe lesions. In the left occipital lobe lesion, ADC at the same day was higher (1.082x10(-3)mm2/s) than that in a normal region (average: 0.841x10(-3)mm2/s). (1)H-MRS of the left occipital lobe lesion at the same day showed a decrease of NAA (3.0mM) and a peak in lactate (13.1mM) (measured by LCModel). In 1H-MRS of the normal left parietooccipital lobe at 4 months before onset, NAA was 7.6mM and there was no peak in lactate (0mM). Perfusion imaging at onset showed high intensity in bilateral occipital lobes, which indicated hyperperfusion in stroke-like lesions. Thereafter, ADC gradually decreased and the peak in lactate partially decreased, and the low concentration of NAA persisted (regardless of the partial recovery) in the lesion. These results suggest that the stroke-like episodes is related to vasogenic edema, hyperperfusion, and neuronal damage. Acute oxidative phosphorylation defect may have a crucial role in the pathophysiology of stroke-like episodes.     

Corticobasal degeneration presenting with progressive conduction aphasia

We report the case of a woman with primary progressive aphasia (PPA) presenting with conduction aphasia. Neurological findings showed bilateral finger tremor and signe de poignet figé in her right hand. Memory, orientation, and activities of daily living were well preserved. Linguistic examination showed severe impairment in repetition, fluent spontaneous speech with phonemic paraphasia, and relatively well preserved comprehension. Limb-kinetic apraxia and parkinsonism were not observed during the course of her illness. T1-weighted magnetic resonance imaging revealed severe atrophy of the left temporal lobe and dilatation of the left Sylvian fissure. Neuropathological findings demonstrated the most severe atrophy in the left superior temporal gyrus and Gallyas–Braak-positive or phosphorylated tau-immunoreactive cytoskeletal structures, which were consistent with corticobasal degeneration (CBD). We speculate that the progressive conduction aphasia of our patient might have been caused by left temporal lobe impairment. We suggest that progressive conduction aphasia may be a feature of CBD presenting with PPA.     

Secondary obsessive-compulsive disorder related to diaschisis after pontine infarction, successfully treated with paroxetine

This report describes a case of secondary obsessive-compulsive disorder related to diaschisis after pontine infarction. A 71-year-old male developed obsessive images, after a pontine infarction. A brain magnetic resonance imaging scan showed a low intensity area in the right pons on T1-weighted image, while brain single photon emission computed tomography showed low cerebral blood flow in the temporal lobe as well as the pons. In this case, infarction in the pons appeared to cause dysfunction in the temporal lobe via the neural projection network, an example of so-called 'diaschisis'. This case suggests that brainstem infarction and decreased temporal lobe perfusion can lead to secondary obsessive-compulsive disorder.     

Unilateral Temporal Lobe Stroke Causing Ischemic Transient Global Amnesia: Role for Diffusion-Weighted Imaging in the Initial Evaluation

Ischemia has been proposed as a cause of transient global amnesia (TGA), but proof has been lacking. The authors performed magnetic resonance imaging on a 77-year-old woman with classic TGA at 4 hours and at 6 days after the onset of symptoms. Her initial diffusion-weighted imaging (DWI) and apparent diffusion coefficient imaging suggested an acute infarct in the left mesial temporal lobe. Follow-up T2-weighted imaging at 6 days confirmed the lesion as an ischemic infarct, despite resolution of her symptoms. DWI permits early detection of small ischemic lesions and may identify patients with ischemic TGA who should be evaluated for potential sources of emboli.     

Kanji-predominant alexia with agraphia in opticospinal multiple sclerosis]

Alexia with agraphia is very rare symptom in multiple sclerosis. We present a patient of opticospinal multiple sclerosis with kanji-predominant alexia with agraphia. A 55-year-old, right-handed man was admitted to our hospital because of difficulty in reading and writing in August 2001. The patient had been diagnosed as having relapsing-remitting opticospinal multiple sclerosis eight years prior to admission. Language examination showed alexia with agraphia predominantly affecting kanji and also mild naming difficulties, but a good comprehension and a normal repetition. T2-weighted MRI demonstrated hyperintensity area in the left temporo-parietal lobe, involving the white matter beneath the postero-inferior temporal lobe and inferior parietal lobule. On brain SPECT, low blood perfusion was observed in the left temporo-parietal regions. Although agraphia for kana and alexia for both kana and kanji improved after steroid therapy, agraphia for kanji did not improve. After the treatment, high intensity area of inferior parietal lobule was disappeared on MRI, and the hypoperfusion of inferior parietal lobule on brain SPECT was also improved, but the lesion of left postero-inferior temporal lobe did not show any remarkable changes. We considered that the kanji-predominant alexia with agraphia was due to the lesions of left inferior parietal lobule and postero-inferior temporal lobe, and agraphia for kanji was due to the lesion of left postero-inferior temporal lobe.     

Herpes simplex encephalitis with expanded cerebral cortex lesions on T1-weighted MRI after clinical improvement: a case report]

We described a 58-year-old woman with herpes simplex encephalitis (HSE), who initially had fever and developed impaired consciousness. Cerebrospinal fluid (CSF) examination showed mononuclear pleocytosis and the existence of herpes simplex virus (HSV) DNA. The first T1-weighted MR image showed symmetrical swelling and low signal intensity lesions in the medial temporal lobes and hippocampus. T2-weighted MR image showed high signal intensity lesions in the medial temporal lobes, the amygdala, the hippocampus, the insula and the cingulate gyri bilaterally. After the treatment with intravenous acyclovir and betamethasone, impaired consciousness and recent memory disturbance gradually improved. On the second T1-weighted MR image examination, eighteen days after the onset, high signal intensity lesions were demonstrated in the right medial temporal lobe, the right hippocampus, the left insula and the bilateral cingulate gyri. Although the clinical symptoms had improved significantly over three months, the high signal intense lesions on T1-weighted MR images were also detected in the left medial temporal lobe, the right insula, and the straight gyrus. Brain CT did not demonstrate any abnormalities. The repeated CSF examinations showed negative HSV DNA and a decreased number of WBC. However, oligoclonal IgG bands were continuously positive. Myelin basic protein level and IgG index increased in parallel with the expansion of the cerebral lesions on T1-weighted MR images. In the present case, the abnormality of T1-weighted MRI was thought to indicate hemorrhagic inflammatory lesions that could not be detected by CT. The increased level of myelin basic protein, the elevated IgG index and the continuous positive oligoclonal IgG indicated continuous immunologic response against HSV in these lesions.     

A case of primary central nervous system lymphoma with the onset of impotence]

A 51-year-old man suffered from impotence for 10 months. Five months before he developed difficulty in urination and walking because of his both leg weakness. He was admitted to the hospital because of urinary incontinence, paraplegia and occipital headache. Neurologic examination revealed neck stiffness and Lhermitte's sign. The cranial nerves were intact with the exception of choked disc. He had weakness of lower extremities and right arm, and sensory loss on the right side including face. The plantar responses were extensor bilaterally. MR images revealed diffuse swelling in the cervical and thoracic spinal cord on a T 1-weighted image without enhancement by Gd-DTPA and diffuse high intensity of the spinal cord on a T 2-weighted image. MR image of the brain revealed the low intensity in the left temporal and occipital lobe with slightly enhancement by Gd-DTPA, the high intensity in the left temporal and occipital lobe white matter on a T 2-weighted image. Diagnosis of malignant lymphoma (B cell type) was made by brain biopsy. Combined chemotherapy was performed and his symptoms resolved and the lesions on MRI disappeared. The number of cases of primary spinal intramedullary malignant lymphoma was very rare and the majority of the cases had weakness or sensory impairment in the lower extremities in the initial symptoms. This is the first case which had impotence as the initial symptom. In addition, it must be taken into consideration of this disease when the patient has myelopathy with unknown etiology.     

Contralateral temporal hypometabolism on positron emission tomography in temporal lobe epilepsy

Introduction — No detailed case studies report lateralised hypometabolism on positron emission tomography (PET) contralateral to the epileptogenic focus in temporal lobe epilepsy (TLE). Material and methods — We performed ¹⁸F fluorodeoxyglucose (FDG) PET in two intractable TLE patients. Results — One had right temporal interictal spikes on electroencephalography (EEG) and a right medial temporal lobe lesion on magnetic resonance imaging (MRI). FDG-PET showed decreased uptake in the left temporal lobe. Right temporal ictal onset, with bilateral interictal epileptiform activity, occurred on intracranial EEG. He is seizure free after right temporal lobectomy and ganglioglioma resection. The second had right temporal lobe interictal and ictal EEG activity. MRI demonstrated right anteriomedial temporal increased T2 signal. Neuropsychology revealed bilateral cognitive dysfunction. FDG-PET showed left anterior temporal and lateral frontal hypometabolism. He is seizure free after right temporal lobectomy. Conclusion — These findings suggest that regional uptake asymmetry on FDG-PET may be give misleading lateralising information in TLE.     

Diffusion tensor imaging in frontal lobe epilepsy

We report a 13-year-old female with refractory frontal lobe epilepsy in whom diffusion tensor imaging was useful for exploring subtle cortical malformation. She had frequent simple partial seizures characterized by clonic movement of the right upper extremity. Conventional magnetic resonance imaging was not conclusive. A diffusion tensor imaging revealed an increased apparent diffusion coefficient and reduced anisotropy in the left frontal lobe. Positron emission tomography disclosed hypermetabolism in the adjacent area. We could identify the relatively thick cortex with an indistinct gray-white matter junction in a thin-slice T(1)-weighted image.     

Serial positron emission tomography (PET) in gliomatosis cerebri treated with radiotherapy: a case report

Results of serial positron emission tomography (PET) in a biopsy-proven case of gliomatosis cerebri (GC) are reported. Computed tomography (CT) with and without contrast failed to detect focal abnormalities, but magnetic resonance (MR) revealed iso-intensity or low-intensity lesions in T1-weighted images and high-intensity lesions in T2-weighted images. Lesions were seen in the left thalamus, right temporal lobe and claustrum, and pons. Radiotherapy remarkably improved clinical and imaging findings. Both before and shortly after radiotherapy, 11C-methionine PET images showed hypermetabolism while 15O-water PET images showed a marked increase in cerebral blood flow in GC lesions. However, 6 months later PET images had remarkably improved, appearing nearly normal.     

Diffusion-weighted MRI in transient global amnesia: Elevated signal intensity in the left mesial temporal lobe in 7 of 10 patients

Prompted by the findings of previous studies with positron emission tomography and single photon emission computed tomography, which demonstrated hypoperfusion or hyperperfusion in the left temporal lobe in isolated patients with transient global amnesia (TGA), we compared the sensitivity of diffusion-weighted (DW) magnetic resonance imaging (MRI) with that of conventional T1- and T2-weighted MRI in patients with TGA. Ten patients with the typical syndrome of a pure TGA were included in the study. For all patients, a coronal DW sequence, a steady-state free precession (SSFP) sequence, and conventional T1- and T2-weighted turbo spin-echo sequences were obtained. Seven of the 10 patients had elevated signal intensity in the left hippocampal region on DW MRI; moreover, 3 of these 7 patients exhibited bilateral signal abnormality in this sequence. All conventional T1- and T2- weighted images as well as all follow-up studies were normal. The signal elevation in DW MRI correlates with a decrease in the interstitial space and with cellular edema in the temporal lobe during TGA. The underlying pathomechanism causing this cellular edema cannot be clearly outlined using DW MRI. Our data are, however, compatible with spreading depression. This is the first study to show that DW MRI is a sensitive MRI method for evaluating TGA, especially in the early stage of the disease.     

Epidermoid tumour within the collateral sulcus: A rare location and atypical presentation

Epidermoid tumours (ETs) are uncommon benign lesions that may grow by spreading in the subarachnoid space of the basal cisterns and expanding to conform to the shape of specific sulci and fissures. A temporobasal location is very rare, and there have been no reports regarding single sulcus involvement of ETs. We describe the case of an ET located on the basal surface of the temporal lobe, predominantly within the collateral sulcus, which separates the parahippocampal gyrus medially from the fusiform gyrus laterally. We report the case of a 25-year-old woman with complex visual hallucinations. MRI of the brain revealed a right temporobasal mass lesion, hypointense on T(1)-weighted and hyperintense on T(2)-weighted images, with minimal contrast enhancement, on the basal surface of the temporal lobe. Right-sided anterior temporal lobectomy, along with microsurgical removal of the collateral sulcus ET were performed with consequent resection of mesial temporal structures (the region of the hippocampus, parahippocampal gyrus, and amygdala). It is important to consider ETs when treating lesions on the basal temporal lobe, since the inferior surface of the temporal lobe, more specifically the collateral sulcus, may be a convenient space for ETs to localize. Total surgical resection should be the goal in these cases; however, the surgical approach may be tailored to include the resection of mesial temporal lobe structures when seizure is the predominant presenting symptom.