Patients with CVID and Infectious Neurological Complications
Ref / Age / Sex / Clinical symptoms / Diagnosis / Treatment / Outcome
1 / 20 cases / Bacterial meningitis / Antibiotics, IVIG / Recurrent pneumococcal meningitis occurred after splenectomy in 2 pt and was fatal in one; both occurred before introduction of IVIG
1 / NM / NM / NM / Mumps encephaliatis / NM / NM
1 / NM / NM / NM / Varicella zoster encephalitis / NM / NM
4 / 56 / M / Ataxia, right sided hemiparesis, pretectaloculomotor syndrome, dysarthria / PML / Mirtazapine, Mefloquine / survived, residual neurologic deficits
8 / 29 / F / Dysarthria, ataxia, oculomotor apraxia, fine motor abnormalities / PML / NM / NM
7 / 40 / M / Right sided hemisensory deficits, ataxia, personality change, chorea / PML / steroids, IL-2, IFN-λ, IV cidofovir / died shortly after onset of neuro symptoms
6 / 38 / M / Diplopia, right homonymous hemianopia, upward gaze palsy / PML / NM / NM
5 / 21 / M / subacute encephalopathy, confusion, disorientation, word-finding difficulty / Atypical PML / IVIG, PEG-IFN / died 2 months later
9 / 54 / F / Altered mental status, meningeal symptoms / HSV encephalitis / acyclovir, IVIG / complete resolution
9 / 42 / M / Tonic-clonic seizure, obtundation / HSV encephalitis / acyclovir, IVIG / complete resolution at 3 year f/u
9 / 25 / M / Delirium, combativeness / HSV encephalitis / acyclovir, IVIG / severe Wernicke's aphasia and cognitive impairment at d/c
25 / 3.5 / M / spastic gait and paraparesis, sensory ataxia, right hand weakness / Echovirus and adenovirus meningoencephalitis / steroids, IVIG / complete resolution at 2 year f/u
25 / 6 / F / Fever, irritability, drowsiness, vomiting, headache / Adenovirus meningoencephalitis / Rickham reservoir, IVIG, VP shunt / died 6 weeks after admission
31 / 20 / F / Left arm weakness / CMV encephalomyelitis, retinitis / ganciclovir, foscarnet, cidofovir / survived with treated recurrences
36 / 38 / F / Meningeal symptoms / West Nile meningitis / NM / complete resolution
30 / 23 / M / Ataxia, behavioral changes, memory impairment, spastic quadriparesis and dysarthria / BKV encephalitis / ganciclovir, IVIG / died 1.5 years later
35 / 14 / F / HA, acute right facial and abdominal paresthesia / Varicella angiitis / aspirin, acyclovir / no further ischemic events
33 / 52 / F / subacute gait ataxia, upper limb ataxia, dysarthria, dysphagia, spastic tetraparesis / Cerebral toxoplasmosis / pyrimethamine, sulfadiazine / tetraparesis resolved, dysarthria prominent at 14 weeks f/u
34 / 40 / F / Gait instability, expressive dysphagia / Toxoplasma encephalitis / Antibiotics indefinitely / survived, residual neurologic deficits
46 / 40 / M / Right sided hemiparesis, dysarthria, personality change / PML / NM / died shortly after onset of neuro symptoms
27 / 22 / F / Altered mental status, GCS 8 / Pneumococcal meningitis / Antibiotics, IVIG / NM
29 / 27 / F / hyperpyrexia, headache, vomiting, sopor, meningismus / Pneumococcal meningitis / Antibiotics / complete clinical and microbiological cure
28 / 66 / M / dizziness, tinnitus, hearing loss, confusion, left central facial paresis, fever / Histoplasmosis / amphoctericin / complete resolution

NM = not mentioned; PML = progressive multifocal leukoencephalopathy, HSV = herpes simplex virus, CMV = cytomegalovirus, BKV = BK virus

Patients with CVID and Autoimmune/Inflammatory Neurological Complications
Ref / Age / Sex / Clinical symptoms / Diagnosis / Treatment / Outcome
11 / 71 / F / spastic gait and paraparesis, weakness of right hand, loss of vibration and position sense in legs / Recurrent cervical/thoracic transverse myelitis / steroids, IVIG / good response to treatment at 3 year f/u
48 / 25 / M / subacute paralysis of legs, urinary retention / Longitudinally extensive transverse myelitis/neurosarcoidosis / steroids, PLEX, infliximab / regained lower extremity strength and bladder control after 2nd dose of infliximab; no response to steroids, PLEX
10 / 41 / F / progressive bilateral leg weakness, urinary hesistancy and frequency, / Transverse myelitis / Steroids / resolution of symptoms
38 / 64 / F / spastic ataxic gait, urinary frequency, hand/LE weakness, distal LE paresthesias / Myelitis / infliximab, IVIG / stabilized with treatment, but deficits remained
12 / 16 / F / new-onset seizure, confusion / Limbic encephalitis / steroids, IVIG / seizure frequency increased with tapering of steroids
45 / 1 / F / Convulsions / Acute disseminated encephalomyelitis / NM / at 7 year f/u, seizure disorder controlled on AED, but patient has psychomotor development of 3 year old
14 / 31 / F / vertigo, spontaneous nystagmus, glossopharyngeal paresis / Acute encephalomyelitis / steroids, IVIG / slow clinical improvement over 2 weeks, complete resolution at 3 months
39 / 7 / F / flaccidtetraparesis, difficulty walking / Chronic inflammatory demyelinating polyneuropathy / IVIG / walking at 6 week f/u; complete resolution at 8 mo f/u
37 / 26 / M / loss of vision bilaterally / Bilateral optic neuritis / Steroids / visual acuity 1 in right eye and 0.8 in left eye
40 / 26 / M / lower extremity muscle weakness with paresthesias in hands and feet / GuillianBarre Syndrome - paraneoplastic phenomenon / IVIG / complete remission after chemotherapy
43 / 11 / F / facial twitch / Granulomatous mass / Resection / had recurrence 10 years later requiring second resection
41 / 67 / M / weight loss, progressive weakness/numbness in lower extremities, gait imbalance, papilledema / Intraspinal granuloma/neurosarcoidosis / fluconazole, steroids / sx halted and MRI showed improvement with IVIG

sx = symptoms, mo = months, AED = antiepileptic drugs, PLEX = plasma exchange, LE = lower extremities

Patients with CVID and Neuroendocrine Complications
Ref / Age / Sex / Clinical symptoms / Diagnosis / Treatment / Outcome
15 / 10 / F / short stature / Growth hormone deficiency / rhGH / NM
16 / 37 / M / thirst, polyuria, nocturia several years / Partial central diabetes insipidus / DDAVP / resolution of symptoms
17 / 6 / F / weakness, cold sweats, pallor, sleepiness, semi-comatose upon admission / Isolated ACTH deficiency / oral cortisone acetate / resolution of symptoms

ACTH = adrenocorticotropic hormone, rhGH = recombinant human growth hormone, DDAVP = desmopressin acetate, NM = not mentioned