Tag Archives: Infectious

Neonatal Myasthenia

Diagnosis: Myasthenia in a new born to a mother with myasthenia gravis AChR antibodies: positive Clinical features: Weakness, hypotonia, dysphagia, weak cry and suck, Edraphonium/Tensilon test positive Mother with myasthenia gravis Treatment: Neostigmine Rarely plasma exchange Related articles: Approach to weakness, neuromuscular disease patterns, neuromuscular junction disorders,

Poliomyelitis

Diagnosis: A combination of clinical features and CSF analysis Clinical features: Prodrome: upper respiratory tract infection Followed by: Mild meningismus +headache Myalgial, paraesthesia, paralysis and respiratory weakness After recovery LMN features and flaccid weakness. Autonomic features (swollen, clammy, cold, purple) in Lower limbs. Findings in Investigations: CSF: Pleocytosis Serology NCS/EMG: Asymmetrical involvement. Motor evoked potential … Continue reading Poliomyelitis

Isolated Facial palsy, CN VII

Upper motor neuron lesion: Diagnosis: This is a clinical diagnosis. The underlying cause is determined by investigations and clinical correlation. Clinical features: Sparing of the forehead muscles Asymmetry of face at rest or on movement: smile, puffing the cheeks and on wincing The palpebral fissure may be widened on the affected side Investigations to consider: … Continue reading Isolated Facial palsy, CN VII

Superior Orbital Fissure Syndrome

Synonyms: Anterior cavernous sinus syndrome, Rochon-Duvigneaud’s syndrome Diagnosis: This is a clinical diagnosis. Underlying causes are identified by investigations and clinical assessment. Dysfunction of cranial nerves oculomotor (III), trochlear (IV), abducens (VI) and first division of the trigeminal nerve (V1). Also sympathetic fibers may be involved causing a Horner syndrome (this becomes cavernous sinus syndrome). … Continue reading Superior Orbital Fissure Syndrome

Klüver-Bucy syndrome

Diagnosis: This is a clinical diagnosis Clinical features: Hypersexuality, hyperorality, visual agnosia, and placidity (diminished emotional reactions). Investigations to consider: MRI: Lesions in bilateral medial temporal lobes, e.g. Traumatic brain injury, herpes, other infections Consider tests for dementias (FTLD, Alzheimer’s) & infections Related articles: Traumatic brain injury, encephalitis, frontotemporal dementia, Alzheimer disease,

Dementia

Diagnosis: Neuropsychological/Psychometric testing Or Clinical features The underlying etiology is determined by a combination of clinical features, neuropsychological testing, imaging and laboratory testing   Clinical features: This is an acquired persistent disorder where there is an impairment of the content of consciousness (intellectual function) with compromise in at least 2, e.g.: Memory Language i.e. aphasia … Continue reading Dementia

Cerebral Edema

This is an abnormality that can occur due to multiple potential causes. It is treated based on the underlying cause and severity. Diagnosis: CT: Hypodensity Local effacement of sulci and ventricles and cisterns Blurring of the grey-white interface MRI: DWI: Allows differentiation between vasogenic (no abnormal diffusion restriction) vs. cytotoxic (abnormal diffusion restriction) types of … Continue reading Cerebral Edema

JC virus Granule Cell Neuronopathy (JCV GCN)

Diagnosis: Clinical features plus MRI and evidence of JC virus infection Clinical features: ataxia Findings on Investigations: MRI: Cerebellar atrophy with or without features of PML CSF: JC virus positive Pathology, biopsy: Cerebellum: focal Internal granule cell layer loss, some enlarged granule cell neurons Special studies: Immunohistochemistry for polyomavirus: positive In situ hybridization ISH for … Continue reading JC virus Granule Cell Neuronopathy (JCV GCN)

Progressive Multifocal Leukoencephalopathy (PML)

Diagnosis: Clinical and imaging features plus laboratory findings Clinical features: Gradual onset & progressive. Seizures & aphasia may occur Occurs in the setting of immunosupression: e.g. HIV infection/AIDS, immunosupressant medications, Findings on Investigations: JC virus in CSF: by PCR MRI: White matter lesions, usually asymmetric but bilateral, periventricular and subcortical involvement of U fibres, spares … Continue reading Progressive Multifocal Leukoencephalopathy (PML)

Variant Creudtzfedt-Jakob Disease (vCJD)

Synonyms: New variant CJD, Diagnosis: Clinical +/-EEG +/-MRI +/-CSF findings +/-brain biopsy Clinical features: Abnormal movements (myoclonus, dystonia, chorea), ataxia, slurred speech, tremor, dementia, akinetic mutism Non-specific sensory symptoms Psychiatric symptoms: withdrawal, delusions, depression Findings on Investigations: MRI: Thalamic hyperintensities: bilateral pulvinar (posterior thalamus) a.k.a. ‘Pulvinar sign’. In some there is also bilateral dorsomedial nucleus … Continue reading Variant Creudtzfedt-Jakob Disease (vCJD)