Sensory Guillian-Barre Syndrome

Oh SJ, LaGanke C, Claussen GC. Sensory Guillian Barre. Neurology 2001; 56:82-82. Describes eight patients collected with a sensory equivalent of classical GBS. These patients had acute onset of a sensory ascending neuropathy that peaked within 4 weeks, generally had an antecedent viral illness, areflexia, CSF albumen-cytologic dissociation, demyelination in at least two nerves, (esp MOTOR nerves), favorable outcome. 3 patients were treated with immunomodulatory treatments.

Neck pain and calcium deposition

Nodera et al. Neurology 2007; 68:383 (Neuroimages). Japanese. A 43 year old man had neck pain, sore throat, dysphagia, and low grade fever upon awakening. He had limited ROM of the neck. CT showed calcification ANTERIOR to C1-2 with thickened prevertebral tissues due to effusion. Retropharyngeal calcific tendinitis is uncommon and confused with abscess but the characteristic calcium deposit is diagnostic. It is self limiting.

Paraneoplastic cerebellar degeneration antibodies

Anti Yo (PCA-1) ovarian and breast cancer; anti Tr antibodies -- Hodgkin's lymphoma; also for Hodgkin's lymphoma: Anti-mGluR1 and Anti Zic4 antibodies. Above are PREDOMINANTLY associated with PCD. SOMETIMES associated with PCD are anti VGCC (LEMS, small cell lung cancer); anti Hu (ANNA-1)(encephalomyelitis, PCD, sensory neuronopathy) (small cell lung and other CA); anti Ri (ANNA 2) (PCD, brainstem encephalitis; paraneoplastic opsoclonus/myoclonus)(breast, gyne and small cell CA);anti CV2/CRMP (encephalomyelitis, PCD, chorea, PN, uveitis)(small cell lung, thymoma, others); anti MA protein (limbic, hypothalamic, brainstem encephalitis, infrequently PCD)( testicular, lung and other cancers); antiamphiphysin (stiff person s, encephalomyelitis, PCD) (breast and small cell) CHART is FROM NEJM 2007; 356:612-620. MORE Tumors with anti Yo antibodies express Yo antigen which is a cytoplasmic protein called CDR2 that interacts with c-Myc. It is expressed in the Purkinje cells of the cerebellum and the large neurons of the brainstem. It sequesters c Myc and downregulates; disruption with antibodies may increase c Myc activity leading to apoptosis (although the T cell immune response may also be important).

rapidly progressive ataxia ddx

in immunocompentent patients:

stroke,
multiple sclerosis
sarcoidosis,
primary or metastatic diseases,
paraneoplastic disorders,
toxins/drugs (lead, anticonvulsants, salicyclates, aminoglycosides, sedatives, fluorouuacil, cytarabine),
Miller Fisher syndrome,
infection (HIV, viral/postviral cerebellitis, Creutzfeld Jacob disease, progressive multifocal leukoencephalopathy),
alcoholic cerebellar degeneration, thiamine deficiency,
autoimmune ( SLE, Sjogrens, Hashimoto's disease, cerebellar ataxia with anti GAD or antigliadin antibodies).

Lack of utility/overuse of muscle biopsy

Filosto M et al. The role of muscle biopsy in investigating isolated muscle pain. Neurology 2007; 68:181-186.
editorial Kissel JT. Muscle biopsy in patients with myalgia. Neurology2007; 68:170-171.

Filosto et al. reviewed clinicals and neuropath on 240 patients presenting with muscle pain isolated or cramping. 80 % had biopsy abnormalities but only 20 %got a specific diagnosis and only 6 % a specific myopathy. Patients with obvious causes eg. statin use were excluded before analysis. CK levels and EMG did not predict pathology. Patients need to go under rigorous selection prior to biopsy.

cryoglobulinemic vasculitis PN

Gemignani F et al. Clinical spectrum of cryoglobulinemic neuropathy. JNNP 2005; 76:1410-1414.

Most common presentation is middle aged female patients who develop small fiber sensory neuropathy (73%). Less often, then sensorimotor neuropathy (18 %) then least often, mononeuritis multiplex (8%) occurs. Symptoms were tingling paresthesias (55%), sensory ataxia (38%), thermal dysesthesias (42%) pain (42%) and RLS (45 %). 29 % were asymmetrical. Patients did not have autonomic dysfunction.


Study occurred of 71 patients referred to academic center. Cryoglobulnemic syndrome was defined as intense recurring purpura and cryocrit>5 %, and "mild" syndrome with minimal purpura and cryocrit <5%. 46 % presented with PN. Hepatitis C caused the cryoglobulinemia in more than 90 %.

Comment--most cases were noo biopsied.