Vladimir Miletić

Cerebral sinuvenous thrombosis: a rare complication of Lyme neuroborreliosis.

SummaryAssociation between neuroborreliosis and cerebral sinuvenous thrombosis is rare, and it can be made only when other, more common predisposing conditions are excluded. In the case of increased intracranial pressure and confirmed neuroborreliosis, early magnetic resonance venography and combination of antibacterial therapy with anticoagulation provide better long-term outcome. We present a case of a patient with cerebral sinuvenous thrombosis who was first treated for neuroborreliosis.ZusammenfassungEine Verbindung von Neuroborreliose und Thrombose der zerebralen Blutleiter ist selten und kann erst dann gemacht werden, wenn andere prädisponierende Ursachen ausgeschlossen worden sind. Bei erhöhtem inkraniellem Druck und bestätigter Neuroborreliose führen eine frühe MRI-Venographie und die Kombination von Antibiotika und Antikoagulantien zu besseren langfristigen Ergebnissen. Wir berichten über einen Patienten mit Thrombose der zerebralen Blutleiter, der anfänglich wegen Neuroborreliose behandelt worden war.

Acute Pisa Syndrome as a Neurological Emergency

To the Editor: Pisa syndrome is a rare clinical entity usually associated with underlying neurodegenerative diseases such as Parkinson’s disease and multiple system atrophy. However, it can also occur as an adverse effect of treatment with antipsychotics, especially in patients with predefined risk factors. Pisa syndrome is defined as sustained lateral flexion of the trunk.When observed in practice, it denotes an incapacitating symptom of underlying neurodegenerative diseases such as Parkinson’s disease and multiple system atrophy. Pisa syndrome can also occur as an adverse effect of prolonged treatment with antipsychotics, especially typical agents. However, reports describing acute drug-induced Pisa syndrome with possible treatment modalities are lacking. Here we report a patient with Alzheimer’s disease with severe risperidoneinduced acute Pisa syndrome in the neurological emergency room,whowas successfully treatedwith low-dose quetiapine.

Parkinsonian syndrome and ataxia as a presenting finding of acquired hepatocerebral degeneration

The term “acquired hepatocerebral degeneration” (AHD) was coined to describe clinical entity distinct from genetically defined Wilson’s disease. AHD is chronic neurological disorder, characterized by extrapyramidal and neuropsychiatric symptoms accompanied with advanced liver disease with portosystemic shunts. In majority of AHD cases, extrapyramidal symptoms appear in the presence of known liver disease. Here we present a patient with subacute onset of bilateral, asymmetric, hypokinetic-rigid syndrome and ataxia as initial presentation of liver cirrhosis. Manganese toxicity have major role in AHD pathogenesis. Failure of liver detoxification and presence of portosystemic shunting enables neurotoxic substance of manganese to avoid hepatic metabolism and to enter and accumulate in central nervous system. Predilection brain regions for manganese deposits are globus pallidum (GP) and substantia nigra (SN). Characteristic MRI findings of bilateral, symmetrical hyperintensities of GP and SN on T1-weighted sequences supported the diagnosis of AHD in our patient. In addition, increased T2 signal in dendate nuclei seen in our patient is rare radiological finding. So far, no consensus guidelines regarding medical treatment of AHD exist. We initiated low-dose levodopa treatment, but failed to provide beneficial effect. In conclusion, AHD is distinct clinical entity that should be included in differential diagnosis of both typical and atypical parkinsonian syndromes. Furthermore, our case highlights the importance of performing MRI in patients with atypical parkinsonism.

Hemidystonia caused by frontal cortical infarction.

A 71-year-old woman was examined in the emergency room because of motor dysphasia, right-sided supranuclear facial palsy and moderate right-sided hemiparesis (3/5) that occurred 5 h pri or to admission. Her past medical history included poorly controlled hypertension, dyslipidemia, atrial fibrillation, ischemic stroke with leftsided hemiparesis and complete resolution of neurological deficit afterward, breast cancer for which she had undergone right mastectomy, chemotherapy and radiation therapy. There was no history of diabetes mellitus, no prior exposure to neuroleptics or recent change in therapy. Initial brain computed tomography (CT) scan showed no responsible lesion. Chest radiography was normal, and atrial fibrillation was observed on electrocardiogram. Routine laboratory investigations including glucose and glycated hemoglobin were normal. 18 h after stroke onset, the patient developed involuntary, patterned, hemidystonic movements of the right extremities characterized by shoulder abduction, flexion at the elbow, knee flexion and foot dorsiflexion (video). During this episode, the patient was conscious and responsive. Brain CT scan was repeated and revealed ischemic lesion

Preserved ability to cycle in patient with progressive supranuclear palsy-parkinsonism.

Differentiation between Parkinson’s disease (PD) and atypical parkinsonism still presents an open issue. Although many attempts have been made to identify specific clinical tests to discriminate the two, making the right diagnosis is challenging [1]. Freezing of gait (FOG) is common phenomenon in patients with PD and various atypical parkinsonism. However, despite severe FOG, patients with PD retain ability to cycle in contrast to patients with atypical parkinsonism [2]. Hence, the ‘‘bicycle sign’’ or loss of cycling abilities was recently suggested as a new red flag and highly specific sign indicative for atypical parkinsonism [3]. Here, we report a patient with progressive supranuclear palsy–parkinsonism (PSP-P) phenotype with maintained and prolonged ability to ride a bicycle. A 57-year-old man was admitted to our movement disorders unit in 2012 because of two-year history of generalized slowness and difficulties in gait initiation associated with sudden stops while walking, but without falling. Still, he was able to immaculately ride a bike every day. There were no signs of autonomic dysfunction, such as erectile dysfunction, orthostatic hypotension, and urinary incontinence. His past medical history included appendectomy, arterial hypertension, and chronic gastritis. There was no prior history of carbon monoxide poisoning, CNS infection, neuroleptic, and toxin exposure. He denied weight loss, respiratory dysfunction, and the presence of any neurological diseases in his family. During initial neurological examination he was alert and fully orientated, scoring 29/30 on Mini–Mental State Examination. He had hypomimia, hypophonic and festinating speech, asymmetric bilateral rigidity more expressed on his left extremities, and there was no tremor. When performing finger-tap test, movements of low amplitude and high frequency without decrement were observed. The patient had difficulties in gait initiation with FOG, and was unable to maintain balance on retropulsion test. The rest of neurological examination was normal. Laboratory tests, including red and white blood cell count, erythrocyte sedimentation rate, glucose, electrolyte panel, urea, creatinine, liver enzymes, coagulation profile, vitamin B12, and folic acid, were all within normal range. Brain magnetic resonance imaging (MRI) showed mildly dilated ventricular system which was in correlation with brain parenchyma atrophy. Tilt-up table test was unremarkable. Scintigraphic assessment of postganglionic sympathetic cardiac innervation with iodine-131 metaidobenzylguanidine (MIBG) was normal (heart/mediastinum ratio [2). Single photon emission computerized tomography of dopamine system of the brain with 123I-ioflupan (DATSCAN) showed severe bilateral functional impairment of striatal dopamine system. Testing for Niemann Pick disease type C was negative and a diagnosis of PSP-parkinsonism was established. Treatment with levodopa and amantadine was initiated, but provided mild and transient benefit. During four-year follow-up, gait disturbances progressed. The patient reported up to three falls per month and required walking assistance. However, ability to cycle was maintained (supplementary video file Electronic supplementary material The online version of this article (doi:10.1007/s10072-016-2735-2) contains supplementary material, which is available to authorized users.

Author’s Reply: Hemidystonia caused by frontal cortical infarction

We read with interest the comments of Prof. Garraux on our recent case report describing a 71-year-old woman presenting with right-sided hemidystonia following an ischemic stroke. The initial title of our paper was: ‘‘Hemidystonia caused by a frontoparietal cortical infarction’’. However, we now receive the comment that according to the published computed tomography axial images of the brain, the causal lesion is located in the postcentral gyrus of the left parietal lobe. Indeed, we must agree that the lesion shown in image 1A of the manuscript is located in the left postcentral gyrus of the parietal lobe. Compliance with ethical standards

Bilateral middle cranial fossa arachnoidal cysts with temporal lobe agenesis associated with astrocytoma: fortuitous finding.

Previously healthy, 36-year-old professional soldier presented with left-sided partial motor epileptic seizure with secondary generalisation. He denied alcohol consumption, any drug abuse, head trauma, recent infections and headaches. Initial examination was unremarkable and routine laboratory investigations were within normal range. Brain magnetic resonance imaging (MRI) showed large bilateral middle cranial fossa arachnoid cysts with compressive effect on adjacent neural structures, associated with infiltrative process in medial part of the right frontal lobe that affects cerebral cortex, subcortical and deep white matter, and spreads over the anterior part of the corpus callosum to the left side (Fig. 1). In addition, brain single-photon emission computed tomography (SPECT) with Tc-99m ECD was performed and showed diffuse areas of hypoperfusion in both frontal lobes together with the absence of radionuclide accumulation in both temporal lobes (Fig. 2). After stereotactic biopsy and pathohistological confirmation of anaplastic astrocytoma (WHO grade III), chemotherapy with lomustine (CCNU) and vincristine was started, followed by radiotherapy. In 1964, Robinson described ten cases with unilateral temporal lobe defects and coined the term ‘temporal lobe agenesis syndrome’ [1]. Bilateral temporal lobe agenesis associated with middle cranial fossa arachnoid cysts, on the other hand, is exceedingly a rare condition and only few cases have been reported so far [2, 3]. Aetiology of this syndrome is still a controversial subject. Original theory proposes that middle cranial fossa cysts represent a passive collection of cerebrospinal fluid related to temporal lobe agenesis [1], while second theory considers temporal lobe defects as a consequence of compression of arachnoid cysts as primary developmental malformation of the arachnoid membrane [4]. Most cases of middle cranial fossa cysts are asymptomatic. Some patients present with nonspecific symptoms and signs, including signs of elevated intracranial pressure, seizures, calvarial bulging, and focal neurological signs, while vision and hearing abnormalities, and behavioural changes are less common. In a recent retrospective study that included 48,417 patients who underwent brain MRI, middle cranial fossa arachnoid cysts were found in 237 patients (0.005 %); yet, cysts were considered to be symptomatic in only five patients [5]. In conclusion, although being impressive radiological finding, bilateral temporal lobe agenesis did not seem to have an impact on our patient’s physical and intellectual development and would probably remain unrevealed if the symptoms of associated malignancy did not occur. B. Radić (&) Department of Neurology, Outpatient Clinic, University Hospital Centre Zagreb, Kišpatićeva 12, 10000 Zagreb, Croatia e-mail: boris.radic105@gmail.com