Tag Archives: Immune

Autoimmune Autonomic Ganglionopathy AAG

Synonyms: a.k.a. Pandysautonomia a.k.a. idiopathic autonomic neuropathy a.k.a. acute panautonomic neuropathy a.k.a. autoimmune autonomic neuropathy, rare: Diagnosis: Clinical features +Anti-ganglionic nAChR antibodies +supported by electrodiagnostic tests Clinical features: Failure of sympathetic (orthostatic hypotension, anhidrosis) and parasympathetic (dry eyes, dry mouth, gastroparesis, constipation, urinary retention) nervous system with relative sparing of somatic nerves Preceded by viral … Continue reading Autoimmune Autonomic Ganglionopathy AAG

Nonsystemic vasculitic neuropathy NSVN

Diagnosis: This is based on clinical features +nerve/muscle biopsy findings of vasculitis +exclusion of systemic causes Clinical features: Clinical features: usually subacute, painful, distal, asymmetric, Findings on investigations: +Nerve conduction studies +EMG: Axonal neuropathy +CSF analysis: increased protein +ESR: might be increased +ANCA negative Pathology, nerve biopsy: Sural nerve or superficial peroneal nerve biopsy +/-muscle … Continue reading Nonsystemic vasculitic neuropathy NSVN

Systemic Vasculitic Neuropathy

Diagnosis: The diagnosis is made based on clinical features +Peripheral neuropathy, +evidence of vasculitis, +evidence of other organ involvement Clinical features: acute, mononuritis multiplex or less commonly mononeuritis simplex or symetric polyneuropathy, sensorimotor +Peripheral neuropathy +evidence of vasculitis +evidence of other organ involvement Findings on investigations: +NCS/EMG: NCS: Axonal neuropathy Conduction block: often transient CMAP: … Continue reading Systemic Vasculitic Neuropathy

Anti-GALOP syndrome: Gait ataxia, autoantibody, late onset polyneuropathy

Synonyms: a.k.a. gait ataxia and polyneuropathy GAPN Diagnosis: Clinical features: Late onset 70 year olds, gait ataxia (wide based with falls) and polyneuropathy (sensorimotor, sensory>motor, impaired proprioception) Anti-GALOP (IgM against central myelin antigen a.k.a. galopin): positive, SPEP +IFE: monoclonal IgM Treatment: Intravenous immunoglobulin IVIG cyclophosphamide Related articles: Approach to weakness, neuromuscular disease patterns, polyneuropathy, Polyneuropathy … Continue reading Anti-GALOP syndrome: Gait ataxia, autoantibody, late onset polyneuropathy

Anti-MAG syndrome

Diagnosis: This is diagnosed by a combination of clinical features, NCS/EMG and antibodies Clinical features: Distal symmetric, sensorimotor (sensory> motor), Findings on investigations: Anti-MAG: positive, IgM against myelin associated glycoprotein MAG SPEP +IFE: IgM paraprotein, monoclonal NCS/EMG: Distal slowing Pathology/nerve biopsy: Demyelination, axonal degeneration IgM deposits at sites of MAG localisation Immunohistochemsitry: Immunofluorescence with Anti-immunoglobulin … Continue reading Anti-MAG syndrome

Polyeuropathy Associated with Paraproteinemia

Diagnosis: This is a category of different neuropathies. Neuropathy due to a gammopathy includes anti-MAG syndrome, POEMS, cryoglobulinemia, IgM MGUS polyneuropathy, Anti-GALOP (IgM against central myelin antigen): Gait Disorder, Autoantibody Late-age Onset Polyneuropathy Evidence of paraproteinemia e.g. SPEP: paraproteinemia Pathology, nerve biopsy: Light microscopy: Nonspecific, loss of myelin and/or axons No inflammation except in cryoglobulinemia … Continue reading Polyeuropathy Associated with Paraproteinemia

Idiopathic Lumbosacral Plexitis

Synonyms: Idiopathic Lumbosacral plexitis a.k.a. lumbosacral plexitis a.k.a. idiopathic neuralgic amyotrophy a.k.a. Lumbosacral plexopathy Diagnosis: The diagnosis is based on clinical features, electrophysiological findings and exclusion of competing causes. Clinical features: Lumbosacral distribution of pain followed by weakness See lumbosacral plexopathy Findings on investigations: +EMG Localises the lesion to the lumbar/sacral plexus Localises the lesion … Continue reading Idiopathic Lumbosacral Plexitis

Cryoglobulinemia, Neurological Manifestations

Diagnosis: Clinical features plus isolation of cryoglobulins Clinical features: Hepatitis Purpura (legs), arthralgia, glomerulonephritis Raynaud’s phenomenon Peripheral neuropathy, precipitated by cold +serum Cryoglobulins: positive Type I cryoglobulin: Monoclonal protein without rheumatoid factor activity Associated with plasma cell dyscrasia Type II mixed cryoglobulin MC (formerly essential mixed cryoglobulinemia): Monoclonal protein with rheumatoid factor activity +polyclonal component … Continue reading Cryoglobulinemia, Neurological Manifestations

Behcet's Syndrome

Diagnosis: Recurrent oral ulceration plus two of the following: Recurrent genital ulceration Eye lesions Skin lesions Pathergy test: skin reaction to intradermal saline injection Findings on Investigations: MRI brain: Infarcts: brainstem and thalamic Cerebral Angiogram/angiography: Narrowing, aneurysm (arterial and venous) Investigations to consider: ESR and CRP: Raised FBC: leukocytosis Treatment: Mucous membrane involvement: Glucocorticoid mouthwash … Continue reading Behcet's Syndrome

Neurosarcoidosis, Sarcoid disease Neurological Manifestations

Neurosarcoidosis manifestations: Myelopathy Cranial neuropathies Pituitary disease Dural based lesion Encephalopathy CNS sarcoid angiitis Peripheral sensory neuropathy Myopathy Diagnosis: Histology: +typical findings +excluding other diseases confirms the diagnosis Histology: noncaseating granuloma Findings on Investigations: CXR: Stage 0: normal Stage I: bilateral hilar lymphadenopathy or paratracheal lymphadenopathy Stage II: hilar lymphadenopathy +pulmonary infiltrates Stage III: pulmonary … Continue reading Neurosarcoidosis, Sarcoid disease Neurological Manifestations