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Table 1 Clinical and paraclinical findings, tumor associations, therapies, and outcome of three patients with septin-3 IgG antibodies

From: Septin-3 autoimmunity in patients with paraneoplastic cerebellar ataxia

Patient #, sex, age at neurological onset

Clinical summary

Cancer

Cancer therapy (start before/after onset of neurological symptoms)

Septin-3 IgG

Coexisting antibodies

Brain MRI

CSF findings

Immunotherapy (response)

Follow-up time (months)

Outcome

#1

male, 64a

Subacute progressive cerebellar syndrome with dysarthria, bilateral limb and gait ataxia

Malignant melanoma (Clark level IV) right groin with inguinal lymph node metastases

Adjuvant immunotherapy with interferon-alpha-2b

(5 months before)

Serum 1:32.000

Antinuclear Abs (1:320), GAD 65 Abs (28 units/ml, reference range 10 units/ml), GABAB receptor Abs 1:100

Few small unspecific microangiopathic frontal lesions

3 cells/µl, TPb 718 mg/l, QAlb mildly elevated, intrathecal IgG synthesis (56%)c

3 × 500 mg IVMP (none),

PE (transient), IVIG (transient), rituximab (transient)

29

Death

#2

male, 69

Subacute progressive cerebellar syndrome with dysarthria and marked gait ataxia

Malignant melanoma (Clark level IV) back with lung, liver and axillary lymph node metastases

Irradiation; adjuvant immunotherapy with nivolumab; dabrafenib and trametinib

(19 months before)

Serum 1:10.000, CSF 1:1000

None

Mild cerebellar atrophy, moderate microangiopathy

13 cells/µl, TPb 611 mg/l, normal QAlb, CSF-specific OCB

5 × 1000 mg IVMP (partial improvement)

32

Cerebellar syndrome stable, but worsening (epileptic seizures) due to cerebral metastases

#3

male, 60

Subacute progressive cerebellar syndrome with dysarthria, ataxia left leg, down beat nystagmus

Small-cell lung cancer with mediastinal lymph node metastases

Cisplatin/etoposide and atezolizumab

(3 months after)

Serum

1:100.000

Antinuclear Abs (1:160)

Normal

20 cells/µl, TPb 889 mg/l, CSF-specific OCB

5 × 250 mg IVMP, oral prednisolone (no further worsening)

15

Neurological symptoms stable

  1. Abs, antibodies; CSF, cerebrospinal fluid; IgG, immunoglobulin G; IVMP, intravenous methylprednisolone; IgG, immunoglobulin G; IVIG, intravenous immunoglobulins; MRI, magnetic resonance imaging; OCB, oligoclonal bands; PE, plasma exchange; TP, total protein
  2. aPatient 1 was previously reported in [7]
  3. bUpper limit of normal of CSF total protein: 450 mg/l
  4. cNormal value for intrathecal IgG production: 0%