Dilaver Kaya

Diffusion tensor imaging of Guillain-Mollaret triangle in patients with hypertrophic olivary degeneration.

The aim of the study is to analyze diffusion tensor imaging (DTI) characteristics of the Guillain‐Mollaret triangle (GMT) in patients with hypertrophic olivary degeneration (HOD) and to investigate their correlation with previously reported histopathology. DTI was performed in 10 patients diagnosed with HOD. Fractional anisotropy, apparent diffusion coefficient, axial diffusivity, and radial diffusivity were measured in the inferior olivary nucleus (IO), the central tegmental tract, the red and the dentate nuclei, and the superior cerebellar peduncle of HOD patients and compared to age, sex, and side‐matched 10 neurologically normal population. The prominent finding on DTI in affected IO was an increase in radial diffusivity compatible with demyelination. While conventional magnetic resonance imaging did not show any sign of involvement in the other components of GMT, DTI demonstrated signal changes in all anatomical components of the GMT. Main DTI findings in GMT of patients with HOD were an increase in radial diffusivity representing demyelination and an increase in axial diffusivity that is reflective of neuronal hypertrophy. DTI parameters can reflect the spatiotemporal evolution of transneuronal degeneration associated with HOD in a manner consistent with the known pathologic stages of HOD.

CACNA1H antibodies associated with headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL).

Background Patients with the syndrome of headache with neurological deficits and lymphocytosis (HaNDL) typically present with recurrent and temporary attacks of neurological symptoms and cerebrospinal fluid lymphocytosis. Aim and methods To identify potential HaNDL‐associated antibodies directed against neuronal surface and/or synapse antigens, sera of four HaNDL patients and controls were screened with indirect immunohistochemistry, immunofluorescence, cell-based assay, radioimmunoassay, protein macroarray and enzyme-linked immunosorbent assay (ELISA). Results Although HaNDL sera did not yield antibodies to any of the well-characterized neuronal surface or synapse antigens, protein macroarray and ELISA studies showed high-titer antibodies to a subunit of the T-type voltage-gated calcium channel (VGCC), CACNA1H, in sera of two HaNDL patients. Conclusion Our results support the notion that ion channel autoimmunity might at least partially contribute to HaNDL pathogenesis and occurrence of neurological symptoms.

Aortic atheromas in stroke subgroups detected by multidetector computed tomographic angiography

BACKGROUND The aim of this study was to investigate aortic atheromas in stroke subgroups. METHODS Two hundred consecutive subjects had acute ischemic stroke confirmed by diffusion-weighted imaging (DWI) (195 cases) or computerized tomography (5 cases). Multidetector computed tomographic angiography (MDCTA) (16- or 64-slice) was used to detect atherosclerotic plaques in vessels. Patient data and diagnostic test results were recorded. Stroke subgroups (TOAST classification) were compared with respect to plaque features in the ascending aorta or aortic arch such as presence of at least 1 plaque, larger than 1mm thick, multiple plaques, and plaque morphology (calcific, soft, mixed and ulcerated). RESULTS Of the patients, 20.3% were in the large-artery atherosclerosis (LAA), 29.4% had small artery occlusion (SAO), 23.8% had cardioembolism (CE), 6.6% had more than one potential cause found (MPC) and 19.8% had cryptogenic stroke (CS). Overall, 49.7% of patients had at least 1 plaque (any size) in the ascending aorta or aortic arch. The corresponding rates for subgroups were as follows: LAA 80%, SAO 50%, CE 44.7%, MPC 61.5% and CS 20.5% (p<0.001). Subgroups also differed significantly with respect to presence of multiple plaques and plaques>1mm thick. Of all plaques 93% were mixed type, of which 19% were ulcerated. CONCLUSIONS Almost half of the stroke cases had atheroma in ascending aorta or aortic arch and most of them had a soft component. Subgroups LAA, SAO, and MPC had higher aortic atheroma density compared to CE and CS.

Anti-neuronal antibodies associated with headache with neurological deficits and cerebrospinal fluid lymphocytosis

Infectious-immunogenic mechanisms have been proposedfor the pathogenesis of the syndrome of transient headacheand neurological deficits with cerebrospinal fluid lympho-cytosis (HaNDL) [1]. To investigate this assumption, weperformed immunohistochemistry and Western blot studieswith sera and cerebrospinal fluid (CSF) of two HaNDLpatients. Serum and CSF IgGs of both patients showedstrong reactivity with the nuclei of rat central nervoussystem (CNS) cells and proteins extracted from neuro-blastoma cells. This reactivity was not observed withsamples of a sporadic hemiplegic migraine (SHM) patientand 12 migraine patients used as controls. The CNSbinding pattern of HaNDL IgG was easily distinguishablefrom that of other well-known anti-neuronal nuclear anti-bodies (ANNA) such as anti-Hu, anti-Ri, and anti-nuclearantibody (ANA). The presence of an anti-neuronal immuneresponse may support the autoimmune hypothesis as apathogenic factor for HaNDL and should be furtherinvestigated in larger series.To show the presence of a possible autoimmuneresponse in this syndrome, sera and CSF of two HaNDLpatients were investigated for anti-neuronal antibodies.Serum/CSF of a SHM patient, sera from 12 migraine, 2anti-Hu, 2 anti-Ri, 34 ANA positive patients and 30 healthyindividuals were used as controls. All headache patientsfulfilled the clinical criteria established by The Interna-tional Headache Society [2].Transient headache and neurological deficits with cere-brospinalfluidlymphocytosispatientswere35-(H1)and38-year-old (H2) men presenting with three episodes of throb-bing headache, dysphasia/dysarthria, marching righthemiparesis and hemihypoesthesia (H1 only) lasting for 2–9 h. Their CSF revealed increased lymphocytes [496/mm

Serial EEG and MRI changes in status epilepticus-induced excitotoxic neuronal necrosis

Prolonged status epilepticus may directly cause selective neuronal necrosis due to excitotoxic mechanisms, as observed in experimental models and described in case reports. A 36-year-oldwoman presented with right hemiplegia and aphasia following a generalised tonic-clonic status epilepticus of two hours duration. Accompanying serial MRI with advanced imaging techniques, EEG and histopathology of the cortical tissue of the patient were all compatible with excitotoxic neuronal necrosis. In this histopathologically-proven rare case of status epilepticus-induced excitotoxic neuronal injury, the observation of delayed cortical laminar necrosis on MRI, together with paroxysmal lateralised epileptiform discharges on the EEG, suggests that these changes may be an early sign of impending and ongoing excitotoxic neuronal injury and delayed cell death caused by glutamate release due to excessive neuronal firing in status epilepticus.

Tuberculous meningitis presenting with nonconvulsive status epilepticus.

Nonconvulsive status epilepticus (NCSE) is an enduring epileptic condition characterized by alteration in consciousness and continuous ictal discharges on the EEG. Various etiologies have been reported. We describe the case of a 66-year-old woman with altered mental status who was diagnosed with NCSE. A workup to explain the etiology revealed tuberculous meningitis (TBM) with increased cerebrospinal fluid protein and positive tuberculous DNA polymerase chain reaction and interferon-γ assay tests. She was treated according to the status epilepticus protocol with a four-drug anti-tuberculosis regimen to which she responded. TBM is a serious disease with insidious presentation and still constitutes a diagnostic challenge with its various presentations. Among the many presentations of tuberculosis, clinicians should consider NCSE.

Ischemic involvement of the primary motor cortex is a prognostic factor in acute stroke

Background The location of the primary motor cortex can be detected in healthy adults using the findings of ‘T2 hypointensity’ and the ‘double layer sign’ on 3 T diffusion-weighted imaging. The aim of this study was to assess whether ischemic involvement of the primary motor cortex can be identified on 3 T diffusion-weighted imaging within six-hours after stroke onset and to evaluate whether this finding could predict clinical outcome three-months after ischemic stroke. Methods Sixty-five patients who had paralysis and ischemia of the anterior circulation underwent 3 T magnetic resonance imaging within six-hours of symptom onset. Follow-up MRI was obtained at 72 h. Anatomic localization and ischemic involvement of the primary motor cortex were evaluated on diffusion-weighted imaging by two investigators. Ischemic involvement on the primary motor cortex was classified into three grades. Ischemic lesion volumes were measured. We compared the favorable outcomes at three-months between subjects with and without ischemic involvement on the primary motor cortex using the NIHSS and modified Rankin Scale. Results Ischemic involvement on the primary motor cortex was identified in 52% of patients. Interrater agreement coefficients were 0·93 for the identification of ischemic involvement of primary motor cortex. As defined by scores on the modified Rankin Scale, among the patients with ischemic involvement of the primary motor cortex were worse than the patients without ischemic involvement of the primary motor cortex (P = 0·01). The mean ischemic lesion volume at baseline diffusion-weighted imaging was 38·7 ± 41·7 cm3 and was 89·8 ± 93·6 cm3 at follow-up T2-WI. Ischemic involvement on the primary motor cortex (odds ratio: 14·7) was a determinant for worse outcome. Conclusions 3T diffusion-weighted imaging can identify ischemic involvement on the primary motor cortex and may provide useful information for predicting outcome during the hyperacute stage. Ischemic involvement on the primary motor cortex has a significant negative impact on recovery.

A new differential diagnosis for acquired demyelinating neuropathy: copper deficiency.

Sir, Copper is a very important trace element. It is required for basic cell functioning and is a cofactor in a number vital enzymes necessary for the nervous system.[1,2] The first case of copper deficiency‐related myelopathy in humans was reported in 2001.[3‐5] Clinically, a presentation similar to B12 deficiency myelopathy has been described, with neurological examination findings suggestive of pyramidal and posterior column abnormalities. Commonly, spinal cord magnetic resonance imaging (MRI) studies display abnormal T2 signals in the posterior cord and EMG studies demonstrate an axonal peripheral neuropathy. We report a case of acquired demyelinating neuropathy due to copper deficiency.

A rare case of multiple sclerosis and cerebral hemorrhage associated with osteopetrosis.

Osteopetrosis, or Albers-Schönberg disease, is a rare hereditary disease characterized by osteoclast dysfunction and consequent diminished bone resorption and disturbed bone building and remodeling, resulting in abnormally dense and brittle bones. Bone marrow failure, pathologic fractures, and neurologic deficits are common. Osteopetrosis is diagnosed on radiographs. Patients have generalized osteosclerosis, and radiographs may show evidence of fractures. We report a case of cerebral hemorrhage and multiple sclerosis associated with the benign adult form of osteopetrosis.