Pantelis Maiovis

Primary antiphospholipid syndrome mimicking demyelinating disorders.

To the Editor: Primary antiphospholipid syndrome (PAPS) is characterized by arterial or venous thrombosis, spontaneous abortions, and the persistent presence of antiphospholipid antibodies, namely the lupus anticoagulant (LA), the anticardiolipin (aCL), and antib2 glycoprotein I (antib2GPI) antibodies. PAPS is a rare and controversial disorder with multiple neurological complications that can mimic various diseases, delaying the final correct diagnosis.

Autoimmune autonomic ganglionopathy after a CNS infection.

AbstractWe report the first Greek case of autoimmune autonomic ganglionopathy seropositive for antibodies against ganglionic acetylcholine receptors, unique for an antecedent viral cerebellitis and long, slowly progressive course, with improvement after treatment with pyridostigmine.

Cognitive Reserve Hypothesis in Frontotemporal Dementia: Evidence from a Brain SPECT Study in a Series of Greek Frontotemporal Dementia Patients

Background and Objective: Cognitive reserve (CR) mediates the clinical expression of brain pathology in Alzheimer’s disease, while there are much less relevant data in frontotemporal dementia (FTD). In the present study we examined whether CR, measured using the Cognitive Reserve Index (CRI), correlated with regional cerebral blood flow (rCBF) in Greek FTD patients. Methods: Eighty FTD patients, i.e., 47 with behavioral variant FTD (bvFTD) and 33 with primary progressive aphasia (PPA), were enrolled into this study. CR was assessed using the CRI questionnaire, which provides a total score (CRI) and 3 subscores, i.e., CRI-education, CRI-working activity, and CRI-leisure time. The FTD-Clinical Dementia Rating Scale was used to assess the severity of dementia and a brain SPECT study was performed to measure rCBF. Finally, multiple regression analyses were conducted to explore correlations between CR indices and frontotemporal rCBF. Results: In both the bvFTD and the PPA groups, higher scores in the CRI, CRI-education, and CRI-leisure time correlated with lower rCBF in the bilateral frontal and left temporal cortex, respectively, controlling for age, sex, time since symptom onset, and disease severity. Conclusion: In the present study, lifetime participation in leisure time activities was found to mitigate the burden of disease in bvFTD and PPA patients. Moreover, FTD patients with a higher educational attainment were able to cope better with greater brain damage. Determination of the most suitable activities to build an adequate level of CR is crucial for dementia prevention.

Sequential peripheral neuropathy and myelopathy in a neurosarcoidosis patient

A 66-year-old male was referred to our department for evaluation of severe quadriparesis along with marked sensory and autonomic dysfunction. His past medical history was remarkable only for a remote gastrectomy due to gastric ulcer. He was in his usual state of health until 18 months ago, when he noted numbness of the left hand and foot along with numbness and weakness of the right hand. These symptoms were gradually worsening, with numbness in all four limbs, gait imbalance and clumsiness of the right arm. Initial clinical and electrophysiological evaluation at another hospital suggested multiple root or plexus damage but MRI of the cervical spine and brachial plexus were unrevealing. Subsequent electrodiagnostic studies indicated a demyelinating polyneuropathy. The patient was treated with periodic infusions of intravenous immunoglobulin (IVIg), with little or no improvement. During the last 4 months the patient had been bedridden, with severe quadriparesis, numbness of the limbs and trunk, loss of sphincter control, dry mouth, dry eyes and episodic hyperidrosis. Neurologic examination on admission at our department revealed asymmetrical flaccid arm and leg weakness, a bilateral Babinski sign, a ‘gloves-stockings’ pattern of hypoesthesia with a sensory trunk level at Th9, severe proprioceptive loss especially in the legs and signs of autonomic dysfunction. Repeat nerve conduction velocity studies were consistent with the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) (Table 1), CSF contained 24 lymphocytes/mm with normal protein and glucose; CT scans of the chest, abdomen and pelvis were negative for tumor as was detailed autoimmune workup. Based on the signs implying a spinal cord lesion, the inflammatory CSF and lack of improvement with IVIg, a repeat MRI scan of the cervical spine revealed an intraspinal enhancing lesion extending from C2 to C6, as well as scattered enhancing lesions in the thoracic cord (Fig. 1a, b). The patient was treated with prednisolone 75 mg/day, with remarkable and immediate improvement of the weakness. Repeat MRI of the cervical and thoracic spine revealed diffuse areas of myelopathy mainly in the cervical level, with milder enhancement, no edema or mass effect (MRI of the brain was unremarkable). Repeat spinal tap confirmed mild inflammatory CSF with eight lymphocytes, normal protein and glucose, no oligoclonal bands, negative for infectious agents, mycobacteria, antitreponema antibodies or malignant cells. Nerve conduction studies were unchanged. A thorough review of the imaging studies identified small mediastinal lymphadenopathy as well as axillar lymphadenopathy, markedly on the right. A biopsy from the right axilla yielded several enlarged lymph nodes with histopathological features of chronic granulomatous inflammation, with few sites of necrosis. P. Maiovis (&) S. Michelaki P. Ioannidis D. Karacostas 2nd Department of Neurology, AHEPA University Hospital, St Kyriakidi 1, 54636 Thessaloniki, Greece e-mail: panmaio@hotmail.com

The Gilliatt-Sumner hand

A 37-year-old woman presented with progressive painless right hand weakness and paresthesias/numbness of the medial forearm. Examination revealed Gilliatt-Sumner hand (Fig. 1), a clinical sign indicative of thoracic outlet syndrome (TOS) [1]. Raising hand above the head and upper limb tension test [2] exacerbated sensory symptoms. Neurological examination revealed moderate muscle weakness (BMRC 3/5) in the adduction and abduction of right thumb and its opposition to the 5th digit, along with mild hyporeflexia of right finger jerk; mild hypesthesia of right hypothenar and 4–5th digit was detected. Nerve conduction studies of the affected (right) limb (Table 1) showed low median and ulnar CMAP amplitudes with normal median and low ulnar SNAP amplitudes; the corresponding values of the non-affected (left) limb were normal (Table 1). Right medial antebrachial cutaneous