Objective: To describe a patient with a massive and widespread tauopathy who presented with an atypical speech disturbance and MRI findings along 6 years of follow-up. Background The spectrum of frontotemporal dementia is characterized by distinct behavioural changes and aphasia, either non-fluent/agrammatic, semantic, or logopenic together with neuroimaging evidence of different patterns of atrophy related to the underlying pathology. Design/Methods: A 46-year-old woman presented with a 2-year history of progressive personality change. Having been an active, cheerful and empathic woman she started having problems with work colleagues and turned apathetic. Her speech became slow and aprosodic, somehow robotic (see video), without aphasic features. She had a healthy twin sister and another older sister diagnosed of schizophrenia. She progressively worsened, showing a childish and disinhibited behaviour. She never suffered hallucinations, autonomic dysfunction or parkinsonism. She died by accident 6 years after disease onset. Results: She did not developed any of the typical patterns of aphasia described in frontotemporal dementia . Brain MRI showed subcortical hyperintensities involving both insulae, that later spread into both frontal cortices. No gross atrophy was detected. Tests for progranulin, prion protein, CADASIL, sequentiation of the MAPT gene and presenilin-1 were negative. The neuropathological study showed a massive neuronal loss in the frontal cortex with deposits of 4R-Tau in the frontal and insular cortex, striatum, amygdala, hippocampus, entorhinal cortex, hypothalamus, thalamus, locus coeruleus and cerebellum. Deposits were predominantly intracytoplasmatic. There was some scattered deposition of TDP-43 and complete lack of amyloid and alpha-synuclein. Conclusions: Aprosodic speech without aphasia and insular hyperintensities add to the unusual manifestations of frontotemporal dementia. This histologically proven case adds to our knowledge of frontotemporal dementia and also reinforces the crucial role of the insula for the development of the behavioural and speech symptoms in frontotemporal dementia. Disclosure: Dr. Gomez Beldarrain has nothing to disclose. Dr. Ruiz Ojeda has nothing to disclose. Dr. Ferrer Abizanda has nothing to disclose. Dr. Cabrera has nothing to disclose. Dr. Garcia Monco has nothing to disclose.
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Spinal cord hematoma is a serious and feared complication of lumbar puncture. We here describe two patients who developed a spinal cord hematoma following diagnostic lumbar punctures.CASE 1: a 22-year-old male with a syndrome of cerebrospinal fluid hypotension, with normal coagulation parameters, underwent a traumatic, diagnostic lumbar puncture followed, a few hours later, by back pain irradiated to the legs. MRI showed the presence of a subdural hematoma from the lower dorsal region to the sacral region. A conservative approach, without surgery, was decided and he showed a complete recovery. CASE 2: a 69-year-old woman underwent a diagnostic lumbar puncture for the study of recent-onset headache with tinnitus and unstable gait. Puncture was traumatic and cerebrospinal fluid was normal. A few hours later, she complained of back pain and sciatica, and examination revealed a bilateral Lassegue sign. She required urinary catheterization. MRI showed an epidural hematoma from the T10 vertebra to the sacral area. Her outcome with a conservative approach was also excellent.Spinal cord hematomas can occur after a traumatic lumbar puncture in people without coagulation disorders or any other predisposing factor. Although surgery has been traditionally advocated in these cases, a conservative approach is an option when symptoms are mild and a close follow-up is possible.