Myelitis and Polyradiculoneuropathy With Severe Pain: Unusual Neurological Manifestations as Presenting Symptoms of Brucellosis.

Brucellosis, an endemic zoonosis in Portugal, is a multisystem disease, presenting with neurological manifestations in up to 25% of cases. Neurobrucellosis diagnostic criteria include evidence of central nervous system invasion, either by documenting increased blood-brain barrier permeability that normalizes after treatment or by Brucella isolation. We report 2 patients with systemic brucellosis presenting with neurological symptoms: A 28-year-old female with progressive hemiparesis associated with severe refractory thoracic and lumbar pain, whose spinal magnetic resonance imaging identified longitudinally extensive myelitis. Brucella agglutination test was positive in blood; however, cerebrospinal fluid cytochemical, serological testing, and cultures were negative. A 58-year-old male with intermittent fever in the evening, associated with severe refractory cervical and lumbar spinal and radicular pain. Blood workup identified leukocytosis, elevated inflammatory markers and positive Brucella agglutination test. Cerebrospinal fluid presented mild protein increase and negative serological testing and cultures. Electromyogram revealed demyelinating polyradiculoneuropathy. In both cases, antibiotic therapy induced symptom resolution. Despite the neurological presentation, no evidence of direct nervous system infection was found. An indirect mechanism appears to be involved, such as a parainfectious syndrome or circulating endotoxins release by the bacteria. Brucellosis should be considered in patients presenting with inflammatory neurological symptoms in endemic regions. Prompt diagnosis and treatment are important as chronic infection has significant morbidity.

Encephalopathy Associated with Autoimmune Thyroid Disease: A Potentially Reversible Condition.

Autoimmune thyroid disease may occasionally associate with unspecific neurological symptoms, which are more commonly insidious, include cognitive or behavioural symptoms, and may associate with tremor, myoclonus, or ataxia. We report a 61-year-old female patient who presented with chronic headache, insidious mood, and cognitive disturbance which evolved in a few months to dementia associated with exuberant limb myoclonus. Diagnostic workup revealed high anti-thyroid peroxidase antibody titers and an inflammatory CSF profile, and it was negative for other possible etiologies. Treatment with steroids induced significant improvement. The diagnosis of encephalopathy associated with autoimmune thyroid disease is still controversial given the fact that the clinical presentation and diagnostic workup are unspecific, the pathophysiology is still undetermined, and the diagnosis is mostly of exclusion. No direct correlation is found between anti-thyroid antibody titers and clinical presentation, and it is currently speculated that other still unrecognized antibodies may be responsible for this clinical entity. It is extremely important to recognize this entity because it is potentially treatable with immunotherapies. It is also increasingly recognized that clinical improvement with first-line treatment with steroids may be absent or incomplete, and other immunotherapies as immunosuppressants, intravenous immunoglobulin, or plasma exchange must be attempted in the clinical suspicion of EEAT.

Biparietal variant of Alzheimer's disease: a rare presentation of a common disease.

Alzheimer's disease (AD) is a clinically heterogeneous disease that may have atypical presentations with focal cortical syndromes and relatively preserved episodic memory. The posterior variant of AD has two subtypes: occipitotemporal, presenting with visuoperceptive impairment, and biparietal, presenting with visuospatial dysfunction and apraxia. We report a case of a 51-year-old woman with progressive limb apraxia and choreiform movements. Her neuropsychological evaluation was compatible with dementia, and revealed ideomotor and ideational limb apraxia, severe visuoconstructive ability impairment, dyscalculia and posterior aphasia. Workup excluded metabolic, infectious, inflammatory or neoplastic causes, and hereditary conditions as Huntington's disease and familial AD. Cerebrospinal fluid biomarkers revealed ß-amyloid reduction and τ protein increase. Brain imaging showed marked biparietal atrophy and hypoperfusion, and widespread cortical ß-amyloid deposition. Biparietal variant of AD was diagnosed and acetylcholinesterase inhibitor treatment induced clinical stabilisation. AD may present with atypical features and a high clinical suspicion is necessary for an early diagnosis.