Halil Onder

Reversible transverse sinus collapse in a patient with idiopathic intracranial hypertension

The association of idiopathic intracranial hypertension (IIH) with stenosis or narrowing of the transverse sinuses (TSs) is well known. However, there is debate as to whether the stenosis is a cause or consequence. Here we describe a case of IIH and narrowing of the TSs, with four relapses and recoveries after repeated CSF diversions with lumbar puncture (LP) over 2 months. Subsequently, implantation of a lumboperitoneal shunt (LPrS) ensured recovery. MR venography 20 months after LPrS showed normally calibrated TSs. We show repeated MR venography findings before and after the LPs, and discuss the pathogenesis of IIH in terms of the cause and effect relationship between IIH and sinus collapse.

Central pontine and extra-pontine myelinolysis after correction of severe hypoglycemia

A 73-year-old man with diabetes mellitus, hypertension, and recent Whipple’s operation for pancreatic cancer was admitted due to fever, cough and weight loss. His hospitalization was complicated by progressive malnutrition despite total parenteral nutrition, difficulties in controlling blood glucose levels and recurrent pneumonia. Two months after his admission, he developed sudden-onset coma attributed to severe hypoglycemia (deep to 13 mg/ dL). Blood glucose level was 214 mg/dL at 3 h prior to the incident and 238 mg/dL at 2 h after prompt replacement. Subsequently, glucose levels were stabilized between 150 and 250 mg/dL. Of note, no imbalance of electrolytes including sodium was noted during the hospital course. Because his status did not recover following glucose replacement, a brain magnetic resonance imaging (MRI) was obtained on the same day, and did not reveal any specific pathology. MRI was repeated after metabolically uneventful 11 days in which he stayed comatose. This MRI was typical for central pontine myelinolysis (CPM), characterized by restricted diffusion (Fig. 1a) and T2hyperintensity involving mid-pontine transverse fibers (Fig. 1b) with capsular (extra-pontine) extension (Fig. 1c). CPM is typically seen in connection with rapid correction of chronic and severe hyponatremia. However, it is well recognized that CPM can also occur in a variety of clinical diseases or conditions resulting in rapid and significant shifts in plasma osmolality in the absence of abnormalities of sodium homeostasis [1]. Fluctuations of blood glucose concentrations can be so brisk and profound as to cause serum tonicity changes wide enough for CPM development. In accordance, case reports, albeit quite rare, connecting CPM to the episodes of severe hypoglycemia [2], severe hyperglycemia [3, 4] or fast transition between hyperglycemia and hypoglycemia [5] can be found in the literature. We add another example, wherein CPM triggered by either profound hypoglycemia or, more likely, its rapid correction, supporting the theory that the pathogenesis of CPM is primarily dependent on tonicity changes, not independently from sodium abnormalities itself. Another feature that warrants further discussion pertaining to this case is unusual extra-pontine lesion location. It is well known that extra-pontine myelinolysis is frequently seen in the setting of CPM. Therefore, ‘‘osmotic demyelination syndrome’’ has recently been coined as an alternative description for the syndrome. Indeed, many parts of the central nervous system can be involved in CPM in addition to basis pontis. Frequently involved extrapontine areas are the thalamus, sub-thalamic nucleus, external geniculate body, putamen, globus pallidum, cerebellar and subcortical white matter. Internal capsule involvement, as documented in the case presented herein, is an exceptional feature for CPM [6]. An MRI documentation of unilateral involvement of the internal capsule has recently been reported in the setting of extra-pontine H. Onder E. M. Arsava M. A. Topcuoglu (&) Neurosonology Laboratory, Neurointensive Care Unit, Department of Neurology, Faculty of Medicine, Hacettepe University Hospitals, Hacettepe University, Sihhiye, 06100 Ankara, Turkey e-mail: mat@hacettepe.edu.tr; matopcuoglu@yahoo.com

Progressive anterior operculum syndrome due to frontotemporal lobar degeneration

To the Editor, Dysarthria and dysphagia are generally atypical for early phase of frontotemporal degeneration (FTD); nonetheless in a subgroup of FTD with motor neuron disease (MND), these symptoms can be seen in the initial phase attributable to bulbar palsy [1]. On the other hand, dysarthria and dysphagia can be the initial symptoms in a distinct subgroup of pure FTD as a clinical presentation of bilateral anterior opercular syndrome which is defined as impairment of voluntary control of facial and/or orobuccal functions without affecting automatic and emotional movements [2]. Here, we demonstrate an illustrative rare case of frontotemporal dementia with anterior opercular syndrome and its imaging findings.

Dramatic improvement by levodopa treatment in a patient with vascular parkinsonism

Sir, Herein, a-60-year-old female patient is described who attended to neurology clinic due to complaints of walking difficulty and postural instability. Upon history taking, it was learnt that the complaints of the patient had started 3 years ago, which had become rather evident following a stroke characterized by dysarthria and left lower extremity paralysis. In the following course, gait problems had insidiously deteriorated. Of note; no prodromal symptoms of Parkinson’s disease (PD) such as hyposmia, constipation, and REM sleep behavior disorder were defined. The patient did not define complaints of memory impairment or hallucinations. She had no symptoms of dysautonomia such as orthostatic hypotension or gastroparesis. On neurological examination, she was evaluated as orientated and fully cooperated. Her speech was dysarthria. Remarkably, her walking was characterized with small steps and she had difficulty particularly during turning which was compatible with lower body parkinsonism (Video 1). Ocular examination revealed normal pursuit and saccadic eye movements. Motor, sensory, and cerebellar examination were in normal ranges. Besides, deep tendon reflexes were hyperactive in the lower extremities (more prominent in the left side). Babinski’s sign was present on the left and a mild muscle stiffness on her left lower extremity (compatible with spasticity) was present. Vital signs revealed mild hypertension. Laboratory investigations including hemogram, biochemistry, lipid profile, thyroid, b12, and folic acid were in normal ranges. She scored 22 on mini-mental state examination. Cranial MRI showed bilateral, multiple, T2 hyperintense lesions in the periventricular areas, subcortical white matter, and basal ganglia (Fig. 1). Clinical history (acute onset and lack of prodromal symptoms of PD) as well as neurological examination (absence of extrapyramidal signs in the upper extremity) was atypical for PD. Cranial MRI had not showed the imaging sings of marked enlarged lateral ventricles, disproportional size of basal, Sylvian fussires, etc. rather excluding NPH. Besides, bradykinesia in the lower extremities were apparent and the clinical onset was characterized by an insidious progression following rather an abrupt onset manifesting with an ischemic stroke which also ruled out primary lateral sclerosis. Taken together, the diagnosis of vascular parkinsonism (VP) was established and levodopa/benserazide (100/ 25 mg) tb was initiated (gradually increased from 4 × 1/4 tb to 4 × 2 tb). A significant improvement in her walking speed and step width was achieved at 4 × 1 tb dosages. A mild, further improvement was observed following increment to the 4 × 2 tb dosages (Video 2). Further etiological investigations of the vascular lesions including CT angiography of the neck and brain, echocardiography and electrocardiography were in normal ranges. Susceptibility weighted imaging showed millimetric, hypointense lesions prominently in the bilateral basal ganglia and brain stem which was compatible with microhemorrhages (Figs. 1 and 2). The patient was diagnosed as VP in the setting of hypertension-related cerebral small-vessel damage and she was discharged on treatments of 4 × 200/50 mg levodopa/benserazide and 5 mg amlodipine for hypertension.

White matter changes in corpus callosum in a patient with idiopathic normal pressure hydrocephalus

altering pressure dynamic in ventricular systems.[4] Furthermore, Lane et al. emphasized that they had not observed any of the callosal signal changes in patients with communicating forms of hydrocephalus.[3] Hence, I think that while associating the callosal hyperintensity with ventriculoperitoneal (VP) shunting in a patient with NPH, a meticulous evaluation needs to be conducted. Based on the presentation of this case, the diagnosis of toxic encephalopathy associated with drug overdosage and related callosal changes cannot be excluded. Remarkably, diffusion‐weighted imaging (DWI) data, which is a crucial tool for understanding the nature of lesion of corpus callosum,[5,6] were not mentioned in the report constituting a major limitation. For instance, high signal in DWI might rather suggest an underlying toxic encephalopathy.[6]

Detailed clinical and electrophysiological illustration of a patient with sturge–weber syndrome presenting with prolonged transient neurological symptoms

Although transient neurological deficits in Sturge–Weber syndrome (SWS) have been acknowledged for many years, the underlying pathogenesis still constitutes a major topic of discussion. Here, we present the case of a 10-year-old boy with SWS presenting with a progressive clinically mimicking right middle cerebral artery syndrome. Cranial magnetic resonance imaging showed unremarkable findings except extensive right hemisphere leptomeningeal angiomas. Antiepileptic drug (AED) treatments provided recovery of the patient, and concurrently recorded electroencephalography (EEG) revealed delta slowing of the lesional hemisphere in the acute period. However, slowing of the background activity extended to the contralateral hemisphere in subacute period, while ipsilateral background activity started to recover. In the 3rd week of follow-up on intensive AED treatments, the patient totally recovered as well as nearly the normalization of EEG was achieved. In this report, we focus on the dramatic revolution of EEG activity in both hemispheres during the recovery period. Based on this rare illustration and limited literature data, the author suggests some hypotheses regarding the pathophysiology of this mysterious manifestation (transient neurological deficit) in SWS. Future studies of large case series, including detailed paraclinical parameters and remarkably electrophysiological data, are warranted to clarify these arguments.

Serial diffusion-weighted imaging in transient global amnesia?

166 Journal of Neurosciences in Rural Practice ¦ Volume 9 ¦ Issue 1 ¦ January-March 2018 to other mechanisms and origins such as neoplastic, infectious, toxic‐metabolic, ictus‐related changes. Hence, considering the controversies in this area, evaluation of serial imaging changes in diffusion‐restricted regions may be a reasonable way of understanding the responsible mechanism. However, the authors did not make any explanation about the possible mechanism underlying this completely normalization of DWI abnormality?[1] For instance, in ischemia model, we would expect to see the effects of pseudonormalization (baseline apparent diffusion coefficient values and hyperintense DWI) in the 2nd week of the clinical presentation (corresponding to the time of second MRI). Technical problems may also be a possible explanation which might be clarified by a third MRI follow‐up? I wonder if the authors may also include the T2‐weighted images (if present) for a better evaluation of the underlying pathophysiology. I would also like to remark that the DWI sections, illustrated in the report (DWI at presentation and 10 days after), are not similar which complicates a proper interpretation of the lesion evolution. Of note, the proper demonstration of the follow‐up image is also important to distinguish the initial lesions from possible DWI artifacts. Future reports of larger case series including follow‐up imaging results may provide crucial contributions regarding the unknown aspects of TGA pathophysiology.

Dramatic improvement of impulsive aggressive behaviour following shunt surgery in a patient with idiopathic normal pressure hydrocephalus

To the Editor A-67-year old male patent patient applied to the emergency department with a clinical complaint of superficial facial laceration following a fall during walking. Not a neurological deterioration according to previous status was determined. However, broad-based gait was apparent (Video 1). Upon interrogation, it was learnt that walking disturbance had started and deteriorated over the last 3 years. In addition, complaints of urinary incontinence (in the form of neurogenic) had started and mild deterioration of cognitive status was found during evaluation. Besides, relatives of the patent suffered from significant impulsive aggressive behavior changes. He had been displaying verbally as well as physically aggressive attitude towards his wife, and impulsive content was apparent in the patient’s manner over the last 2 years. There was no family history of mental illness, substance abuse, or suicide. Other medical history was also unremarkable. Due to these psychiatric symptoms, they had admitted to psychiatry polyclinics multiple times and ketiapine (25 mg) was started which had failed to provide any improvement. Of note, they had not applied to a neurology evaluation, previously. After first evaluation in the emergency department, based on the classical triad of gait disturbances, dementia, and urinary incontinence, a provisional diagnosis of normal pressure hydrocephalus (NPH) was considered in the forefront. Besides, the patient’s pattern of behaviour was characterized by little forethought and considerably informal that during the physical examination the patient tried to pull me by the nape of my neck, several times. Cranial magnetic resonance imaging (MRI) revealed enlarged lateral and third ventricle out of proportion to the cortical sulcal enlargement (supporting the diagnosis of idiopathic normal pressure hydrocephalus (INPH)) and also accompanying encephalomalacia in the left frontal lobe (Fig. 1). The patient scored 22 points on the Mini-Mental Status Exam (MMSE), losing 4 points for orientation, 2 points for recall, and 2 points for language. In addition, the patient could remember digits forwards and only one digit backwards. He got one point from clock drawing test. Hence, CSF tap test was performed. Opening pressure was evaluated as 160 mm H2O, and 40 cc CSF was drained which led to a moderate improvement in the patient’s gait disturbance. The patient referred to the shunt operation. After the ventriculoperitoenal shunt procedure, on the first month of follow-up, a moderate response in gait and urinary incontinence was achieved (Video 2). However, more dramatic was that his impulsive and aggressive behaviour was totally recovered which was also realized by his relatives since soon after the shunt procedure. During polyclinic evaluation, the behaviour and speech content was found to be considerably much more in a formal style which was in accordance with his premorbid status. Electronic supplementary material The online version of this article (doi:10.1007/s10072-017-3015-5) contains supplementary material, which is available to authorized users.

Post-stroke totally recovery of tremor in a patient with Parkinson’s disease

Pallidotomy remains as a treatment option in Parkinson’s disease (PD) with unilateral disabling dyskinesia and tremor, though deep brain stimulation of GPi and the other targets largely replaced ablative surgeries because of reversibility. Here, we present an illustrative rare case, a 65-year-old man with PD, at whom his unilateral parkinsonian tremor was totally recovered after stroke limited to lentiform nucleus.

Hemichorea-hemiballismus in the setting of posterolateral putaminal lesion and treatment with topiramate

A-60 year old female with medical history of hypertension, diabetes mellitus (with good glycemic control) and hyperlipidemia suffered from right sided involuntary movements of upper and lower extremities progressing over the past 4 years (Video 1). She had had an ischemic stroke characterized with right sided weakness 8 years ago. Motor examinations revealed 4+/5 motor weakness solely in the right triceps muscle and her walking was antalgic due to gonarthrosis. Cranial MRI showed chronic ischemic lesion in the left thalamic and posterior putamen (Fig. 1). With the diagnosis of vascular hemichorea hemiballismus (HC/HB), oral haloperidol was started up to the 2 × 5 mg dosage. However, on the second week evaluation on haloperidol treatment, not any improvement was achieved. Hence, haloperidol was discontinued and oral topiramate was started (up to 2 × 50 mg). On follow-up evaluation, three weeks later, a moderate response was achieved which was prominent in the lower extremity movements (Video 2).